Tyldesley - S Oral Medicine 5th Ed - Field

Authors: Field, Anne; Longman, Lesley
Title: Tyldesley's Oral Medicine, 5th Edition

Copyri ght 2003 Oxford Uni versi ty Press
> Front of Book > Authors
Authors
Anne Field
Seni or Lecturer & Honorary Consul tant i n Oral Medi ci ne
Department of Cl i ni cal Dental Sci ences, Li verpool Uni versi ty Dental Hospi tal , Pembroke Pl ace,
Li verpool , UK
Lesley Longman
Consul tant & Honorary Seni or Lecturer i n Restorati ve Denti stry
Department of Cl i ni cal Dental Sci ences, Li verpool Uni versi ty Dental Hospi tal , Pembroke Pl ace,
Li verpool , UK
Secondary Author
William R. Tyldesley
Former Dean of Dental Studi es
Uni versi ty of Li verpool , UK
i n col l aborati on wi th
Specialist advisers
David Casson
Consul tant i n paedi atri c gastroenterol ogy
Li verpool Chi l drens' Hospi tal , Li verpool , UK
John Cooper
Consul tant i n oral and maxi l l ofaci al surgery
Uni versi ty Hospi tal Ai ntree, Li verpool , UK
Patrick Chu
Consul tant haematol ogi st
Royal Li verpool and Broadgreen Uni versi ty Hospi tal Trust, Li verpool , UK
Mark Davies
Consul tant anaestheti st
Li verpool Uni versi ty Dental Hospi tal and Royal Li verpool and Broadgreen Uni versi ty Hospi tal
Trust, Li verpool , UK
Luke Dawson
Cl i ni cal l ecturer i n oral surgery
Li verpool Uni versi ty Dental Hospi tal , Li verpool , UK
John Field
Professor of mol ecul ar oncol ogy
The Uni versi ty of Li verpool , UK
John Hamburger
Seni or l ecturer and honorary consul tant i n oral medi ci ne
The Uni versi ty of Bi rmi ngham School of Denti stry, UK
Eileen Manning
Consul tant i n cl i ni cal chemi stry
Royal Li verpool and Broadgreen Uni versi ty Hospi tal Trust, Li verpool , UK
Michael Martin
Seni or l ecturer and honorary consul tant i n oral mi crobi ol ogy
Shevaun Mendelsohn
Consul tant dermatol ogi st
Countess of Chester Hospi tal , Chester, UK
Paul Nixon
Consul tant i n oral and maxi l l ofaci al radi ol ogy
Simon Rogers
Consul tant i n oral and maxi l l ofaci al surgery and honorary reader
Uni versi ty Hospi tal Ai ntree, Li verpool , UK
Jane Setterfield
Seni or l ecturer/honorary consul tant i n dermatol ogy
Ki ngs Col l ege, London, UK
Eileen Theil
Cl i ni cal l ecturer i n peri odontol ogy
Emma Varga
Associ ate speci al i st i n oral surgery and l ecturer in oral medi ci ne
Julia Woolgar
Seni or l ecturer and honorary consul tant i n oral pathol ogy
Joanna Zakrzewska
Seni or l ecturer and honorary consul tant
Barts and the London, Queen Mary' s School of Medi ci ne and Denti stry, London, UK
Title: Tyldesley' s Oral Medicine, 5th Edition
> Front of Book > Pr eface to Fi ft h Edit i on
Preface to Fifth Edition
The 5
t h
Edi ti on of Tyl desl ey' s Oral Medi ci ne has been thoroughl y updated i n l i ne wi th current
teachi ng practi ces. It conti nues to serve as a comprehensi ve textbook of oral medi ci ne, not
onl y f or undergraduate dental students, but al so f or conti nui ng medi cal and dental educati on.
The emphasi s throughout the book i s on cl i ni cal presentati on, i nvesti gati on and management
of orofaci al di seases. A new author, Lesl ey Longman, demonstrates the i nval uabl e
contri buti on made by speci al i sts i n other di sci pl i nes to mul ti di sci pl i nary cl i ni cs, i n whi ch
compl ementary ski l l s and experi ence combi ne to i mprove the qual i ty of pati ent care i n oral
medi ci ne.
Speci al i st advi sors, each wi th thei r own area of experti se, have made a si gni fi cant
contri buti on to the book and ensured that recent advances i n cl i ni cal practi ce have been
i ncorporated i nto the text.
Hi gh-qual i ty, col our i l l ustrati ons are i ncl uded to add i nterest and demonstrate si gni fi cant
poi nts; no attempt has been made to i l l ustrate al l the l esi ons and condi ti ons descri bed i n the
text, these wi l l be found i n the comprehensi ve range of oral medi ci ne col our atl ases currentl y
avai l abl e.
Thi s edi ti on has undergone major revi si on i n terms of l ayout and content. The chapters on
faci al pai n, psychogeni c orofaci al probl ems, sal i vary gl and di sorders and oral carci noma have
been si gni fi cantl y extended. A secti on on renal di sease has al so been added, together wi th a
chapter on medi cal emergenci es i n denti stry. Case studi es, demonstrati ng oral medi ci ne
condi ti ons, have been i ntroduced at the begi nni ng of each rel evant chapter, wi th a di scussi on
of thei r di ff erenti al di agnosi s and management opti ons at the end of the chapter. These are
i ncl uded to promote sel f-di rected and acti ve l earni ng but we hope that they wi l l al so promote
di scussi on and debate. A number of projects have also been added at the end of chapters
wi th the purpose of encouragi ng students to consul t other ref erence sources and on-
l i ne i nformati on.
Tyl desl ey' s Oral Medi ci ne has become fi rml y establ ished as the textbook of choi ce for
successi ve generati ons of dental students and practi ti oners and we hope that thi s compl etel y
revi sed edi ti on wi l l accuratel y refl ect current cl i ni cal practi ce and i deas i n the rapi dl y
changi ng di sci pl i ne of oral medi ci ne.
> Front of Book > Di scl ai mer
Disclaimer
Oxf ord Uni versi ty Press makes no representati on, express or i mpl i ed, that the drug dosages
i n thi s book are correct. Readers must theref ore al ways check the product i nf ormati on and
cl i ni cal procedures wi th the most up to date publ i shed product i nf ormati on and data sheets
provi ded by the manuf acturers and the most recent codes of conduct and saf ety regul ati ons.
The authors and the publ i shers do not accept responsi bi l i ty or l egal l i abi l i ty for any errors i n
the text or f or the mi suse or mi sappl i cati on of materi al i n thi s work.
> Front of Book > Dedi cat i on
Dedication
Anne Fi el d woul d l i ke to dedi cate thi s book to the memory of her l ate father, John El ton
Costl ey, and Lesl ey Longman to the memory of her l ate mother, Patri ci a McLoughl i n.
> Front of Book > Acknowl edgement s
Acknowledgements
The authors are gratef ul f or the support and encouragement of thei r fami l i es duri ng the
preparati on of thi s compl etel y revi sed edi ti on of Tyl desl ey' s oral medi ci ne.
Our speci al i st advi sers have made an i nval uabl e contri buti on to the book and we are i ndebted
to them for thei r pati ence, knowl edge, and encouragement duri ng the preparati on of thi s new
edi ti on. Thanks are al so due to col l eagues who have contri buted i l l ustrati ve materi al s and to
our enthusi asti c juni or staff who have read draft chapters of the book and made hel pf ul
suggesti ons f or i mprovement.
Fi nal l y, we woul d l i ke to thank Mrs Jan Vi cary for her excel l ent typi ng and word-processi ng
ski l l s duri ng the many months we have been rewri ti ng the fi fth edi ti on of the book.
> Front of Book > List of abbreviati ons
List of abbreviations
Abbreviations
5-HT
5-hydroxytryptami ne (al so known as serotoni n)
ACE
angi otensi on-converti ng enzyme
ACTH
adrenocorti cotrophi c hormone
AIDS
acqui red i mmune defi ci ency syndrome
ANA
anti nucl ear anti body
ANUG
acute necroti zi ng ul cerati ve gi ngi vi ti s
ASA
Ameri can Soci ety of Anesthesi ol ogi sts
AZT
azi dothymi di ne
BMS
burni ng mouth syndrome
BNF
Bri ti sh Nati onal Formul ary
BP
bul l ous pemphi goi d
CAPD
conti nuous ambul atory peri toneal di al ysi s
CBT
cogni ti ve behavi oural therapy
CD
Crohn' s di sease
CMC
chroni c mucocutaneous candi dosi s
CO
centri c occl usi on
CR
centri c rel ati on
CRF
chroni c renal fai l ure
CRP
C-reacti ve protei n
CRST or CREST
C, cal ci fi cati on; R, Raynaud' s phenomenon; E, oesophageal dysf uncti on; S, scl erodactyl y; T,
tel angi ectasi a (syndrome)
CT
computeri zed tomography
DLE
di scoi d l upus erythematosus
DM
di abetes mel l i tus
DPT
dental panorami c tomograph
EB
epi dermol ysi s bul l osa
EBV
Epstei nBarr vi rus
EDTA
ethyl enedi ami ne tetraaceti c aci d
EM
erythema mul ti forme
EMG
el ectromyography
ENT
ear, nose, and throat
ESR
erythrocyte sedi mentati on rate
FAPA
(peri odi c) fever, aphthous ul cers, pharyngi ti s, and cervi cal adeni ti s (syndrome)
FBC
ful l bl ood count
FISH
fl uorescent i n si tu hybri di zati on
FNA
fi ne needl e aspi rate (bi opsy)
FTA (abs)
fl uorescent Treponema anti body absorbed (test)
GFT
gl omerul ar fi l trati on rate
GORD
gastro-oesophageal ref l ux di sease
GP
general practi ti oner
GT
gl utaryl transf erase
GTN
gl yceryl tri ni trate
GUM
geni touri nary medi ci ne
HAD
Hospi tal Anxi ety and Depressi on (scal e)
Hb
haemogl obi n
HHV-8
herpes vi rus 8
HIV
human i mmunodefi ci ency vi rus
HLA
human l eukocyte anti gen
HPV
human papi l l omavi ruses
HSV
herpes si mpl ex vi rus
HU
herpeti f orm ul cerati on
IBD
i nfl ammatory bowel di sease
IBS
i rri tabl e bowel syndrome
IMF
i mmunofl uorescence
INR
i nternati onal normal i zed rati o
LDE
Li chenoi d drug erupti on
LE
l upus erythematosus
MAGIC
mouth and geni tal ul cers wi th i nfl amed carti l age (syndrome)
MALT
mucosa-associ ated l ymphoi d ti ssue
MCV
mean corpuscul ar vol ume
MiRAS
mi nor recurrent aphthous stomati ti s
MjRAS
major recurrent aphthous stomati ti s
MMP
mucous membrane pemphi goi d
MRI
magneti c resonance i magi ng
MRS
Mel kerssonRosenthal syndrome
MRTA
magneti c resonance tomographi c angi ography
MS
mul ti pl e scl erosi s
NRL
natural rubber l atex
NSAID
nonsteroi dal anti -i nfl ammatory drug
NSU
non-speci f i c urethri ti s
OFG
orof aci al granul omatosi s
OSCC
oral squamous cel l carci noma
OSF
oral submucous fi brosi s
OTC
over the counter
PAS
peri odi c aci dSchi f f (reagent)
PCR
pol ymerase chai n reacti on
PPK
pal mopl antar keratoderma
PTA
pol ymyxi n E, tobramyci n, and amphoteri ci n (l ozenges)
PTH
parathormone
RAS
recurrent aphthous stomati ti s
RNP
ri bonucl eoprotei n
RT-PCR
reverse transcri ptase pol ymerase chai n reacti on
SCC
squamous cel l carci noma
SLE
systemi c l upus erythematosus
SNP
si ngl e nucl eoti de pol ymorphi sm
SPF
sun protecti on factor
SS
Sjgren' s syndrome
SSRI
sel ecti ve serotoni n reuptake i nhi bi tor
SV40
si mi an vi rus 40
TMJ
temporomandi bul ar joi nt
TMPDS
temporomandi bul ar pai n dysfuncti on syndrome
TNF
ti ssue necrosi s factor al pha
TPHA
Treponema pal l i dum haemagl utti nati on assay
TPI
Treponema pal l i dum i mmobi l i zati on (test)
TPMT
thi opuri ne methyl transferase
TSG
tumour suppressor gene
UC
ul cerati ve col i ti s
USG-FNA
ul trasound-gui ded fi ne needl e aspi rati on
VDRL
Venereal Di sease Reference Laboratory (test)
VVG
vul vovagi nal gi ngi val (syndrome)
VZV
vari cel l a zoster vi rus
WCC
whi te cel l count
TABLE OF CONTENTS

[+] 1 - The oral mucosa
[+] 2 - Principles of oral medicine: assessment and investigation of patients
[+] 3 - Therapy
[+] 4 - Infections of the gingivae and oral mucosa
[+] 5 - Oral ulceration
[+] 6 - Diseases of the lips and tongue and disturbances of taste and halitosis
[+] 7 - Swellings of the face and neck
[+] 8 - Salivary glands and saliva
[+] 9 - Inflammatory overgrowths, developmental and benign lesions, and pigmentation of the oral mucosa
[+] 10 - Precancerous lesions and conditions. Oral carcinoma and carcinogenesis
[+] 11 - Mucocutaneous disease and connective tissue disorders
[+] 12 - Gastrointestinal disease
[+] 13 - Blood and nutrition, endocrine disturbances, and renal disease
[+] 14 - Immunodeficiency, hypersensitivity, autoimmunity, and oral reactions to drug therapy
[+] 15 - Facial pain and neurological disturbances
[+] 16 - Temporomandibular disorders
[+] 17 - Psychogenic orofacial problems
[+] 18 - Disorders of the teeth and bone
[+] 19 - Medical emergencies in dentistry
BACK OF BOOK

[-] Resources
[-] Appendix
- Chlortetracycline mouthwash
- Triamcinolone (plain) mouthwash
- Triamcinolone with 2 per cent chlortetracycline mouthwash
- Suggestions for further reading and reference sources


> Tabl e of Cont ent s > 1 - The oral mucosa
1
The oral mucosa
Normal oral mucosa
Structure
In i ts basi c structure the oral mucous membrane resembl es other l i ni ng mucous membranes,
for exampl e, those of the vagi na or the oesophagus. Wi thi n the mouth, however, there i s a
wi der range of epi thel i al thi ckness than that seen at other mucosal si tes. These vari ati ons
depend l argel y on di f ferences i n the degree of kerati ni zati on shown by the mucosae i n
di f ferent areas of the mouth. Some of the reacti ons of the oral mucous membrane resembl e
those of the ski n, presumabl y because of i ts posi ti on i n the transi ti on area between the
gastroi ntesti nal tract and the ski n. As a resul t of thi s, di seases of both mucous membranes
and ski n may produce l esi ons i n the mouth. The oral mucosa, however, characteri sti cal l y
behaves as a mucous membrane. Its behavi our i n di sease processes perhaps most cl osel y
resembl es that of the vagi nal mucosa.
The epi thel i um coveri ng the oral mucosa shows a wi der vari ati on i n thi ckness and i n i ts
pattern of kerati ni zati on than mucous membranes at other si tes.
The oral mucous membrane consi sts both anatomi cal l y and functi onal l y of two l ayers: one
(the cori um or l ami na propri a) essenti al l y of mesodermal ori gi n and one epi thel i al (Fi g. 1. 1).
When consi deri ng vari ati ons of structure, the behavi our of the cori um must be taken i nto
account even though the major changes may appear to be wi thi n the epi thel i al l ayer.
Fig. 1.1 A secti on of normal mucous membrane f rom the hard pal ate wi th a kerati ni zed
The oral epithelium
The kerati nocytes are the mai n cel l component of the oral epi thel i um but other cel l types
i ncl ude mel anocytes, Langerhans cel l s, and Merkel cel l s.
THE KERATINOCYTES
In normal mucous membrane the i ntegri ty of the epi thel i um i s mai ntai ned by the di vi si on of
kerati nocytes i n the basal l ayer. As each cel l di vi des one resul ti ng cel l remai ns effecti vel y i n
si tu, whi l e one mi grates towards the surf ace, undergoi ng vari ous structural modi fi cati ons as
i t passes through the epi thel i um (Fi g. 1. 2). These modi fi cati ons, whi ch are dependent on the
process of kerati ni zati on, vary accordi ng to the preci se si te of the mucosa i nvol ved and
resul t i n the producti on of a surface l ayer of cel l s that are ei ther f ul l y, parti al l y, or non-
kerati ni zed and whi ch are shed i nto the oral cavi ty at a rate dependent on the rate of mi tosi s
i n the basal l ayer. For each di vi di ng cel l , one cell i s l ost from the surface and, thus, the
i ntegri ty and di mensi ons of the epi thel i al l ayer are mai ntai ned. The rate of turnover of these
cel l s, that i s, the transi t ti me, f or a kerati nocyte i n the basal l ayer to reach the
surface i n vari ous human epi thel i a has been determi ned by a number of techni ques. The rate
i n human ski n i s general l y quoted as bei ng of the order of 50 to 70 days and that i n the
gi ngi val epi thel i um much the same, but that i n the buccal epi thel i um i s much fasterof the
order of 25 days.
The epi thel i um of the oral mucosa shows wi de vari ati ons i n the extent of the kerati ni zati on
process. In the f ul l y kerati ni zed si tuati on, the rather cubi cal cel l s f ormed by mi tosi s at or
near the basal l ayer mi grate towards the surf ace, becomi ng more pol yhedral and shari ng
i ntercel l ul ar attachments, whi ch have gi ven the name pri ckl e cel l l ayer (or stratum
spi nosum) to thi s zone. In the l i ght mi croscope these i ntercel l ul ar pri ckl es appear as
si ngl e attachments of the cel l wal l s, but by el ectron mi croscopy these i ntercel l ul ar juncti ons
(ref erred to as desmosomes) are seen to be of much greater compl exi ty. It i s probabl e that
the desmosomes act i n a mechani cal manner to gi ve strength to the epi thel i um. In several
di seases marked by epi thel i al f ragi l i ty the desmosome attachments are l ost or i mpai red. It
shoul d perhaps be added that si mi l ar, one-si ded structures, hemi desmosomes, attach the

pl asma membrane of the basal kerati nocytes to the l ami na l uci da of the basement membrane
compl ex. As the cel l s of the stratum spi nosum mi grate to the surface they begi n to fl atten
and granul ar structures (keratohyal i n granul es) appear wi thi n them. These granul es gi ve the
characteri sti c appearance to the stratum granul osum i n kerati ni zed epi thel i a. Fi nal l y,
at or near the surf ace, the epi thel i al cel l s l ose thei r detai l ed i nner structure, the nucl ei
degenerate, the keratohyal i n granul es fragment and di sappear, and the i nsol ubl e protei n
compl exes menti oned above fi l l the cel l , now f ul l y kerati ni zed (Fi g. 1. 2). At thi s stage the
desmosomes have ef fecti vel y degenerated al so and the f l attened cel l s (squames) are
eventual l y l ost i nto the oral cavi ty. As has been poi nted out, each kerati ni zed cel l l ost i n thi s
way must be matched by a di vi di ng cel l i n the prol i ferati ng compartment of the epi thel i um i n
order for stabi l i ty to be mai ntai ned. Thi s process of renewal appl i es onl y to ful l y kerati ni zed
epi thel i umas seen, for i nstance, i n the mucous membrane overl yi ng the hard
pal ateand i s usual l y ref erred to as orthokerati ni zati on. In other areas (as i n some parts of
the buccal mucosa and the fl oor of the mouth), thi s process of kerati ni zati on does not take
pl ace, keratohyal i n granul es are not formed, and nucl ei and organel l es (al though somewhat
eff ete) can be seen i n the surf ace l ayers (Fi g. 1. 3). In an i ntermedi ate form (parakeratoti c
epi thel i um), nucl ei may sti l l be seen i n the surface l ayers and keratohyal i n i s sparse or
absent. For the purpose of understandi ng the cl i ni cal si gni fi cance of these di f ferences i n
surface l ayer of epi thel i um overl yi ng the cori um.
P. 4
kerati ni zati on, they shoul d be regarded as bei ng part of a spectrum rangi ng f rom compl ete
non-kerati ni zati on at one extreme, through varyi ng degrees of parakerati ni zati on, to ful l
orthokerati ni zati on at the other.
Fig. 1.2 Di agram of a kerati ni zi ng squamous epi thel i um. Compare wi th Fi g. 1. 1.
The di stri buti on of these di f feri ng epi thel i a i n the normal oral mucosa has a cl ose
rel ati onshi p wi th the functi on of the ti ssues at the si te. In normal mucosa, non-kerati ni zed or
parakerati ni zed epi thel i um i s seen on the buccal mucosa, the fl oor of the mouth, and the
ventral surf ace of the tongue, whereas orthokerati ni zed epi thel i um i s seen on the hard pal ate
and parts of the gi ngi vae. The dorsal surface of the tongue i s al so orthokerati ni zed, but
di f fers from the other oral mucosal surf aces i n that there are a number of speci al i zed
structures present, predomi nantl y the papi l l ae. There are four types of l i ngual papi l l ae:
anteri orl y the fi l i form and fungi form; posteri orl y f ol i ate and val l ate papi l l ae. The fi l i f orm and
fungi f orm papi l l ae are of cl i ni cal si gni fi cance i n that thei r atrophy i s of ten an earl y si gn of
mucosal abnormal i ty.
Fig. 1.3 A secti on of normal buccal mucosa. Compare wi th the kerati ni zed pal atal
mucosa shown i n Fi g. 1. 1.
There are four types of l i ngual papi l l ae: anteri orl y the fi l i f orm and fungi form; posteri orl y
fol i ate and val l ate papi l l ae.

There i s currentl y a great deal of research concerni ng geneti c abnormal i ti es of kerati ni zati on,
parti cul arl y di sturbances i n the genes codi ng for speci f i c kerati nsthat are responsi bl e for
di seases, such as epi dermol ysi s bul l osa (Chapter 11). In addi ti on, cel l adhesi on and the
mol ecul es associ ated wi th i t (f or exampl e, cadheri ns and i ntegri ns) are of great i mportance i n
di sorders of the ski n and mucous membranes. In the pemphi gus group of i mmunobul l ous
di seases, there i s faul ty adhesi on between kerati nocytes due to the devel opment of
autoanti bodi es agai nst desmogl ei n I and III, whi ch are members of the cadheri n fami l y of
adhesi on mol ecul es (Chapter 11).
THE MELANOCYTES
The mel anocytes appear i n, or very cl ose to, the basal l ayer and on el ectron mi croscopy show
granul ar structures (mel anosomes) that are the precursors of mel ani nthe bl ack pi gment
that modi fi es the col our of both ski n and mucous membranes. These cel l s, l i ke the
Langherhans cel l s, are dendri ti c wi th cytopl asmi c prol ongati ons extendi ng between the cel l s
of basal and suprabasal areas of the kerati nocytes. Mel anocytes synthesi ze but do not retai n
mel ani n; i t i s transferred by the dendri ti c processes to adj acent kerati nocytes. The mel anoti c
pi gmentati on of oral mucosa, l i ke that of the ski n, shows great raci al vari ati on. However, thi s
does not depend on vari ati on of the numbers of mel anocytes but on the number and acti vi ty
of the mel anosomes wi thi n them. The epi thel i a of al l races contai n approxi matel y the same
number of mel anocytes. It i s the rate of producti on of mel ani n and i ts di stri buti on that are
di f ferent. It i s known that hormonal i nfl uences are i mportant i n the sti mul ati on of mel anocyte
acti vi ty, al though the preci se mechani sms remai n obscure. In some ci rcumstances the
mel anocytes may be sti mul ated to produce excess mel ani n by a wi de range of non-hormonal
sti mul ae (Chapter 9).
Mel anocytes synthesi ze but do not retai n mel ani n. It i s transferred by the dendri ti c processes
to adjacent kerati nocytes.
LANGERHANS CELLS
The Langerhans cel l s were fi rst i denti f i ed i n 1860, and f or a l ong ti me were somethi ng of a
mystery. There i s a substanti al number of these cell s present near the basal compl ex of the
oral epi thel i um, wi th dendri ti c processes extendi ng between the kerati nocytes and wi th
recogni zabl e ul trastructural features. Over the l ast 34 decades research has shown that
these cel l s have an i mmunol ogi cal functi on, acti ng as peri pheral scavengi ng cel l s of the
i mmune system, rather l i ke macrophages but l acki ng thei r abi l i ty to phagocytose eff ecti vel y.
It woul d seem that at l east one f uncti on of these cel l s i s to act as anti gen-presenti ng cel l s
and sti mul ate the acti vati on of T l ymphocytes agai nst them. Langerhans cel l s are therefore
consi dered to be i mmunol ogi cal l y competent dendri ti c cel l s, si mi l ar to those f ound i n the
peri pheral l ymphati c system. Thei r ori gi n appears to be the bone marrow and not the
epi thel i um.
Kerati nocytes and Langerhans cel l s pl ay an i mportant part i n the i mmunosurvei l l ance of the
oral epi thel i um, and both secrete and respond to i mmunol ogi cal l y acti ve cytoki nes, i ncl udi ng
the i nterl euki ns and i nterferons. The concept of l ocal i zed mucosal i mmuni ty i s currentl y
under i nvesti gati on wi th regard to both i ts functi on i n mai ntai ni ng the i ntegri ty of normal
ti ssue and i ts rol e i n mucosal di sease. The pathogenesi s of l i chen pl anus i s not yet ful l y
understood, but probabl y i nvol ves a cel l -medi ated i mmune response to an external agent, i n
whi ch Langerhans cel l s and kerati nocytes rel ease cytoki nes and adhesi on mol ecul es to whi ch
T l ymphocytes can bi nd. Subsequent acti vati on of cytoxi c l ymphocytes i s thought to be
P. 5
responsi bl e for damage to the basal cel l s, whi ch i s a characteri sti c feature of thi s condi ti on.
Langerhans cel l s are i mmunol ogi cal l y competent dentri ti c cel l s.
The corium
The cori um (l ami na propri a) of the oral mucous membrane i s separated f rom the submucosal
l ayer, by a zone of gradual transi ti on rather than a cl ear boundary. In the cori um and
submucosa l i e the mi nor sal i vary gl ands and sebaceous gl ands of the oral cavi ty. These are
wi del y vari abl e i n di stri buti on, the mucous gl ands bei ng most frequent i n the mucosa of the
l i ps and posteri or pal ate, whi l st the sebaceous gl ands are mostl y concentrated i n the buccal
mucosa where they may appear as yel l ow spots, known as Fordyce' s spots. The submucosal
ti ssue components are al so wi del y vari abl e: bl ood vessel s, fat, and fi brous ti ssue bei ng
present i n di f feri ng proporti ons accordi ng to the preci se si te. Wi thi n the cori um and
submucous ti ssues are scattered cel l s of the l eukocyte seri es i n varyi ng proporti ons and
concentrati ons. Duri ng di sease processes these may al ter radi cal l y, both i n number and i n
type, dependi ng on the basi c nature of the pathol ogi cal process i nvol ved.
Fordyce' s spots are the sebaceous gl ands of the oral mucosa. They are frequentl y mi staken
for pathol ogi cal l esi ons but are compl etel y normal .
The basement membrane
Lyi ng between the epi thel i um and cori um of the oral mucous membrane i s a compl ex
mul ti l ayered structure, the basement membrane. On ul trastructural study i t i s seen that the
components of the basal zone are much fi ner than suggested by l i ght mi croscopy and that,
rather than a si ngl e membrane, a number of l ayers are vi si bl e, i ncl udi ng the l ami na l uci da
and the l ami na densa. In the basement membrane, anchori ng fi bres attach the l ami na densa
to the underl yi ng ti ssue and hemi desmosomes attach the basal cel l s of the epi thel i um to the
l ami na l uci da. Bel ow the l evel of the hemi desmosomes i s the subbasal dense pl ate, through
whi ch anchori ng fi l aments connect the l ami na l uci da to the l ami na densa. Autoanti bodi es to
components of the l ami na l uci da have been i denti fi ed i n bul l ous pemphi goi d, whi ch i s an
i mmunobul l ous ski n di sease (Chapter 11).
Function
Al though the oral mucous membrane has several f uncti ons, sensory and secretory among
them, i ts mai n purpose i s probabl y that

of acti ng as a barri er. It protects the deeper structures from mechani cal i nsul ts, such as
masti catory trauma and al so prevents the entry of mi cro-organi sms and some toxi c
substances. The oral mucosa has an extensi ve sensory i nnervati on that can di scri mi nate
touch and temperature. Taste buds are al so l ocated i n oral epi thel i um. In consi deri ng the
protecti ve functi on of the oral mucosa, i t i s al so necessary to di scuss other f actors, i n
parti cul ar the rol e of sal i va (Chapter 8). The oral mucosa i s constantl y bathed by sal i va,
whi ch not onl y mai ntai ns the physi ol ogi cal envi ronment necessary for the mai ntenance of
epi thel i al i ntegri ty but al so i ncl udes several protecti ve, anti bacteri al components. A number
of these have been descri bed, but perhaps the most i mportant are the secretory
i mmunogl obul i ns, predomi nantl y of the IgA cl ass, that are f ound i n sal i va and that attach to
si tes on the epi thel i al surf ace.
In spi te of thi s barri er f uncti on of the oral mucosa, there i s a degree of permeabi l i ty that,
apart from i ts theoreti cal and sci enti fi c i nterest, i s al so of cl i ni cal si gni fi cance. Duri ng l ocal
therapy wi th mouthwashes and si mi l ar preparati ons, drugs may be transported across the
oral mucosa and may exert eff ects si mi l ar to those resul ti ng from systemi c therapy. Thi s i s
P. 6
an i mportant factor when consi deri ng the use of hi gh-concentrati on steroi d mouthwashes for
ul cerati ve l esi ons of the oral mucosa. The permeabi l i ty of the oral mucosa i s uti l i zed i n the
treatment of angi na by gl yceryl tri ni trate. In these ci rcumstances rapi d absorpti on of the
drug throughout the oral mucosa i s an obvi ous advantage.
The oral mucosa has an i mportant barri er functi on.
Al though the f ul l si gni fi cance of the rol e of sal i va i n mai ntai ni ng the heal th of the oral
mucosa i s, as yet, not ful l y understood, there can be no doubt that a f ree sal i vary fl ow i s an
essenti al part of the oral envi ronment. If the f l ow i s di mi ni shed, ei ther by degenerati ve
changes i n the sal i vary gl ands or by the acti on of drugs, soreness and atrophi c changes i n
some areas of the oral mucosa rapi dl y fol l ow. The tongue i s perhaps most markedl y affected
i n thi s way. In some condi ti ons (f or exampl e, Sj gren' s syndromeChapter 8), i t i s
di f fi cul t to di sti ngui sh between pri mary mucosal changes and those secondary to di mi ni shed
sal i vary f l ow, but on a cl i ni cal basi s i t i s reasonabl e to accept that atrophi c changes i n the
oral epi thel i um are regul arl y associ ated wi th dryness of the mouth. The mai n i mmunol ogi cal l y
acti ve component of sal i va i s IgA, whi ch i s present i n a much hi gher rati o to other
i mmunogl obi ns, than i n the serum.
A further component to be consi dered as part of the normal heal thy oral envi ronment i s the
mi crobi al fl ora of the mouth. A wi de range of mi cro-organi sms may be present i n the oral
cavi ty, l i vi ng i n a commensal rel ati onshi p wi th the host. When thi s rel ati onshi p i s upset by a
change i n the l ocal or general i zed condi ti ons, the commensal mi cro-organi sms may become
pathogeni c. Oral candi dosi s i s an exampl e of thi s, al though the preci se change i n the host
l eadi ng to cl i ni cal i nfecti on i s not easy to i dentify. The aeti ol ogi cal factors for some common
oral i nf ecti ons are di scussed i n Chapter 4.
A wi de range of mi cro-organi sms may be present i n the oral cavi ty, exi sti ng i n a commensal
rel ati onshi p wi th the host.
Age changes
Changes occur i n the oral mucosa of heal thy i ndi vi dual s wi th i ncreasi ng age. Reducti ons i n
overal l epi thel i al thi ckness, fl exi bi l i ty of the col l agen f i bres, i nnervati on, bl ood suppl y, and
permeabi l i ty of the mucosa have been descri bed. It has general l y been assumed that changes
of thi s ki nd occur wi th comparati ve suddenness at a l ate age, but recent evi dence i mpl i es
that, at l east i n the case of the epi thel i um of the tongue, a conti nuous trend towards atrophy
occurs throughout adul t l i f e. The cl i ni cal si gni fi cance of thi s observati on i s that sudden
changes i n the structure of the oral mucosa, such as depapi l l ati on of the tongue, shoul d
never be consi dered as bei ng due to age al one wi thout f ul l i nvesti gati on and the el i mi nati on
of other factors such as haematol ogi cal and nutri tional di sorders (see Chapter 13).
Age changes may al so af fect the functi on of sal i vary gl ands. It has been shown that
gradual l y i ncreasi ng degrees of atrophy and fi brosis affect the secretory uni ts of both the
submandi bul ar and the l abi al sal i vary gl ands throughout l i f e, even i n the absence of di sease
processes that mi ght be associ ated wi th such change.
Abnormal oral mucosa
Many oral l esi ons represent the end resul t of breakdown or abnormal i ty of the normal
structuri ng of the epi thel i um. Vari ati on i n the rate of kerati n formati on, di sproporti on
between the di fferent l ayers of the cel l s, breakdown of the normal i ntercel l ul ar bonds of the
pri ckl e cel l s, spl i tti ng of the epi thel i um from the connecti ve ti ssue, and many other si mi l ar
abnormal i ti es may occur i n di f ferent di seases. For i nstance, i n a number of mucosal
abnormal i ti es hyperkeratosi s occurs (Fi g. 1. 4). This may ari se as a resul t of abnormal
i rri tati on of the mucosa or apparentl y spontaneously i n some condi ti ons. In other l esi ons,
atrophy of the epi thel i um may occur. Thi s represents a thi nni ng of the normal epi thel i al
l ayer, perhaps to onl y a few l ayers of cel l s, often accompani ed by i ncompl ete kerati ni zati on
(Fi g. 1. 5). Such

epi thel i um i s easi l y l ost fol l owi ng a mi nor degree of trauma and thus atrophi c l esi ons of the
mucosa readi l y become ul cerated. Many of the so-cal l ed erosi ve l esi ons are of thi s
type. It shoul d be remembered that ul cerati on i s i n i tsel f a qui te unspeci f i c process and
i mpl i es onl y the l oss of epi thel i um f rom the mucosal surf ace fol l owed by i nf l ammatory
changes i n exposed connecti ve ti ssue. Bul l ae or bl isters of the mucosa may occur i n one of
two ways, ei ther by degenerati on of the cel l s and of the i ntercel l ul ar l i nks i n the pri ckl e cel l
l ayer of the epi thel i um (Fi g. 1. 6) or by separati on of the whol e of the epi thel i um from the
underl yi ng cori um (Fi g. 1. 7). Frequentl y, there are al so changes i n the supporti ng ti ssues
and, i n some cases, the vi si bl e epi thel i al changes may be secondary to changes i n the
underl yi ng cori um that aff ect the nutri ti on and metabol i sm of the epi thel i um. The greatest
practi cal si gni fi cance of thi s fact i s, perhaps, the necessi ty, when taki ng a bi opsy of l esi ons
of the oral mucosa, to i ncl ude a representati ve thi ckness of cori um i n the ti ssue removed for
mi croscopi c exami nati on. In many cases, a bi opsy consi sti ng l argel y of epi thel i um al one i s
vi rtual l y usel ess for di agnosi s.
P. 7
Fig. 1.4 Hyperkeratoti c epi thel i um i n an al veol ar mucosal l esi on.
The i ntegri ty of the oral mucosa i s mai ntai ned by a compl ex of i nteracti ng f actors
superi mposed on the l ocal i zed stabi l i zi ng mechani sms di scussed above. The general hormonal
status of the pati ent and a number of nutri ti onal and metabol i c factors are i nvol ved i n
mai ntai ni ng the cel l metabol i sm and the ordered structure of the mucous membranes. The
rol e of i ron metabol i sm i n the mai ntenance of the structure of the oral mucosa has been the
subject of much i nvesti gati on. It i s certai nl y the case that i ron defi ci ency, even when
rel ati vel y mi l d i n cl i ni cal terms, can resul t i n general i zed oral epi thel i al atrophy and l oss of
the papi l l ary pattern of the l i ngual mucosa. It seems that other def i ci enci es that mi ght aff ect
Fig. 1.5 Atrophi c epi thel i um from the dorsum of the tonguethe normal compl ex
structure i s enti rel y l ost.
Fig. 1.6 An i ntra-epi thel i al bul l a formed as a resul t of l oss of i ntracel l ul ar cohesi on i n
the pri ckl e cel l l ayer.
i ron metabol i sm and erythrocyte producti on, such as fol ate and vi tami n B
12
defi ci enci es, may
al so contri bute to thi s destabi l i zati on of the oral epi thel i um. Thi s wi l l be di scussed at greater
l ength i n Chapter 13. If any si ngl e factor i s di sturbed, then sequenti al changes occur and
cl i ni cal l y si gni fi cant abnormal i ti es of the oral mucosa may fol l ow. It i s often di ff i cul t to
deci de whi ch of the vari ous possi bl e factors are i nvol ved i n i ni ti ati ng these changesthese
may evi dentl y occur ei ther as a pri mary mani f estati on of l ocal i zed mucosal abnormal i ty or as
a secondary ef fect of general i zed di sease processes. It i s the rol e of oral physi ci ans to assess
the possi bl e aeti ol ogi cal factors associ ated wi th mucosal l esi ons of thi s ki nd and to ensure
appropri ate i nvesti gati ons and (i f needs be) management.
The reacti ons of the oral mucosa are not excl usi vely those of a mucous membrane. As has
been poi nted out, a number of di seases of the ski n al so fi nd expressi on i n oral l esi ons. Thi s
i s not enti rel y surpri si ng on anatomi cal grounds si nce the l arger part of the oral mucosa i s
deri ved from an embryoni c i nvagi nati on that carri es i nwards some of the precursor epi thel i al
cel l s f rom whi ch both faci al ski n and oral mucosa are devel oped. As mi ght be expected, the
l esi ons of oral mucosa and ski n that occur i n these mucocutaneous di seases are of ten
superfi ci al l y di fferent, al though the basi c hi stol ogi cal changes seen i n the ti ssues are si mi l ar.
Such di ff erences are seen i n the pri mary l esi ons and, presumabl y, depend on the di ff erences
between the structure of the mouth and that of the ski n. Qui te often secondary changes al so
occur i n oral l esi ons. The conti nual l y damp envi ronment of the mouth, i n combi nati on wi th
repeated mi l d trauma of the ti ssues by teeth and f oodstuf fs, and the presence of a wi de
range of mi crobi al fl ora further modi fy the nature of the l esi ons produced i n a number of

di seases. For i nstance, shoul d the epi thel i um be thi nned by atrophy or weakened by the
formati on of bl i sters, i t i s l i kel y to be l ost and the i ni ti al l esi on be repl aced by an ul cer. For
reasons such as these, oral l esi ons, parti cul arl y at an advanced stage, may show f eatures
l ess characteri sti c than those of the equi val ent ski n l esi ons of the same di sease. Cl i ni cal
di agnosi s i n such ci rcumstances may be qui te di ff i cul t si nce onl y areas of ul cerati on of a
rel ati vel y non-speci f i c nature may be present rather than ful l y devel oped speci fi c l esi ons.
Histopathological changes
It may be hel pful to recal l some of the terms used to descri be changes seen on hi stol ogi cal
Fig. 1.7 A subepi thel i al bul l a formed by the separati on of the enti re epi thel i al l ayer
(i ncl udi ng the basal cel l l ayer) f rom the underl yi ng cori um.
P. 8
study of the oral mucosa.
Hyperkeratosi s: an i ncrease i n the thi ckness of the kerati n l ayer of the epi thel i um, or
the presence of such a l ayer i n a si te where none woul d normal l y be expected (Fi g.
1. 4). Hyperorthokeratosi s i s the term used to speci fy a thi ckened, compl etel y
kerati ni zed l ayer, whereas i n hyperparakeratosi s there i s i ncompl ete kerati ni zati on wi th
nucl ei remai ni ng i n the surface cel l s.
Acanthosi s: an i ncrease i n thi ckness of the pri ckl e cel l l ayer of the epi thel i um. Thi s may
or may not be accompani ed by hyperkeratosi s.
Atrophy: a decrease i n the thi ckness of the epi thel i um (Fi g. 1. 5).
Oedema: the col l ecti on of fl ui d i n or between the pri ckl e cel l s, i ntra- or i ntercel l ul ar,
the two forms often occurri ng si mul taneousl y. Oedema may al so occur between the
epi thel i um and the cori um i n the regi on of the basal compl ex.
Acanthol ysi s: l oss of the i ntercel l ul ar attachments i n the pri ckl e cel l l ayer l eadi ng to
separati on of the cel l s. When associ ated wi th i ntercel l ul ar oedema thi s l eads to the
producti on of i ntra-epi thel i al bul l ae or bl i sters (Fi g. 1. 6).
Atypi a: a term used to descri be vari ati ons i n the maturati on of the epi thel i al cel l s that
may be associ ated wi th mal i gnancy or premal i gnant potenti al . Such f eatures as
abnormal mi toses and l ack of normal structure of the epi thel i um are taken i nto account
i n the assessment of atypi a.
The oral mucosa in generalized disease
Oral l esi ons may occur i n a wi de vari ety of generali zed di seases. Thi s fact i s i mportant, not
onl y because of the need to treat the of ten pai nful oral l esi ons, but al so i n vi ew of thei r
si gni f i cance i n provi di ng a di agnosti c i ndi cator. The mouth i s readi l y avai l abl e for i nspecti on
(and for bi opsy) and oral l esi ons may appear earl y i n some di seases. Thus, a number of
i mportant condi ti ons may be f i rst di agnosed fol l owing the proper eval uati on of oral l esi ons.
The rel ati onshi p between general i zed and oral di sease i s a very compl ex one, but i t may be
hel pful to i denti f y three types of such i nterrel ationshi ps (Tabl e 1. 1).
Cl assi fi cati on of the oral general di sease rel ationshi ps i s di ff i cul t and may resul t i n
oversi mpl i f i cati on. It i s evi dent that more than one form of associ ati on mi ght occur i n a
si ngl e case. For i nstance, a pati ent wi th a di sease of the l ower gastroi ntesti nal tract mi ght
wel l produce pri mary l esi ons of the di sease on the oral mucosa, as wel l as secondary mucosal
change consequent to mal absorpti on.
The periodontium in generalized disease
Al though gi ngi val changes may occur i n some generali zed condi ti ons, the gi ngi vae are
usual l y not parti cul arl y good di agnosti c i ndi cators of the di sease process i nvol vedthe
changes are often cl i ni cal l y non-speci f i c. The hi stol ogi cal changes i n abnormal gi ngi vae are
al so often very di ff i cul t to i nterpret. A pre-exi sti ng i nfl ammatory cel l i nfi l trate and, often,
secondary i nf l ammatory features conf use the pi cture and make gi ngi val bi opsi es much l ess
useful than mi ght be expected. If the al ternati ve exi sts between taki ng a gi ngi val bi opsy or
one from another area (for exampl e, the buccal mucosa), the gi ngi val si te i s best avoi ded.
A few systemi c condi ti ons have been descri bed i n whi ch cl i ni cal l y recogni zabl e gi ngi val
changes occur. For i nstance, i n Wegener' s granul omatosi s, an uncommon vascul i ti c di sease
wi th wi despread systemi c i mpl i cati ons, a qui te characteri sti c gi ngi val pi cture has been
descri bed al though i t certai nl y does not occur i n al l cases. In Wegener' s granul omatosi s, the
gi ngi val l esi on may be an i ni ti al si gn of the di sease. Cl i ni cal l y, the gi ngi val surf ace

appears granul ar and f l ecked wi th yel l ow, an appearance resembl i ng that of over-ri pe
strawberri es. The prognosi s of Wegener' s granul omatosi s, i f untreated, i s poor and
theref ore earl y recogni ti on of the gi ngi val l esi on i s i mportant.
In Wegener' s granul omatosi s, the gi ngi val l esi on may be an i ni ti al si gn of the di sease.
Cl i ni cal l y, the gi ngi val surface appears granul ar and f l ecked wi th yel l ow, an appearance
resembl i ng that of over-ri pe strawberri es.
Such speci fi c and recogni zabl e changes are rare compared to the non-speci f i c gi ngi val
changes that may occur i n other di sease processes and that represent an accentuati on or
modi fi cati on of the wi despread changes of chroni c peri odontal di sease occurri ng i n a l arge
Table 1.1 Interrelationships between generalized and
oral disease
In one group of condi ti ons the oral l esi ons are si mi l ar i n aeti ol ogy and
hi stol ogy to those found el sewhere i n the body, being modi fi ed onl y by the
oral envi ronment. Many of the oral l esi ons of ski n di seases fal l i nto thi s group
as wel l as those i n some gastroi ntesti nal condi ti ons. These are di scussed i n
Chapters 11 and 12.
A second group of oral l esi ons resul ts f rom changes i n the metabol i sm of the
ti ssue under the i nfl uence of abnormal i ti es of nutri ti on, endocri ne, and other
factors (Chapter 13). These abnormal i ti es themsel ves are the resul t of some
di stant pathol ogi cal process. The oral l esi ons associ ated wi th mal absorpti on
fal l i nto thi s group.
In thi s group both oral and more general i zed l esi ons resul t from a systemi c
(and di fferent) abnormal i ty. Sjgren' s syndrome, a mani festati on of a
general i zed autoi mmune di sease resul ti ng i n sal i vary gl and hypof uncti on, i s
an exampl e of such an associ ati on. Thi s i s further di scussed i n Chapter 8.
P. 9
proporti on of the popul ati on. For exampl e, pati ents wi th severe, untreated di abetes mel l i tus
may devel op a rapi dl y destructi ve peri odontal di sease. In the mucocutaneous di seases, such
as l i chen pl anus and pemphi goi d, the gi ngi val changes are the resul t of a col l i si on
l esi on between the general i zed condi ti on and pre-exi sti ng or superi mposed changes of
chroni c gi ngi vi ti s. The non-speci f i c nature of the resul ti ng gi ngi val changes i s i l l ustrated by
the adopti on of the term desquamati ve gi ngi vi ti s, used to descri be the reacti on of
the gi ngi vae i n both these and other condi ti ons (Fi g. 1. 8). Gi ngi val l esi ons i n di seases of the
ski n are di scussed i n Chapter 11 and endocri ne-i nduced changes i n Chapter 13.
Projects
1. Di scuss the f uncti ons of the basement membrane i n the oral epi thel i um.
2. Descri be the di fferent zones wi thi n the basement membrane. Whi ch protei ns are known
to become target anti gens i n the acqui red bl i steri ng di seases? (see Chapter 11).
Fig. 1.8 Desquamati ve gi ngi vi ti s. The defi ni ti ve di agnosis was of l i chen pl anus.
> Tabl e of Cont ent s > 2 - Pr incipl es of or al medi ci ne: assessment and i nvest i gat i on of pat i ent s
2
Principles of oral medicine: assessment and
investigation of patients
The speciality of oral medicine
Oral medi ci ne i s general l y understood as bei ng the study and non-surgi cal treatment of the
di seases aff ecti ng the orof aci al ti ssues, especi al ly the oral mucous membrane, but al so other
associ ated ti ssues and structures such as the sal i vary gl ands, bone, and the faci al ti ssues.
Oral medi ci ne i s predomi nantl y an out-pati ent special i ty. The boundari es of oral medi ci ne are
poorl y defi ned. For i nstance, the i nvesti gati on of faci al pai n and other neurol ogi cal
di sturbances may be consi dered to be i n the fi el d of oral medi ci ne or of oral surgery. It i s the
responsi bi l i ty of the general dental practi ti oner to di agnose and manage some of these
condi ti ons. Others are often better treated i n speci al i st cl i ni cs, but the general dental
practi ti oner, to a very great extent, bears the responsi bi l i ty for the recogni ti on of oral
di sease at an earl y stage.
Definition of oral medicine
Oral medi ci ne i s that area of speci al competence concerned wi th the heal th of and wi th
di seases i nvol vi ng the oral and panoral structures. It i ncl udes those pri nci pl es of medi ci ne
that rel ate to the mouth, as wel l as research i n biol ogi cal , pathol ogi cal , and cl i ni cal spheres.
Oral medi ci ne i ncl udes the di agnosi s and medi cal management of di seases speci fi c to the
orof aci al ti ssues and of oral mani f estati ons of systemi c di seases. It further i ncl udes the
management of behavi oural di sorders and the oral and dental treatment of medi cal l y
compromi sed pati ents.
Proposed by the Worl d Workshop on Oral Medi ci ne, hel d i n Chi cago, USA, 1998
The devel opment of the di sci pl i ne of oral medi ci ne has depended l argel y on the adopti on of
an anal yti cal approach based on the appl i cati on of fundamental pri nci pl es. It fol l ows that the
practi ce of oral medi ci ne as a speci al i ty depends largel y on the avai l abi l i ty of di agnosti c
faci l i ti es, often greater than those avai l abl e to the general dental or medi cal practi ti oner.
Perhaps the most i mportant rol e of those worki ng i n the f i el d of oral medi ci ne i s i n the
recogni ti on of changes i n the oral cavi ty resul ti ng from general i zed di sease processes. Many
oral l esi ons that, i n the past, were consi dered to be of enti rel y l ocal ori gi n are now known to
be associ ated wi th systemi c abnormal i ti es. For thi s reason speci al i sts i n oral medi ci ne have a
cl ose worki ng rel ati onshi p wi th a l arge number of medi cal and surgi cal speci al i ti es. The most
potent f actor i n the expansi on of the scope of oral medi ci ne was the change of emphasi s
from the purel y descri pti ve to the i nvesti gati ve. The modern concept of the subject i mpl i es a
recogni ti on of basi c aeti ol ogi cal f actors, of the hi stopathol ogi cal and mol ecul ar changes
occurri ng i n the i nvol ved ti ssues, and of the si gni fi cance of such matters as the general
medi cal status of pati ents. The chal l enge for the future of the speci al i ty i s to devel op
evi dence-based management protocol s.
Patient assessment
History taking
The basi s of any i nvesti gati on i s a caref ul and detai l ed cl i ni cal hi story and exami nati on. The
pati ent shoul d be al l owed to descri be thei r compl aint(s) and concerns i n thei r own words. It
i s, however, often necessary to ask the pati ent f or more preci se or detai l ed i nf ormati on. The
speci f i c questi ons asked wi l l depend upon the presenti ng compl ai nt and thi s wi l l be
addressed i n the appropri ate chapters. Regardl ess of the orofaci al condi ti on that the pati ent
has, i t i s i mportant that the cl i ni ci an, when questi oni ng the pati ent, does not try to i nfl uence
the pati ent' s response. In addi ti on, the pati ents must not feel as i f they are bei ng hurri ed.
Sensi ti vi ty may be especi al l y requi red for some condi ti ons. As wi th al l pati ent care,
confi denti al i ty i s of the utmost i mportance.
It i s of great i mportance to obtai n detai l s of the medi cal hi story of the pati ent and of any
current or recent drug therapy. Si mi l arl y, the pati ent shoul d be asked at thi s stage about
thei r use of al cohol and tobacco. Some peopl e are poor hi stori ans wi th regard to thei r
medi cal hi story and i t i s theref ore of ten necessary to ask the pati ent' s general medi cal
practi ti oner to fi l l i n the detai l s. Thi s i s parti cul arl y rel evant when a pati ent has a chroni c
condi ti on that has been managed by several speci al ists. The correspondence that the general
medi cal practi ti oner has concerni ng such a pati ent can gi ve great i nsi ght i nto the pati ent' s
compl ai nt and care. In the hospi tal envi ronment, the request for and careful readi ng through
of the pati ent' s general hospi tal case

sheet can be, by far, the most producti ve method of assessment i n compl i cated cases. When
deal i ng wi th the past medi cal hi story i t i s necessary to use di rect questi oni ng on some
poi nts. As an exampl e, sof t-ti ssue l esi ons of the mouth may be associ ated wi th ski n rashes,
eye and geni tal l esi ons. The connecti on wi th mouth l esi ons may seem qui te tenuous to the
pati ent who may very wel l f ai l to vol unteer i nformati on on these poi nts unl ess di rectl y asked.
Patient assessment: sources of helpful information
1. Hospi tal case notes
2. Rsum of medi cal and soci al hi story from general medi cal practi ti oner
3. Correspondence from other cl i ni ci ans i nvol ved i n cari ng for the pati ent
The examination
When exami ni ng the mouth, the whol e of the oral mucosa must be careful l y exami ned. Al l
removabl e appl i ances shoul d be taken out. The practi ti oner shoul d approach the exami nati on
of the pati ent i n a systemati c manner to ensure that al l the rel evant ti ssues have been seen.
The l i ps and cheeks must be gentl y retracted to di spl ay the ful l extent of the sul ci and the
tongue gentl y hel d wi th the ai d of a gauze napki n, and extended f orward and to each si de.
Care must be taken that the whol e of the fl oor of the mouth and undersurf ace of the tongue
i s seen. The posteri or part of the tongue, tonsi l l ar fauces, soft pal ate, and part of the
pharynx are exposed by gentl e pressure on the tongue and hel ped by phonati on of ah
by the pati ent. Thi s exami nati on of the oral mucosa must be combi ned wi th a careful
assessment of the other dental structures, f aci al skel eton, sal i vary gl ands, and sof t ti ssues
of the face and neck. A search for pal pabl e l ymph nodes shoul d be made, rememberi ng that
normal l ymph nodes are not detectabl e by si mpl e palpati on. Swel l i ngs of the l ymph nodes are
further di scussed i n Chapter 7. The assessment of neurol ogi cal abnormal i ti es i s di scussed i n
Chapter 15. Practi ti oners shoul d be fami l i ar wi th the tests requi red to assess gross functi on
of the crani al nerves, i n parti cul ar the fi f th and seventh crani al nerves.
The extraoral exami nati on may be extended to i ncl ude the general appearance and
demeanour of the pati ent. The eyes, scal p, neck, hands, and the ski n of the f ace and arms
shoul d usual l y be i nspected to obtai n si gni f i cant i nformati on (Tabl e 2. 1). Each of these
vi sual l y accessi bl e areas can demonstrate si gns that al ert the practi ti oner to possi bl e
P. 14
underl yi ng systemi c di sease.
The i ntraoral exami nati on shoul d take pl ace wi th an adequate l i ght source. Someti mes
ti ssues requi re dryi ng to enabl e thorough vi sual i zati onthi s i s parti cul arl y rel evant to
teeth. Occasi onal l y, i n pati ents wi th a parchment dry oral mucosa, i t i s hel pf ul to
al l ow the pati ent to ri nse wi th water before conti nui ng wi th the i ntraoral exami nati onthi s
wi l l hel p l ubri cate the soft ti ssues and prevent your gl oves or mi rror sti cki ng to and
traumati zi ng the ti ssues. Conversel y, i n some pati ents wi th copi ous vi scous sal i va, a
mucol yti c mouthwash may be hel pf ul pri or to i ntraoral exami nati on.
Table 2.1 Useful information from extraoral examination
Observation Information (examples of associated conditions)
General
demeanour,
appearance, and
manner
Wasted, undernouri shed, cachecti c appearance, (e. g.
mal nutri ti on, eati ng di sorder, underl yi ng mal i gnancy); l ow
mood, anxi ety, agi tati on (e.g. depressi on)
Breathl essness Cardi orespi ratory probl ems
Face Shape and symmetry (masseteri c hypertrophy, crani ofaci al
syndromes); Cushi ngoi d appearance (e. g. corti costeroi d
therapy); neurol ogi cal def i ci ts (e. g. Bel l ' s pal sy, crani al
tumours); cyanosi s (e. g. cardi orespi ratory di sease); pal l or
(e. g. anxi ous, unwel l , anaemi c)
Scal p and faci al
hai r
Scant hai r (e.g. ectodermal dyspl asi a)
Eyes Conjuncti val scarri ng (e. g. pemphi goi d, keratoconjuncti vi ti s
si cca); pal e scl era (anaemi a); yel l ow scl era (jaundi ce);
exophthal mi a (hyperthyroi di sm); xanthomas of peri ocul ar
ski n (hyperchol esterol aemi a)
Neck Enl arged l ymph nodes (oral i nfecti on, neopl asms); goi tre
Hands Raynaud' s phenomenon; koi l onychi a and other l esi ons of the
nai l s; fi ngers: joi nt swel l i ng and acqui red di sfi gurement
(rheumatoi d arthri ti s); Hebden' s nodul es (osteoarthrosi s);
pal mar keratosi s (Papi l l onLef vre syndrome); l i ver
pal ms; tobacco stai ni ng; fi nger cl ubbi ng (chroni c
cardi orespi ratory probl ems, i ncl udi ng i nfecti ve endocardi ti s)
Wri sts Purpl e papul es consi stent wi th l i chen pl anus
It i s essenti al that case note entri es are:
l egi bl e
contemporaneous
dated
si gned
It i s of paramount i mportance that al l the detai l s of the hi story and exami nati on of the
pati ent are comprehensi vel y recorded i n the pati ent' s records. It i s often hel pful to make a
sketch of some l esi ons i n the case notes. Thi s wi l l i ndi cate the shape and posi ti on of the
l esi on and hel p cl i ni ci ans at future appoi ntments. A topogram of the oral cavi ty(Fi g. 2. 1) i s
parti cul arl y useful for thi s purpose. A wel l taken cl i ni cal photograph can al so provi de a
val uabl e record of an orof aci al l esi on. It wi l l al so be useful f or teachi ng and essenti al i f a
case report i s to be submi tted

for publ i cati on. The pati ent shoul d be i nformed as to why photographs are requi red and the
pati ent' s permi ssi on i s al ways requi red. It i s essenti al to obtai n wri tten consent i f i t i s
envi saged that the photograph wi l l be used i n a publ i cati on.
Ski n Petechi ae or ecchymoses (e. g. bl ood dyscrasi a); cyanosi s
(cardi ac or pul monary i nsuf fi ci ency); j aundi ce; pi gmentati on
(possi bl e endocri ne probl ems)
P. 15
Investigations
A l arge number of di agnosti c tests and procedures must be avai l abl e to those worki ng i n the
fi el d of oral medi ci ne. Tabl e 2. 2 l i sts many of the i nvesti gati ons and di agnosti c tests used;
however, thi s l i st i s not exhausti ve. The advantages of cl ose associ ati on wi th speci al i zed
departments, such as those of cl i ni cal i mmunol ogy, haematol ogy, mi crobi ol ogy, cl i ni cal
chemi stry, gastroenterol ogy, and dermatol ogy, are evi dent, and vi rtual l y al l centres i n whi ch
oral medi ci ne cl i ni cs are successful l y conducted enjoy such associ ati ons. Al though the range
of i nvesti gati ons carri ed out i n the oral medi ci ne cl i ni c i tsel f may be wi de, i t i s evi dent that,
i n many i nstances, i t i s proper to refer the pati ent to a col l eague i n some other speci al i ty f or
subsequent i nvesti gati on f ol l owi ng i ni ti al di agnosis i n the oral medi ci ne cl i ni c. Some uni ts
wi l l have speci al i nterest or experti se i n certai n di seases and may be abl e to offer
sophi sti cated i nvesti gati ons and i ntegrated mul ti di sci pl i nary care that i s not routi nel y
avai l abl e i n al l oral medi ci ne cl i ni cs.
The i mportance of taki ng a pati ent' s temperature when there i s a
di ssemi nated i nfecti on shoul d not go unmenti oned. Thi s wi l l i ndi cate i f there
are any systemi c ef fects from the i nfecti on.
Speci al i nvesti gati ons wi l l be di scussed subsequentl y, i n the appropri ate chapters and i n
rel ati on to the subject of the i nvesti gati on. However, i t i s hel pful to present an overvi ew of
the tests i n thi s secti on. It shoul d be remembered that very f ew speci al i nvesti gati ons
provi de a defi ni ti ve di agnosi s. It i s more usual for the resul ts of several i nvesti gati ons to be
combi ned wi th the cl i ni cal hi story and presentati on before a defi ni ti ve di agnosi s can be

madethi s i s exempl i f i ed by the di agnosti c cri teri a for Sj gren' s syndrome. The most
common l aboratory i nvesti gati on requested i n the oral medi ci ne cl i ni c i s a screeni ng
procedure f or possi bl e bl ood abnormal i ti es. In vi ew of i ts wi despread appl i cati on thi s meri ts
prel i mi nary di scussi on.
Fig. 2.1 A topogram of the oral cavi ty. (Reproduced wi th the permi ssi on of Zi l a
Europe. )
P. 16
Blood examination
The assessment of oral medi ci ne pati ents of ten i nvol ves haematol ogi cal , bi ochemi cal , and
serol ogi cal i nvesti gati ons. These tests may be done ei ther as screeni ng tests to hel p i n the
di agnosi s of some uni denti fi ed condi ti on or to confi rm a di agnosi s by the appl i cati on of
speci f i c tests. In the f i rst i nstance i t may be helpful for the reader to categori ze tests
generi cal l yaccordi ng to the department that performs the i nvesti gati on.
Table 2.2 What to consider in patients assessment
History Investigations
Compl ai nt and hi story Haematol ogy
Ful l medi cal hi story Cl i ni cal Chemi stry
Speci f i c questi ons Immunol ogy
Ski n i nvol vement Endocri ne studi es
Eye probl ems Uri nal ysi s
Geni tal symptoms Bi opsy
Gut probl ems Mi crobi ol ogy
Drug hi story Imagi ng
Soci al hi story (l i festyl e) Pl ai n radi ographs
Tobacco Sal i vary gl and i magi ng
Al cohol CT scan
MR scan
Ul trasound
Clinical examination Bl ood pressure
Extra-oral Body Temperature
Intra-oral Bi ochemi stry
Haematology
As a screeni ng procedure for possi bl e haematol ogi cal abnormal i ty, a haemogl obi n esti mati on,
whi te cel l count, and bl ood f i l m exami nati on were once consi dered to be suff i ci ent. Oral si gns
and symptoms may, however, accompany rel ati vel y mi nor changes i n the bl ood and oral
l esi ons may occur earl y i n pati ents wi th haematol ogi cal abnormal i ti es, wel l before these are
shown up by the si mpl e exami nati on of peri pheral blood. A si gni fi cant l oweri ng of serum or
red cel l f ol ate l evel s or (l ess f requentl y) l owered serum B
12
l evel s may occur i n the absence
of any detectabl e change i n the peri pheral erythrocytes, parti cul arl y i n pati ents wi th
stomati ti s and recurrent ul cerati on. It theref ore fol l ows that the si mpl e ful l bl ood count i s an
i nsuf fi ci ent procedure for the i ni ti al i nvesti gati on of such pati ents. Haematol ogy reports (and
al so those of other i nvesti gati ons) shoul d be i nterpreted wi th the ai d of the normal val ues of
the l aboratory i nvol ved. The fi gures quoted i n Table 2. 3 must be regarded as a gui del i ne
onl y, parti cul arl y i n rel ati on to borderl i ne val ues. In the case of borderl i ne resul ts of cl i ni cal
si gni f i cance i t i s recommended that a second sampl e shoul d be taken i f at al l possi bl e,
l aboratory f i gures are subject to some degree of vari ati on. It must be repeated that such
resul ts shoul d al ways be i nterpreted i n the l i ght of the age of the pati ent and the normal
val ues gi ven by the l aboratory i nvol ved.
Table 2.3 Representative laboratory normal values for
haematological tests
Test Normal value*
Haemogl obi n 12. 517. 5 g/dl (mal e);
11. 516. 0 g/dl (femal e)
Mean Cel l Vol ume 8095 fl
ESR 015 mm/h
Red cel l count 46 10
12
/l (mal es); 45
10
12
/l (femal es)
Whi te bl ood cel l count 410 10
9
/l
Haematocri t 4050 l /l (mal es): 3447 l /l
(femal es)
Pl atel ets 150400 10
9
/l
Serum Fol ate 1. 55. 5 g/l
Red cel l Fol ate 125600 g/l
In Tabl e 2. 4 the meani ngs of some of the terms used to descri be vari ati ons from normal i n
the si ze and shape of erythrocytes are gi ven, together wi th an i ndi cati on of some of the
condi ti ons i n whi ch these forms may occur.
Haematol ogi cal i nvesti gati ons are hel pf ul i n the diagnosi s of the fol l owi ng condi ti ons:
Leukopeni as
Anaemi as
Thrombocytopeni as
Inf ecti ous mononucl eosi s
Myel omas
Pol ycythaemi as
Leukaemi as
Serum B
12
170590 g/l
* Al l resul ts shoul d be compared to the normal
val ues of the testi ng l aboratory.
Table 2.4 Variation in size and shape of erythrocytes
Description Erythrocyte
characteristics
Seen in
Hypochromi c Pal e stai ni ng Iron defi ci ency anaemi a
Hyperchromi c Dense stai ni ng Perni ci ous anaemi a
Mi crocyti c Smal l Iron defi ci ency anaemi a
Macrocyti c Large (1012 ) Perni ci ous anaemi a
Megal ocyti c Very l arge (1225
)
Perni ci ous anaemi a
A si gni f i cant proporti on of pati ents attendi ng the oral medi ci ne cl i ni c wi l l requi re ful l
haematol ogi cal screeni ng. It i s suggested that the fol l owi ng groups warrant thi s extended
screeni ng procedure (see al so Chapter 13):
pati ents wi th recurrent apthous stomati ti s

pati ents wi th a persi stentl y sore and/or dry mouth.
pati ents wi th oral l esi ons wi th an atypi cal hi story or unusual l y resi stant to treatment;
pati ents compl ai ni ng of a sore or burni ng mouth or tongue, or abnormal taste
sensati on, even though no mucosal changes can be seen;
al l pati ents wi th persi stent orofaci al candi dosi s.
Pati ents showi ng abnormal i ti es fol l owi ng an i ni ti al screeni ng (i e haemogl obi n and ful l
bl ood count).
Ani socytosi s Much vari ati on i n si ze Most anaemi as
Poi ki l ocytosi s Much vari ati on i n
shape
Most anaemi as
Spherocyti c Spheri cal Congeni tal haemol yti c anaemi as
Target cel l
(l eptocytes)
Concentri cal l y stai ned Any chroni c anaemi a
Erythrobl ast Nucl eated Denotes excessi ve erythropoi esi s
Normobl ast Normal si ze Af ter haemorrhage, very severe
anaemi as and l eukaemi as
Mi crobl ast
Megal obl ast
Smal l
Large
Perni ci ous anaemi a, carci noma
stomach, af ter total gastrectomy
Reti cul ocyte Reti cul ated when
stai ned wi th vi tal
stai ns
If hi gher than 1% i n adul tsan
acti ve marrow response to a
demand f or erythrocytes
P. 17
Table 2.5 Useful biochemical investigations
Investigation Description
Gl ucose Rai sed i n di abetes mel l i tus, Cushi ng' s syndrome
Hypogl ycaemi a occurs most commonl y i n di abeti c pati ents and
may occur i n severe l i ver di sease
Urea Rai sed i n dehydrati on, renal fai l ure
Creati ni ne Rai sed i n renal fai l ure
El ectrol ytes
Sodi um El evated i n dehydrati on
Low i n condi ti ons causi ng overhydrati on, e. g. cardiac fai l ure
Potassi um Rai sed i n renal fai l ure, di abeti c ketoaci dosi s
Hyperkal aemi a i s commonl y artefactual due to haemol ysi s,
del ayed speci mens, or ethyl enedi ami ne tetraaceti c aci d (EDTA)
contami nati on
Hyperkal aemi a i s a medi cal emergency and must be corrected
i mmedi atel y
Hypokal aemi a i s commonl y due to di ureti cs or gastroi ntesti nal
l osses of potassi um
Cal ci um Hi gh i n pri mary hyperparathyroi di sm, mal i gnancy, vi tami n D
excess
Low i n ri ckets, osteomal aci a, hypoparathyroi di sm
Phosphate Hi gh i n renal fai l ure
Low i n ri ckets / osteomal aci a
Al kal i ne
phosphatase
Rai sed i n condi ti ons wi th i ncreased bone turnover such as
Paget' s di sease, ri ckets/osteomal aci a
Al so rai sed i n l i ver di sease, parti cul arl y chol estasi s
Total protei n Rai sed i n dehydrati on, l i ver di sease, myel oma, connecti ve
ti ssue di seases, and sarcoi dosi s
Reduced i n overhydrati on, enteropathy, renal fai l ure
Al bumi n Rai sed i n dehydrati on
Reduced when there i s an acute phase response, e. g.
i nfl ammati on, postoperati vel y, carci noma
Al so rai sed i n severe l i ver di sease, mal absorpti on, nephroti c
syndrome, connecti ve ti ssue di seases
Ferri ti n Rai sed i n l i ver di sease, haemachromatosi s, l eukaemi a
Si nce these groups consti tute a si gni f i cant proporti on of pati ents attendi ng the oral medi ci ne
cl i ni c, the above screen i s al most a routi ne procedure i n thi s envi ronment.
Clinical chemistry
There are several bi ochemi cal i nvesti gati ons that are f requentl y requested for pati ents
presenti ng wi th oral medi ci ne probl ems and several of these are gi ven i n Tabl e 2. 5.
Pl asma gl ucose measurements are requi red to di agnose di abetes mel l i tus (DM) and i n the
moni tori ng of thi s seri ous condi ti on. The di agnosti c cri teri a for the di agnosi s of DM were
al tered i n 1999 and are as fol l ows: symptoms pl us random pl asma gl ucose >11. 1 mmol /l ;
repeated random bl ood gl ucose >11. 1 mmol /l i n asymptomati c i ndi vi dual s; or fasti ng pl asma
gl ucose >7. 0 mmol /l . Formal gl ucose tol erance tests are now performed l ess frequentl y than
previ ousl y and are requi red onl y i n compl i cated pati ents.
Immunological tests
A wi de range of i mmunol ogi cal tests i s avai l abl e to assi st i n the di agnosi s of di seases
aff ecti ng the oral cavi ty and many such tests form an essenti al part of the di agnosti c
processes of the oral medi ci ne cl i ni c. Tabl e 2. 6 highl i ghts some of the tests that may be
requested. Many i mmunol ogi cal l y based tests are now matters of routi ne. Others are onl y
avai l abl e i n speci al i st centres.

Reduced i n i ron defi ci ency anaemi a
Li ver enzymes Di sturbed i n l i ver di sease, some drug therapi es
Enzyme-i nduci ng drugs, e. g. carbamazepi ne, phenytoin,
phenobarbi tone, cause a mi l d el evati on i n al kal i ne phosphatase
and gl utaryl transf erase (GT)
P. 18
Table 2.6 Useful immunological tests
Autoanti bodi es
Rheumatoi d factor
Anti nucl ear factor
SS-A, SS-B anti bodi es
Pari etal cel l anti body
Anti -gl i aden
Anti -endomysi al
Epi thel i al i ntercel l ul ar cement
Epi thel i al basement membrane
C1 esterase i nhi bi tor (reduced i n heredi tary angi oedema)
Haematological screening protocol
When a screeni ng procedure i s deci ded upon, a reasonabl e scheme of i nvesti gati on i s as
fol l ows:
1. Ful l bl ood count and fi l m exami nati on. From thi s, evi dent anaemi as are demonstrated
by vari ati ons i n red cel l morphol ogy and l owered haemogl obi n val ues. Abnormal i ti es of
the whi te cel l s and pl atel et numbers are al so shown. Haematol ogi cal i ndi ces such as
the red and whi te bl ood cel l counts, the mean corpuscul ar haemogl obi n, mean
corpuscul ar vol ume, the haematocri t and pl atel et count are i mportant and abnormal
val ues may i ndi cate underl yi ng systemi c di sease.
2. Esti mati on of serum ferri ti n as a measure of ful l body i ron status. Thi s test has al most
enti rel y repl aced the esti mati ons of serum i ron, total i ron bi ndi ng capaci ty, and
saturati on formerl y used as a screeni ng test, al though these are sti l l used i n the
i nvesti gati on of compl ex i ron-def i ci ency states.
3. Serum B
12
, fol ate and red cel l fol ate esti mati ons. These are val uabl e i ndi cators of
mal absorpti on and, hence, of gastroi ntesti nal di seases of many ki nds. The red cel l
fol ate l evel i s a rel ati vel y stabl e i ndi cator of fol ate defi ci ency, whereas the serum
fol ate l evel s are more l abi l e and i ndi cate the current status. It has been shown that
these may show i ndependent cl i ni cal l y si gni f i cant vari ati on i n pati ents presenti ng wi th
oral si gns and symptoms. Coel i ac di sease i s an exampl e of a gastroi ntesti nal di sease
that can resul t i n mal absorpti on of haemati ni cs. Low f ol ate l evel s may al so be due to
anti convul sant drug therapy, pregnancy, and al cohol i sm.
A vi tami n B
12
defi ci ency shoul d be suspected i f there i s a macrocytosi si ndi cated by
a rai sed mean corpuscul ar vol ume and packed cel l vol ume.
4. As an addi ti onal test an erythrocyte sedi mentati on rate (ESR) measurement i s useful as
a non-speci f i c gui de to underl yi ng pathol ogi cal processesal ternati vel y measurement
of C-reacti ve protei n (CRP) may be used as a si mi l ar marker for pre-exi sti ng di sease
(see Cl i ni cal Immunol ogy). The ESR i s rai sed i n pregnancy, chroni c i nfl ammatory
condi ti ons, acute i nfecti on, gi ant cel l arteri ti s, and neopl asi a.
The assay of ci rcul ati ng autoanti bodi es i s an i mportant procedure i n the oral medi ci ne cl i ni c.
Some autoanti bodi es are cl osel y associ ated wi th speci fi c di sease processesfor i nstance,
gastri c pari etal cel l anti bodi es wi th perni ci ous anaemi a. In other autoi mmune di seases,
however, a wi de range of autoanti bodi es may be produced. For thi s reason i t i s usual to carry
out a range of autoanti body tests rather than a si ngl e one.
Di rect i mmunofl uorescent techni ques, for the detecti on of i mmunogl obul i ns and other
Vi ral anti bodi es
HIV
Epstei nBarr vi rus
C-reacti ve protei n (rai sed i n i nfl ammati on and mal i gnancy)
i mmunol ogi cal l y acti ve protei ns fi xed wi thi n ti ssue, have acqui red great i mportance i n the
di agnosi s of oral mucosal l esi onsparti cul arl y those associ ated wi th ski n di seases and
connecti ve ti ssue di sease. The pri nci pl e of the techni que depends on the fact that anti bodi es
combi ned wi th fl uorescei n retai n both thei r i mmunol ogi cal acti vi ty and the property of the
fl uorescei n to fl uoresce under ul travi ol et l i ght. The anti bodi es can, therefore, be l ocated at
the exact si te of combi nati on wi th thei r anti geni c antagoni sts by mi croscopi c observati on
under ul travi ol et i l l umi nati on. A wi de range of hi ghl y speci fi c anti bodi es to the vari ous
i mmunogl obul i ns and compl ement components i s avai l abl e and can be used to demonstrate
the type and si te of bound compl exes. In some conditi ons the resul ts may be hi ghl y speci fi c
and di agnosti c (i n pemphi gus and pemphi goi d, for exampl e). In other condi ti ons (such as
l i chen pl anus) they are not cl ear. The fi ndi ngs i n a number of condi ti ons are di scussed i n
Chapter 11. The speci al requi rements f or the taki ng of bi opsy materi al f or thi s techni que are
menti oned l ater i n thi s chapter.
Endocrine function
Endocri ne di sorders are i mportant i n oral medi ci ne for the fol l owi ng reasons.
Pati ents may present wi th a compl ai nt that l eads to the di agnosi s of an endocri ne
di sturbance. An exampl e i s a pati ent wi th oral dysaesthesi a or xerostomi a who upon
i nvesti gati on i s found to be an undi agnosed di abetic.

Poorl y control l ed hormonal i mbal ances may l ead to orofaci al di sease.
Therapy of orof aci al di sease may l ead to hormonal imbal ances. An exampl e of thi s i s
the use of systemi c steroi ds to treat severe ul cerati on or bul l ous di seasel ong-term
therapy wi l l i nduce adrenal suppressi on and predi sposes towards di abetes and
osteoporosi s.
Concurrent endocri ne di sease or hormone repl acement therapy may i nfl uence the
management of the pati ent.
Exampl es of hormone studi es that may be requested i n oral medi ci ne are thyroi d functi on
tests and parathormone, growth hormone, and corti sol l evel s.
Urinalysis
Uri nal ysi s, whi ch can be done qui ckl y and cost-eff ecti vel y, i s used to i denti f y the presence of
gl ucose, bl ood, protei ns, ketones, and bi l e products. Thi s can al ert the cl i ni ci an to the
possi bi l i ty of underl yi ng systemi c di sease and, therefore, further i nvesti gati ons wi l l be
requi red. Uri ne sampl es are usual l y col l ected from the mi dstream fl owavoi di ng the uri ne
fl ow at the begi nni ng and end of mi cturi ti on.
Biopsy
A bi opsy i nvol ves the removal of part or al l of a l esi on so that i t can be exami ned by
hi stopathol ogi cal techni ques.
Many l esi ons may be di agnosed onl y after exami nati on of an appropri ate bi opsy speci men of
the affected ti ssue. Thi s i s so, not onl y i n cases of suspected neopl asi a, but, for exampl e,
al so i n the di fferenti al di agnosi s of whi te patches that may occur i n the oral mucosa and of
the bul l ous, ul cerati ve, and desquamati ve l esi ons in the mouth. Many bone condi ti ons are,
si mi l arl y, di agnosed by exami nati on of a bi opsy sampl e. It i s general l y agreed that, i n the
case of suspected or possi bl e mal i gnancy of the oral mucosa, bi opsy i s mandatory and, wi th
si mpl e precauti ons, i s unl i kel y to cause di ssemi nati on of tumour cel l s. There are several
P. 19
methods of obtai ni ng bi opsi es and these wi l l be deal t wi th under the fol l owi ng headi ngs:
exci si onal , i nci si onal , and fi ne needl e aspi rati on. The deci si on to take an exci si onal or
i nci si onal bi opsy wi l l depend upon the nature of the l esi on, i ts si ze, and l ocati on.
Pri or to bi opsy the pati ent shoul d be i nformed about:
the reasons for the procedure
what to expect
any di scomf ort
possi bl e compl i cati ons
A pati ent i nformati on l eaf l et i s a hel pful ai d i n obtai ni ng i nformed consent.
Biopsy techniques
Inci si onal
Exci si onal
Fi ne needl e aspi rati on
Excisional biopsy
If the l esi on i n questi on i s smal l , i t may be best to remove i t enti rel y by l ocal exci si on,
i ncl udi ng a smal l area of normal ti ssue. The speci men may then be secti oned and i ts
hi stol ogy revi ewed to determi ne whether further treatment wi l l be needed. The bi opsy i s far
better taken wi th the kni fe than wi th the cutti ng di athermy, whi ch may cause consi derabl e
di storti on of the ti ssues. After i ts removal , the bi opsy speci men shoul d be pl aced wi th the
mi ni mum of del ay i nto a fi xati ve, 10 per cent formol sal i ne bei ng the most uni versal l y used.
Ful l cl i ni cal detai l s shoul d al ways be gi ven to the pathol ogi st who i s to exami ne the
speci men.
Exci si on bi opsy i s parti cul arl y usef ul for the di agnosi s of si ngl e smal l ul cers and smal l ,
l ocal i zed, sof t-ti ssue swel l i ngs. In these cases i t i s possi bl e to combi ne pri mary treatment
wi th bi opsy.
Incisional biopsy
Thi s i s the removal of a secti on of a l esi on for hi stol ogi cal study wi thout any attempt bei ng
made to remove the whol e of the l esi on. In taki ng such a bi opsy of the oral soft ti ssues, the
ai m shoul d be to i ncl ude wi thi n one speci men, i f possi bl e, a cl i ni cal l y typi cal area of the
l esi on and al so the edge of the l esi on. If the choice must be made between the two
possi bi l i ti es, the cl i ni cal l y typi cal area shoul d be chosena l arge area of normal ti ssue
beyond the l esi on i s qui te unnecessary. The speci men shoul d be bi g enough to al l ow the
pathol ogi st to make a di agnosi s, as too smal l a bi opsy i s di ffi cul t to handl e and to ori entate
for secti oni ng.
The techni que for bi opsy of a l esi on of the oral epi thel i um i s to make a wedge-shaped cut
i nto the chosen area, to compl ete the tri angl e by a thi rd cut, and to then take of f the
epi thel i al l ayer, together wi th a thi ckness of corium, by sl i di ng the kni f e bel ow and paral l el
wi th the surface. Even i f i t i s the epi thel i um that i s of parti cul ar i nterest, i t i s essenti al that
a suff i ci ent l ayer of cori um shoul d be i ncl uded i n order that the subepi thel i al reacti ons may
be seen. If the bi opsy i s of a l ump, then the wedge secti on must be taken i nto the swel l i ng,
maki ng sure that any capsul ar ti ssue i s cut through and that a representati ve area of the
l esi on proper i s obtai ned.
Anaesthesi a f or the bi opsy shoul d be obtai ned by the i njecti on of l ocal anaestheti c as f ar
from the bi opsy si te as consi stent wi th obtai ni ng a sati sfactory resul t. It i s cl earl y unwi se to
i nject di rectl y i nto an area of doubtf ul mal i gnancy and, qui te apart f rom any questi on of
di ssemi nati on of neopl asti c cel l s, there i s a danger of di storti on of the hi stol ogi cal pi cture i f
the area i s i nfi l trated by anaestheti c. The bi opsy si te may be cl osed by one or two sutures.

If the speci men i s a thi n one, as i s often the case wi th bi opsi es of the oral mucosa, i t i s often
most conveni ent to l ay i t f l at on a pi ece of card or a swab before pl aci ng i nto the fi xati ve.
The ti ssue practi cal l y al ways adheres to thi s backi ng, and curl i ng and di storti on of the
speci men i s prevented. Mul ti pl e bi opsi es may be requi red of l arge mucosal l esi ons or i n cases
wi th wi despread oral l esi ons that are cl i ni cal l y dissi mi l ar.
Frozen secti ons for rapi d di agnosi s are rarel y requi red i n oral medi ci ne. Very of ten the
hi stol ogi cal secti ons requi re careful and detai l ed study, thi s bei ng di f fi cul t wi th frozen ti ssue
speci mens. Rapi d reporti ng of frozen secti ons i n the urgent si tuati on i s possi bl e, for i nstance,
duri ng surgery when i t i s essenti al to i denti fy mal i gnancy, but thi s i s not a si tuati on rel evant
to the regul ar practi ce of oral medi ci ne. The major use of frozen secti ons i n oral medi ci ne i s
i n i mmunof l uorescent di agnosti c techni ques.
Biopsy for immunofluorescence
Anti genanti body compl exes i n ti ssues can be i denti fi ed by vari ous stai ni ng and l abel l i ng
techni ques. Fl uorescei n i s a commonl y used l abel . Di rect i mmunofl uorescent studi es of bi opsy
materi al have become an essenti al part of the di agnosti c procedure for i mmunobul l ous oral
l esi ons (as di scussed earl i er i n thi s chapter and i n the appropri ate l ater chapters). In the
case of non-bul l ous and non-erosi ve l esi ons there are no parti cul ar probl ems. The speci men
i s taken f rom the l esi onal ti ssue wi th some margi nal cl i ni cal l y normal ti ssue i f conveni ent. In
the case of bul l ous or erosi ve l esi ons, however, the si tuati on i s qui te di f ferent i n that the
most characteri sti c i mmunol ogi cal fi ndi ngs are l i kel y to be i n the cl i ni cal l y normal ti ssue
adjacent to the l esi on. When bul l a formati on or erosi on has occurred, the bi opsy techni que of
the l esi on i tsel f becomes very di ffi cul t and the resul ts (l argel y because of secondary i nfecti on
and si mi l ar f actors) become much more di ffi cul t to i nterpret.
It i s essenti al when taki ng bi opsy speci mens f or i mmunof l uorescent studi es that the
l aboratory shoul d be pre-warned so that the fresh, unfi xed ti ssue i s passed on di rectl y f or
i mmedi ate processi ng (or f or deep f rozen storage). It can be safel y transported i n a
pol ythene bag or pl aced on an i ce tray. Pri or to taki ng a bi opsy the practi ti oner shoul d deci de
what type of bi opsy i s requi red and what i s goi ng to happen to the bi opsy speci men. Thi s wi l l
prevent unnecessary repeat bi opsi es.
Both di rect and i ndi rect i mmunofl uorescent studi es are of val ue i n the di agnosi s of
i mmunobul l ous di seases (for exampl e, pemphi gus and pemphi goi d).
Fine needle aspiration (FNA) biopsy
Soft-ti ssue l esi ons can be col l ected for mi croscopic exami nati on usi ng a needl e aspi rate. Thi s
techni que i s al so useful for col l ecti ng fl ui d contents of a l esi on, parti cul arl y pus or cysti c
fl ui d. A 20/21 gauge needl e i s empl oyed f or sampl i ng ti ssue and ul trasound can be used to
gui de the posi ti oni ng of the needl e i n an attempt to ensure that the bi opsy i s taken f rom the
centre of the l esi on. Someti mes i t i s not possi bl e to obtai n a def i ni ti ve di agnosi s from a FNA
bi opsy. Often there i s enough i nformati on to di ff erenti ate between mal i gnant and beni gn. An
experi enced cytol ogi st i s requi red to i nterpret the sampl e and i t shoul d be remembered that
the speci men harvested may not al ways be representati ve of the l esi on.
Microbiological investigations
P. 20
Not al l orofaci al i nf ecti ons requi re the servi ces of a di agnosti c mi crobi ol ogy l aboratory.
However, when such servi ces are requi red, the cl i nici an needs to be aware of the range of
servi ces avai l abl e and the type of speci men requi red. Laboratori es can l ook f or the f ol l owi ng
evi dence of i nfecti on.
1. Vi abl e organi sms. Mi cro-organi sms can someti mes be seen mi croscopi cal l y f rom a di rect
smear but usual l y the speci men i s cul tured. Thi s wil l then al l ow the organi sms i n the
cul ture to be i denti fi ed and undergo sensi ti vi ty testi ng to anti mi crobi al agents. For
cul ture and sensi ti vi ty testi ng an aspi rate of pus i s preferabl e to a swab. The l atter
may be contami nated wi th normal oral fl ora and the putati ve pathogens are l ess l i kel y
to survi ve the journey to the l aboratory.
2. Mi crobi al products. It i s possi bl e to detect the presence of mi cro-organi sms by the
products that they produce, such as toxi ns, or by identi f yi ng thei r DNA. The appl i cati on
of mol ecul ar techni ques to i denti fy geneti c materi al has al l owed for the i denti fi cati on of
mi cro-organi sms wi thout the need for cul ture. Gene ampl i fi cati on usi ng the pol ymerase
chai n reacti on (PCR) and i n si tu hybri di zati on techni ques al l ows for the rapi d
i denti fi cati on of organi sms. Thi s i s parti cul arl y benefi ci al f or organi sms that are
hazardous or not easi l y grown i n the l aboratory. Hepati ti s C i s i denti f i ed i n thi s
manner.
3. Anti body detecti on. The presence of ci rcul ati ng anti bodi es i n the serum, cerebrospi nal
fl ui d (CSF), or i n sal i va may be i ndi cati ve of i nfecti on. Serol ogi cal techni ques are of ten
empl oyed for vi ral i nf ecti ons such as hepati ti s B.
Bacteriology
If a bacteri al aeti ol ogy i s suspected for a l esi on, both di rect smears and swabs for cul ture
and i denti f i cati on may be taken. Di rect smears from the gi ngi val crevi ce may be of some
val ue i n the i denti f i cati on of the fusobacteri a and spi rochaetes i n acute ul cerati ve
gi ngi vi ti sal though thei r use i s l i mi ted. In many i nstances onl y normal oral fl ora wi l l be
reported. Thi s i s the case, for exampl e, i n vi ral infecti ons. In these ci rcumstances, however,
as i n many other oral mucosal di seases, the bal ance of the oral fl ora i s soon di sturbed by the
onset of abnormal envi ronmental condi ti ons.
Specimens for the identification of micro-organisms
When a col l ecti on of pus i s present an aspi rate shoul d i deal l y be taken.

Mycology
Candi da may be recogni zed on di rect smears, by cul ture, and, i f an esti mate of densi ty of
organi sms i s requi red, by i mpri nt cul ture or an oral ri nse. They survi ve wel l on a dry swab or
when pl aced i n an appropri ate transport medi um. It must be remembered that i n some forms
of candi dosi s, where the organi sms are wi thi n the ti ssues (as i n chroni c hyperpl asti c
candi dosi s; see Chapters 4 and 6), there may be very l i ttl e growth from a swab. They are
best i denti fi ed by hi stol ogi cal methods. Suscepti bil i ty testi ng to vari ous anti fungal agents
can be undertaken, but the reproduci bi l i ty of some tests, parti cul arl y those rel ati ng to
azol es, are questi onabl e.
Virology
Vi rus i denti f i cati on remai ns a l engthy and rel ati vel y di ffi cul t process. Confi rmati on may be
gi ven by di rect el ectron mi croscopy i n the few centres where thi s i s avai l abl e. Ti ssue cul ture,
anti gen detecti on, and i denti fi cati on of geneti c materi al are commonl y empl oyed techni ques.
P. 21
Serum anti body studi es can al so f orm the basi s f or the di agnosi s of vi ral i nf ecti ons. In
herpes si mpl ex the basel i ne l evel of anti bodi es i n any i ndi vi dual before cl i ni cal i nfecti on i s
vari abl e, dependi ng on the past hi story and the degree of the i mmune response. At the ti me
of an acti ve cl i ni cal i nfecti on these l evel s are rai sed consi derabl y. If pai rs of sera from the
pati ent, taken at an i nterval (of about 10 days i n thi s case), can be compared, a si gni fi cant
ri se i n ti tre confi rms the di agnosi s. Qui te cl earl y, thi s i s not a parti cul arl y useful techni que,
except i n retrospect, si nce, i n the case of pri mary herpeti c stomati ti s, the l esi ons wi l l have
gone i nto remi ssi on before the confi rmati on of the di agnosi s i s avai l abl e.
Bacteri a and vi ruses can be i denti fi ed rapi dl y usi ng mol ecul ar techni ques
such as pol ymerase chai n reacti on (PCR) or f l uorescent i n si tu hybri di zati on
(FISH).
Imaging techniques
Pl ai n fi l m radi ography i s the most common i magi ng techni que used i n dental practi ce. The
vari ous vi ews are shown i n Tabl e 2. 7. The val ue of pl ai n radi ography l i es i n the si mpl i ci ty of
the method and al so the f act that i t i s wi del y avai l abl e. The di sadvantages are that i oni zi ng
radi ati on i s used and i magi ng of soft-ti ssue l esi ons i s often unsati sfactory for most
di agnosti c purposes. In areas of compl ex bony anatomy the superi mposi ti on of adjacent
structures on the regi on of i nterest can of ten l i mit the di agnosti c val ue of the i mage. Thi s
probl em i s reduced by the techni que of tomography whi ch uses movement of the X-ray tube
and fi l m to produce a sl i ce through the pati ent wi th bl urri ng of the nei ghbouri ng structures.
The dental panorami c tomograph (DPT) or panorami c vi ew uses thi s pri nci pl e.
When i magi ng bony swel l i ngs, two vi ews shoul d be taken that are at ri ght angl es to each
other.
Di gi tal radi ography uses conventi onal radi ographi c techni ques, but the fi l m i s repl aced wi th a
sensor that transmi ts the i mage to a computer. These systems al l ow some mani pul ati on of
i mage to be carri ed out, whi ch can enhance the i mage qual i ty. Whi l st the equi pment i s
expensi ve, thi s techni que has grown i n popul ari ty over recent years. The advantages i ncl ude
the use of a l ower dose of radi ati on than for conventi onal f i l ms, the el i mi nati on of f i l m
processi ng, and the i mmedi ate vi sual i zati on of an image on a vi sual di spl ay uni t. Tabl e 2. 8
l i sts further speci al i zed i magi ng techni ques that may be useful i n oral medi ci ne.
Table 2.7 Radiographic views
Extraoral views Intraoral views
Panorami c (dental panorami c tomograph, DPT)
Lateral vi ews
Obl i que l ateral
True l ateral
Occi pi tomental
Posteri oranteri or mandi bl e
Submentovertex
Peri api cal
Bi tewi ng
Occl usal
Contrast studi es. The appearance of certai n soft-ti ssue structures vi sual i zed by usi ng i oni zi ng
radi ati on can be enhanced by al teri ng the radi odensi ty of a pati ent' s ti ssues by the
i ntroducti on of contrast medi a. The temporomandi bular joi nt space and sal i vary gl ands are
parti cul arl y amenabl e to thi s techni que. The i magi ng of these structures wi l l be di scussed i n
greater detai l i n the appropri ate chapters.
Contrast studi es are successf ul i n i magi ng:
sal i vary gl ands (si al ography)
joi nts (arthrography)
bl ood vessel s (angi ography)
gastroi ntesti nal tract (bari um swal l ow, meal , and enema)
Radi oi sotope studi es. Certai n ti ssues wi l l preferenti al l y concentrate speci f i c compounds. Thi s
can be expl oi ted f or i magi ng i f the chemi cal i s l abel l ed wi th a radi oacti ve compound. Thi s
techni que wi l l al l ow functi onal studi es to be carried out over ti me. For exampl e sal i vary
functi on of the major gl ands can be assessed by thei r entrapment and rel ease of techneti um
(
99m
Tc) whi ch i s carri ed i n i oni c f orm as pertechnetate. Thi s radi oi sotope emi ts gamma rays
but the short hal f -l i f e of
99m
Tc (6. 5 hours) mi ni mi zes

the exposure of the pati ent to radi ati on. Bone may al so be i nvesti gated i n a si mi l ar manner
usi ng methyl ene di phosphonate as the carri er f or the radi oi sotope. In thi s manner the
acti vi ty of bone tumours and mandi bul ar condyl ar growth and i nf ecti on may be studi ed. The
di sadvantage of radi oi sotope studi es i s that they subject the whol e body to radi ati on and are
ti me-consumi ng to perf orm.
Computeri zed tomography (CT) uses a sophi sti cated X-ray machi ne wi th a ri ng of X-ray
detectors around the pati ent. A computer then generates an i mage that represents a sl i ce
through the pati ent. Mani pul ati on of the data by computer al l ows the i mage to be adjusted to
hi ghl i ght bone or sof t ti ssue. Computeri zed tomography i s of val ue when i nvesti gati ng
i ntracrani al l esi ons, soft- and hard-ti ssue tumours of the head and neck, faci al fractures, and
osteomyel i ti s. It wi l l show i f there i s i nvol vement of adjacent structures. CT scans are
expensi ve and use hi gh doses of radi ati on. Metal l i c objects can cause streak artefacts that
obscure i nf ormati on. Thus, extra- and i ntracoronal restorati ons and i mpl ants can be
P. 22
Table 2.8 Specialized imaging techniques
Utilizing ionizing radiation Not using ionizing radiation
Di gi tal radi ography Ul trasound
Contrast studi es
Radi oi sotope studi es
Computeri zed tomography (CT)
Magneti c resonance i magi ng (MRI)
probl emati c.
Ul trasound uses a very hi gh-frequency pul sed ul trasound beam that i s emi tted f rom a
transducer that i s pl aced agai nst the ski n. The same transducer pi cks up the refl ected beam
and an echo pi cture i mage i s produced. Ul trasound i s saf e and i s usef ul for eval uati ng
vascul ar di sorders and soft-ti ssue swel l i ngs of the neck and face, i ncl udi ng sal i vary gl ands
and l ymph nodes. It i s i deal for di fferenti ati ng between sol i d and cysti c masses. Ul trasound
can be used i n conjuncti on wi th f i ne needl e aspi rati on to ensure correct posi ti oni ng of the
bi opsy needl e. Si mi l arl y, ul trasound may be used to l ocate sal i vary cal cul i that are to be
broken up wi th a si al ol i thotri pter. The di sadvantages of ul trasound i n the head and neck
regi on i s that i ts use i s restri cted to superfi ci al structures because bone wi l l absorb the
sound waves.
Magneti c resonance i magi ng (MRI) uti l i zes the behavi our of protons i n a magneti c fi el d to
produce i mages. Thi s techni que i s val uabl e i n assessi ng i ntracrani al l esi ons, l esi ons of the
sal i vary gl ands, si nuses, and pharynx, and al so of the temporomandi bul ar joi nt. MRI
produces excel l ent di fferenti ati on between soft ti ssues, hard ti ssues, and normal and
abnormal ti ssues, but gi ves poor hard-ti ssue detai l. Thi s i magi ng techni que i s more ti ssue-
sensi ti ve than CT and does not use i oni zi ng radi ation. MRI i s expensi ve and i s
contrai ndi cated i n pati ents wi th certai n types of surgi cal cl i ps, cardi ac pacemakers, and
cochl ear i mpl ants (whi ch contai n magneti c el ements). Pati ents who have cl austrophobi a are
al so unsui tabl e f or MRI because they are unl i kel y to enter i nto the tunnel formed by the core
of the magnet.
Si nce oral medi ci ne represents the whol e fi el d of medi ci ne as rel ated to the oral cavi ty, i t i s
evi dent that as wi de a range of i nvesti gati ons must be appl i ed as i n other speci al i ti es of
medi ci ne. It woul d, qui te cl earl y, be i mpossi bl e to even bri efl y outl i ne these i n the present
context. The major i nvesti gati ve procedures have been outl i ned above. Others are di scussed
i n the appropri ate l ater chapters.
Diagnosis
The di agnosi s may be obvi ous f rom the hi story and exami nati on of the pati ent. Further
i nvesti gati ons may not be requi red and the cl i ni ci an may make a def i ni ti ve di agnosi s.
The cl i ni ci an may have a strong suspi ci on of the di agnosi s but cannot confi rm thi s unti l
further i nformati on i s returned, such as a bi opsy or bl ood resul ts. In thi s si tuati on the
practi ti oner can make a provi si onal , cl i ni cal, or worki ng di agnosi s and may start therapy for
the suspected condi ti on. It i s not uncommon, however, that after exami nati on there remai ns
more than one possi bl e di agnosi s. These condi ti ons make up the di f ferenti al di agnoses. In
thi s si tuati on the cl i ni ci an needs to assess the l ikel i hood of each di f ferenti al di agnosi s, taki ng
i nto account the pati ent' s age, gender, and race, the presenti ng symptoms, the medi cal and
drug hi story, and the cl assi cal features of the vari ous possi bl e di agnoses. Thi s i s a ski l l that
becomes defi ned wi th experi ence and requi res l ogi cal thought and knowl edge of the vari ous
condi ti ons.
Projects
1. Identi fy the medi cal and surgi cal di sci pl i nes that speci al i sts i n oral medi ci ne may l i ai se
cl osel y wi th and gi ve reasons why these rel ati onshi ps are necessary.
2. The parti ci pati on of an oral medi ci ne speci al i st i n some mul ti di sci pl i nary (j oi nt) cl i ni cs
may benefi t pati ent management. What joi nt cl i ni cs do you thi nk woul d be advi sabl e
and l i st the orofaci al condi ti ons that coul d be managed on these cl i ni cs?
> Tabl e of Cont ent s > 3 - Therapy
3
Therapy
Principles of therapy
In some l esi ons of the oral mucosa, speci fi c treatment i s di rectl y i ndi cated by the di agnosi s.
For i nstance, i nf ecti ons by an i denti f i abl e organi sm may be treated by the use of an
appropri ate anti bi oti c. In many oral l esi ons such rati onal therapy i s not, however, avai l abl e,
of ten because of a l ack of knowl edge of the f actors causi ng the condi ti on, and so i t i s
necessary to fal l back on to non-speci f i c treatment modal i ti es, such as topi cal anti bi oti cs,
anti septi cs, l ocal and systemi c corti costeroi ds, and i mmunomodul ati ng drugs.
Topical therapy
Topi cal therapy has several advantages over the systemi c admi ni strati on of drugs. Si de-
eff ects, al though not el i mi nated, are usual l y reduced and l ocal appl i cati on al l ows f or
maxi mum concentrati on of the drug at the si te of the l esi on. Agents tend to be more
eff ecti ve i f they can be retai ned l ocal l y for as l ong as possi bl e.
Covering agents
A number of gel s and pastes are avai l abl e to provi de mechani cal protecti on of ul cerati ve
areas i n the mouth. Agents such as carmel l ose gel ati n gel may al so be used as carri ers for
more acti ve substances such as steroi ds or anti fungal agents. A number of preparati ons of
thi s ki nd are commerci al l y avai l abl e and others can be made up by a pharmaci st.
Unfortunatel y, pati ents of ten report di ffi cul ty appl yi ng these pastes, parti cul arl y at the back
of the mouth.
Topical antiseptics
Chl orhexi di ne has anti bacteri al and some anti candi dal acti vi ty and i s currentl y the most
eff ecti ve anti pl aque agent. Chl orhexi di ne can be used as a mouthwash, spray, or gel to
control secondary i nfecti on i n mucosal ul cerati on and as an adjunct, or short-term
al ternati ve, to toothbrushi ng and other oral hygi ene measures. However, i t frequentl y stai ns
teeth brown and may cause di scol orati on of the tongue. Stai ni ng of the teeth i s reversi bl e
but can be a probl em wi th l ong-term use. Some pati ents do not l i ke the taste of
chl orhexi di ne and occasi onal l y i t causes i di osyncrati c mucosal i rri tati on. Paroti d swel l i ng has
been reported i n a few cases f ol l owi ng use of chl orhexi di ne.
Chl orhexi di ne has anti bacteri al and anti candi dal acti vi ty and i s currentl y the most ef fecti ve
anti pl aque agent.
Topical analgesics
A topi cal anal gesi c preparati on such as benzydami ne (Di f fl am) may provi de temporary
symptomati c rel i ef for erosi ve and ul cerati ve oral l esi ons and enabl e the pati ent to eat and
dri nk. Topi cal anaestheti c agents such as l i docai ne gel (or ri nse) can al so be used for short-
term symptomati c rel i ef but shoul d be prescri bed with cauti on si nce secondary trauma coul d
easi l y be pai nl essl y ef fected duri ng the peri od of anaesthesi a. A f urther precauti on to be
taken i ncl udes the avoi dance of preparati ons of suff i ci ent strength to aff ect the l aryngeal
refl exes. Li docai ne ri nses shoul d not be used for long peri ods of ti me because of the
possi bi l i ty of systemi c absorpti on and si de-eff ects. In spi te of these di ff i cul ti es, i t i s, from
ti me to ti me, justi fi abl e to prescri be treatment of thi s ki nd. Local anaestheti cs are al so
i ncl uded i n some throat l ozenges and mouth ul cer pasti l l es (or paste) on sal e to the publ i c.
Many contai n sugar, however, and pati ents shoul d be warned about thei r cari ogeni c
potenti al . The use of anaestheti c mouthwashes i s further di scussed i n Chapter 5 (Oral
ul cerati on).
Topical antibiotics
There are i nherent di sadvantages associ ated wi th the use of topi cal anti bi oti cs because of
the possi bi l i ti es of sel ecti on f or resi stant strai ns and of i nduci ng hypersensi ti vi ty reacti ons i n
the pati ent. The val ue of topi cal anti bi oti cs outwei ghs such ri sks i n some cases. Tetracycl i ne
(or chl ortetracycl i ne) i s a useful topi cal anti bi oti c. As a 2 per cent sol uti on, i t i s often
eff ecti ve i n reduci ng secondary i nf ecti on (and thus the di scomfort) i n cases of aphthous
stomati ti s, pri mary herpeti c stomati ti s, erosi ve l ichen pl anus, and other severe ul cerati ve
condi ti ons. Interesti ngl y, (chl or)tetracycl i ne mouthwashes are parti cul arl y ef fecti ve i n
reduci ng the di scomfort of herpeti f orm aphthous stomati ti s (see Chapter 5). Use of topi cal
anti bi oti cs cannot normal l y be accepted as l ong-term treatment for recurrent condi ti ons and
i t i s best regarded as treatment reserved for acute epi sodes. The mouthwash may be made
by the pati ent di ssol vi ng the contents of a 250 mg tetracycl i ne capsul e i n 10 ml of water to
gi ve a 2 per cent sol uti on. It may be more eff ecti ve to have the sol uti on accuratel y made up
by the pharmaci st, i ncl udi ng 10 per cent of gl ycerol as a demul cent (see Appendi x).

If the treatment i s not undul y prol onged, there i s mi ni mal troubl e f rom overgrowth of
resi stant organi sms i n the mouth, al though a candi dal i nfecti on may occur and must be
appropri atel y deal t wi th.
Many of the authors pati ents have oral l esi ons that are persi stent and severe. In such
cases, the prol onged use of anti bi oti c-based mouthwashes i s cl i ni cal l y justi fi ed, parti cul arl y
i n the chl ortetracycl i netri amci nol one combi nati on (see Appendi x) that i s used i n such
condi ti ons as pemphi gus and major erosi ve l i chen planus.
Tetracycl i ne mouthwash may be made by the pati ent di ssol vi ng the contents of one 250 mg
capsul e i n 10 ml of water to gi ve a 2 per cent sol uti onuse as a mouthwash, three ti mes
dai l y.
Topical corticosteroids
One of the most i mportant factors to be consi dered when usi ng topi cal steroi ds i s the degree
of suppressi on of adrenal f uncti on that may occur when these drugs are admi ni stered. The
degree of adrenal suppressi on vari es not onl y from steroi d to steroi d and accordi ng to the
method of use, but there i s al so consi derabl e i ndi vi dual vari ati on. For i nstance, a dose of
systemi c predni sol one that may apparentl y cause no si de-eff ects i n one pati ent may render
another markedl y Cushi ngoi d. It i s the authors practi ce to use hi gh-concentrati on, l ocal l y
appl i ed steroi ds to repl ace systemi c medi cati on wherever possi bl e. Excessi ve use of topi cal
steroi d preparati ons can, however, resul t i n a si gni f i cant amount of systemi c absorpti on.
Appl i cati on of more potent topi cal steroi ds al so i ncreases the l i kel i hood of a superi mposed
oral candi di asi s and some oral physi ci ans advocate the concomi tant use of a prophyl acti c,
topi cal anti fungal agent.
Steroid mouthwashes
Steroi d mouthwashes can be made by the pati ent di ssol vi ng sol ubl e betamethasone or
P. 26
predni sol one tabl ets i n 1015 ml of water. Steroid mouthwashes shoul d not be swal l owed
and pati ents shoul d be moni tored f or si de-eff ects, as for systemi c steroi ds, because of the
ri sks of systemi c absorpti on.
Al ternati vel y, a tri amci nol one mouthwash, of ten i n a chl ortetracycl i ne base can be used, at a
total dosage that can be vari ed accordi ng to the severi ty of the condi ti on. Mouthwashes
contai ni ng l evel s of tri amci nol one varyi ng from 0. 751. 5 mg to 36 mg dai l y are most
commonl y used. At the hi gher end of thi s wi de dose-range there i s al most certai n to be
si gni f i cant systemi c absorpti on, and pati ents must be careful l y moni tored f or the onset of
corti costeroi d si de-eff ects. The concentrati on of steroi d i n the mouthwash can be easi l y
vari ed, however, and shoul d be caref ul l y ti trated agai nst the cl i ni cal response (see Appendi x
for formul ati on).
Betamethasone ri nse: di ssol ve a sol ubl e betamethasone sodi um phosphate (0. 5 mg) tabl et i n
10 ml of H
2
O and hol d i n mouth for 3 mi nutes, before spi tti ng out. Use three ti mes dai l y, i f
requi red. Do not swal l ow.
Steroid sprays
Steroi d sprays or i nhal ers such as becl omethasone or budesoni de can be used for treati ng
one or two i sol ated ul cers or erosi ons, parti cul arl y i f they occur i n the anteri or part of the
mouth whi ch i s accessi bl e (Tabl e 3. 1).
Intralesional steroids
Intral esi onal i njecti ons of depot preparati ons of sui tabl e steroi ds, often used by
dermatol ogi sts to produce l ocal i zed hi gh concentrati ons of the acti ve drug, may be used to
treat oral l esi ons. In general , they do not appear to be as ef fecti ve i n oral medi ci ne practi ce
and are onl y occasi onal l y used. Intral esi onal steroi d i njecti ons have been used f or treati ng
swol l en l i ps i n pati ents wi th orof aci al granul omatosi s (OFG) (see Chapter 12).
Steroid pastes
Tri amci nol one acetoni de (0.1 per cent) i s avai l abl e i n an adhesi ve paste and can be appl i ed
to aphthous ul cers 24 ti mes dai l y. It i s most eff ecti vel y used at ni ght, when sal i vary fl ow
decreases and the pati ent i s not eati ng. Other topical steroi ds (f or exampl e, f l uoci noni de or
Table 3.1 Examples of steroid sprays that can be used
for ulcerative oral lesions
Becl ometasone di propi onate spray del i veri ng a 50 mi crograms metered dose.
Budesoni de spray del i veri ng a 100 mi crograms metred dose.
cl obetasol ) mi xed wi th Orabase (equal proporti ons) can be used for i sol ated, ul cerati ve
l esi ons, parti cul arl y those i n the anteri or part of the mouth (see Chapter 5).
Steroid lozenges
Lozenges (pel l ets) contai ni ng 0. 1 per cent hydrocorti sone can be used for recurrent aphthous
stomati ti s (RAS). Thi s steroi d i s l i kel y to be most eff ecti ve i f used i n the prodromal phase of
ul cerati oni t shoul d be pl aced on (or near) the ul cer and al l owed to di ssol ve. The use of
steroi d l ozenges i n ul cer-free peri ods, al bei t at a reduced frequency, has been recommended
by some oral physi ci ans f or RAS. Whether or not this reduces the occurrence of new aphthae
has yet to be proven (see Chapter 5).
Creams and ointments
Creams and oi ntment are used as vehi cl es for acti ve i ngredi ents, such as anti mi crobi al
agents and steroi d preparati ons. They may be used to treat a number of condi ti ons seen i n
the oral medi ci ne cl i ni c that af fect the l i ps (f or exampl e, angul ar chei l i ti s) and peri oral
ti ssues.
Creams are emul si ons of oi l and water and are usuall y wel l absorbed i nto ski n. General l y,
creams are cosmeti cal l y more acceptabl e than oi ntments because they are l ess greasy and
theref ore sui tabl e for use on the f ace. Creams usual l y contai n preservati ves (for exampl e,
chl orocresol and parabens) unl ess thei r acti ve

i ngredi ent has suf fi ci ent i ntri nsi c anti mi crobi al acti vi ty. Preservati ves may cause
sensi ti zati on and ul ti matel y an al l ergi c contact dermati ti s.
Oi ntments are greasy preparati ons that are i nsol ubl e i n water. They are parti cul arl y useful
for treati ng chroni c, dry ski n condi ti ons and are l ess easi l y washed off than creams.
Systemic therapy
Systemi c therapy wi th non-speci f i c i mmunomodul ati ng drugs such as steroi ds and
azathi opri ne, together wi th substances such as thal i domi de, are i ncreasi ngl y bei ng used for
severe ul cerati ve and erosi ve oral condi ti ons. These drugs have si gni fi cant si de-eff ects,
however, and pati ents requi re careful assessment and moni tori ng under hospi tal supervi si on
(see bel ow).
Systemic corticosteroids
The use of systemi c corti costeroi ds for the treatment of oral mucosal l esi ons i s justi fi abl e i f
topi cal therapy has fai l ed. Predni sol one has predomi nantl y gl ucocorti coi d acti vi ty and i s the
corti costeroi d most commonl y taken oral l y f or l ong-term di sease suppressi on. Oral l esi ons,
such as major aphthae or erosi ve l i chen pl anus, may need to be managed by a short (26
week) reduci ng course of predni sol one. Other i ntractabl e and severe cases may justi fy and
necessi tate l ong-term predni sol one therapy, wi th i ts associ ated di sadvantages and si de-
eff ects. In many of these cases the oral l esi ons are part of a wi despread and more
general i zed condi ti on, such as pemphi gus or Behet' s syndrome. Scruti ny of a pati ent' s
medi cal hi story i s essenti al bef ore commenci ng corti costeroi d therapy and i t i s essenti al that
regul ar moni tori ng i s carri ed out to detect any si de-eff ects. Moni tori ng shoul d i ncl ude
measurements of bl ood pressure, wei ght, and bl ood gl ucose esti mati ons. Steroi d treatment
warni ng cards shoul d be i ssued to pati ents and carri ed by them at al l ti mes.
Steroi d treatment warni ng cards shoul d be i ssued to pati ents and carri ed by them at al l
ti mes.
Basel i ne measurements of bl ood pressure, f asti ng bl ood gl ucose, and wei ght are checked
P. 27
bef ore commenci ng therapy and a chest radi ograph may be i ndi cated i n some cases. Pati ents
shoul d be made aware of the potenti al adverse eff ects of the medi cati on (Tabl e 3. 2) and the
need for regul ar moni tori ng. In addi ti on, corti costeroi ds shoul d not be stopped abruptl y.
Pati ents who are taki ng systemi c corti costeroi ds may be at ri sk f rom a hypotensi ve adrenal
cri si s i f they undergo dental or maxi l l ofaci al procedures, parti cul arl y under general
anaesthesi a, and consi derati on shoul d be gi ven to provi di ng them wi th addi ti onal steroi d
cover. There are, however, no evi dence-based protocol s currentl y avai l abl e to i ndi cate
whi ch pati ents undergoi ng dental treatment shoul d be gi ven addi ti onal steroi d cover.
Nei ther i s there any cl ear i ndi cati on of the opti mum dose and ti me of del i very. There i s
evi dence that adrenal suppressi on i s reduced i f corti costeroi ds are gi ven once a day (i n the
morni ng) and possi bl y on al ternate days.
Fi fty per cent of pati ents on l ong-term corti costeroi ds devel op osteoporosi s and bone l oss i s
greatest i n the fi rst 36 months of steroi d therapy. Preventi ve therapy, for exampl e,
di sodi um eti dronate (Di dronel PMO), shoul d therefore be commenced at the outset of
steroi d therapy (predni sol one 5 mg or more per day) that i s l i kel y to l ast for more than 3
months. A basel i ne bone densi ty scan shoul d i deal l y be organi zed f or adul t pati ents. If the
pati ent i s f ound to have osteoporosi s pri or to treatment wi th steroi ds, more potent
bi sphosphonates (f or exampl e, al endronate or ri sedronate) may be requi red and the pati ent
may need to be referred to a metabol i c bone di sease cl i ni c.
Pati ents on predni sol one 5mg/day (or equi val ent) f or more than 3 months shoul d be
gi ven prophyl acti c bi sphosphonates.
Azathioprine
Steroi d-spari ng drugs such as azathi opri ne may be gi ven to mi ni mi ze the l ong-term ef fects of
systemi c steroi ds. Azathi opri ne i s a cytotoxi c i mmunosuppressant wi th potenti al l y seri ous
si de-eff ects. It shoul d not be prescri bed unl ess adequate moni tori ng i s avai l abl e throughout
the durati on of therapy (see drug data sheet). Patients are at ri sk of devel opi ng bone marrow
suppressi on, parti cul arl y i f they have a defi ci ency of thi opuri ne methyl transf erase (TPMT), a
cytopl asmi c enzyme i nvol ved i n the metabol i sm of azathi opri ne. Pati ents can now be tested
Table 3.2 Complications of systemic corticosteroid
therapy
Suppressi on of adrenal functi on
Hypertensi on
Sodi um and water retenti on
Potassi um l oss
Di abetes
Osteoporosi s
Mental di sturbances (psychoses, mood changes, euphori a)
Muscl e wasti ng
Fat redi stri buti on
Pepti c ul cerati on
Cushi ng' s syndrome
Suscepti bi l i ty to i nf ecti ons (candi dosi s, bacteri al and vi ral i nf ecti ons)
Growth suppressi on i n chi l dren
Neopl asms, e. g. l ymphomas
for TPMT defi ci ency.
Other systemic drugs used in oral medicine
Other systemi c drugs currentl y used i n oral medi ci ne i ncl ude dapsone, ci cl ospori n, col chi ci ne,
thal i domi de, and mycophenol ate. The i ndi cati ons for and moni tori ng of si de-eff ects
associ ated

wi th these drugs are di scussed i n the rel evant chapters throughout thi s book. There i s al so
i ncreasi ng i nterest i n the use of anti -TNF (ti ssue necrosi s f actor) al pha therapy for severe
i nfl ammatory di sease (for exampl e, Behets di sease). These drugs are used on a l i mi ted
basi s onl y for severe oral di sease and have no pl ace i n the non-speci al i st practi ce of oral
medi ci ne.
Limitations of therapy
There i s a pauci ty of objecti ve evi dence concerni ng the eff ecti veness of a number of current
therapeuti c regi mens for oral medi ci ne condi ti ons and there i s cl earl y a need for the
devel opment of evi dence-based management protocol s. Cl i ni ci ans shoul d al so be aware,
parti cul arl y when prescri bi ng topi cal formul ati ons of drugs such as steroi d mouthri nses or
sprays, that most are not l i censed for use i n oral condi ti ons and dosage schedul es are
theref ore not establ i shed. Pati ents shoul d be made aware that they are bei ng gi ve a drug
outsi de i ts l i censed i ndi cati on as that they cannot gi ve i nformed consent for treatment
wi thout thi s knowl edge.
Project
1. Vi si t your l ocal pharmacy and enqui re whi ch therapi es are avai l abl e to buy over the
counter (OTC) f or mouth ul cers. What advi ce woul d the pharmaci st gi ve to someone
seeki ng i nformati on about mouth ul cers?
P. 28
> Tabl e of Cont ent s > 4 - Inf ecti ons of t he gi ngi vae and or al mucosa
4
Infections of the gingivae and oral mucosa
Problem cases
Case 4.1
A 28-year-ol d l ady attends your dental practi ce f or her 6-monthl y exami nati on. You noti ce
that she has a heal i ng col d sore (herpes l abi al i s) on her l ower l i p. On cl oser
questi oni ng thi s pati ent reports that she suff ers from col d sores every 23 months
and that they are becomi ng a real nui sance. She i s parti cul arl y af fected when on
hol i day abroad and exposed to the sun.
Case 4.2
A 21-year-ol d ful l y dentate man presents at your dental practi ce compl ai ni ng of a sore mouth
and throat of 6 weeks durati on. A pharmaci st recommended that he use anaestheti c throat
l ozenges and a chl orhexi di ne ri nse but these have not hel ped. The pati ent appears wel l and
reports no rel evant medi cal hi story. Extraoral exami nati on reveal s no l ymphadenopathy.
Intraoral l y, hi s oral mucosa appears erythematous and there i s evi dence of extensi ve whi te
fl ecks on the soft pal ate and both buccal mucosae. These can easi l y be wi ped off wi th a
gauze napki n. You make a cl i ni cal di agnosi s of pseudomembranous candi dosi s. There are no
other abnormal i ti es of the oral mucosa or gi ngi vae.
Introduction
It i s possi bl e to demonstrate a wi de range of potenti al l y pathogeni c organi sms i n the normal
mouth. Many of these are present i n rel ati vel y hi gh numbers, but i n spi te of thi s the oral
mucosa shows a remarkabl y l ow suscepti bi l i ty to primary i nfecti on. Thi s i s probabl y due, at
l east i n part, to the acti vi ty of sal i va, from whi ch a number of anti bacteri al substances may
be demonstrated. IgA i s i n the sal i va and i t has been suggested that, as wel l as a possi bl e
di rect ef fect of thi s on the oral mi cro-organi sms, a compl ex may be formed between the
i mmunogl obul i n and the oral epi thel i um i tsel f resulti ng i n a protecti ve surface coati ng on the
mucosa. There i s al so a further mechani cal protective acti vi ty of sal i va i n that forei gn
materi al , i ncl udi ng mi cro-organi sms, i s washed from the oral cavi ty to the stomach where
bacteri a are destroyed by gastri c fl ui ds.
These l ocal factors, however, represent no more than a fi rst l i ne of defence for the pati ent
agai nst i nvasi on by potenti al pathogens, and depend for thei r effecti veness on the i ntegri ty
of the i mmune and other general i zed protecti ve responses of the host. If the bal ance
between the host and the commensal mi cro-organi sms i s di sturbed by some factor that
i mpai rs the i mmune defences then opportuni sti c organi sms may begi n to act i n a pathogeni c
Q1 What advi ce woul d you gi ve thi s l ady?
Q2 Does the l esi on on her l i p present a hazard to members of the dental team?
Q1 How woul d you manage thi s pati ent?
manner. Thi s i s a resul t of thei r i ncrease i n numbers that exceeds a mi ni mum i nf ecti ous
dose. It i s i n such a way that many cases of oral candi dosi s and acute necroti zi ng ul cerati ve
gi ngi vi ti s are thought to occur. Apart f rom i nfecti ons of the oral mucosa brought about by a
di sturbance of a normal hostcommensal rel ati onshi p there are others, parti cul arl y those
of vi ral ori gi n, that represent the fi rst response of the pati ent to the i nfecti ve agent. Even i n
some of these (orofaci al herpes i nfecti ons are an exampl e) there may be compl ex
i mmunol ogi cal changes i n the pati ent that are of great si gni fi cance i n understandi ng the
cl i ni cal course of the di sease. The i mportance of pri mary and secondary i mmune defi ci enci es
i n rel ati on to oral i nfecti ons i s di scussed i n Chapter 14.
The rol e of bacteri a i n the pathogenesi s of cari es, non-speci f i c gi ngi vi ti s, and peri odonti ti s i s
outsi de the scope of thi s chapter and readers shoul d refer to speci al i st texts on these
subjects.
In previ ous edi ti ons of thi s book, streptococcal stomati ti s was i ncl uded as a bacteri al
i nfecti on possi bl y aff ecti ng the oral mucosa. There woul d seem to be no doubt that there i s a
readi l y avai l abl e reservoi r of streptococci i n the oral cavi ty that mi ght be expected to
respond to a di sturbance i n the hostcommensal rel ati onshi p by behavi ng i n a pathogeni c
manner. It i s equal l y evi dent that a general i zed i nfecti on of the mouth and oropharynx mi ght
occur as a resul t of i nvasi on by exogenous cocci . In spi te of thi s, i t i s by no means cl ear that
a true streptococcal stomati ti s does, i n f act, exi st, al though unti l rel ati vel y recentl y i t was
general l y accepted as a cl i ni cal enti ty. The conf usi on may have ari sen because of the
si mi l ari ty between the symptoms ascri bed to a streptococcal stomati ti s and those caused by
mi l d vi ral i nfecti ons. The symptoms of streptococcal stomati ti s were general l y descri bed as a
general i zed erythema of the oral mucosa together with a marked gi ngi vi ti s, submandi bul ar
l ymphadeni ti s, and a mi l d degree of mal ai se and fever.

Bacterial infections
Acute necrotizing ulcerative gingivitis (ANUG)
Acute necroti zi ng ul cerati ve gi ngi vi ti s (ANUG) was, unti l recentl y, rel ati vel y common, but i s
currentl y much l ess so i n devel oped countri es. ANUG does sti l l occur i n smokers and
i mmunocompromi sed pati ents, parti cul arl y i n those wi th human i mmunodefi ci ency vi rus (HIV)
i nfecti on. When i rreversi bl e peri odontal desctruction has occurred the condi ti on i s now more
appropri atel y termed acute necroti zi ng ul cerati ve peri odonti ti s. Throughout thi s
secti on, the term ANUG wi l l be used to descri be both condi ti ons. The exact cause of ANUG i s
not known, but there i s no doubt that duri ng an attack there i s a prol i ferati on of spi rochaetes
and fusi form bacteri a. There i s al so a prol i ferati on of obl i gate anaerobi c, non-sporul ati ve
rods. These rods are thought to be i mportant and probabl y more si gni fi cant than the
spi rochaetes. Al though there are many theoreti cal specul ati ons about the exact aeti ol ogy of
ANUG, there i s no doubt that, f or practi cal purposes, el i mi nati on of the overgrowth of the
mi cro-organi sms i s coi nci dent wi th cl i ni cal remi ssion of the di sease.
Clinical features
The cl i ni cal f eatures of ANUG are soreness and bl eedi ng of the gi ngi vae, together wi th the
devel opment of crater-l i ke ul cers, due to the necrosi s of the gi ngi val papi l l ae (Fi g. 4. 1). Thi s
ul cerati on subsequentl y spreads al ong the gi ngi val margi n. Pati ents devel op a marked
hal i tosi s, whi ch has a characteri sti c odour (Tabl e 4. 1). In a few pati ents wi th ANUG there are
fever, mal ai se, and l ymphadeni ti s. Most, however, are young adul ts and, i n the more usual ,
uncompl i cated case, the pati ent i s i ni ti al l y perfectl y heal thy.
Any pati ent, for whom predi sposi ng factors cannot be i denti f i ed, shoul d be suspected of
suff eri ng f rom an underl yi ng systemi c di sorder and, i n vi ew of the possi bi l i ty of a bl ood
dyscrasi a i n such pati ents, haematol ogi cal exami nati on shoul d be undertaken. One of the
P. 32
greatest cl i ni cal probl ems i n the management of ANUG i s that of recurrence. It i s evi dent
that the pati ent wi th poor oral hygi ene or wi th gi ngi val contours di storted by a previ ous
attack may, because of these l ocal factors, be suscepti bl e to recurrent i nfecti on, parti cul arl y
i f predi sposi ng f actors sti l l persi st.
Predisposing factors
The i nfl uence of poor oral hygi ene i n the i ni ti ati on of ANUG has been of ten stressed, but
there i s no doubt that there are some pati ents whose standard of hygi ene must be
consi dered by normal cri teri a to be good. The most common predi sposi ng factors other than
systemi c ones are tobacco smoki ng and psychol ogi cal stress, al though i t i s by no means easy
to see how these factors operate. Ni coti ne causes vasoconstri cti on of bl ood vessel s wi th a
Fig. 4.1 Acute necroti zi ng ul cerati ve gi ngi vi ti s, showi ng destructi on of the gi ngi val
papi l l ae.
Table 4.1 Clinical features of acute necrotizing ulcerative
gingivitis (ANUG)
Soreness and bl eedi ng of the gi ngi vae
Necrosi s of the gi ngi val papi l l ae
Hal i tosi s
consequent reducti on of bl ood suppl y to the ti ssues and i ncreased suscepti bi l i ty to i nf ecti on
and damage.
Decreased host resi stance or depressed i mmune responses are i mportant systemi c factors
predi sposi ng to ANUG. A chroni c necroti zi ng gi ngi viti s i s associ ated wi th HIV i nfecti on.
In normal heal thy pati ents wi th ANUG, spread of the i nfecti on from the gi ngi val margi ns i s
rel ati vel y rare. In a pati ent weakened by debi l i tati ng di sease the i nfecti on may spread to
surroundi ng ti ssues. An exampl e of the spread of ANUG i n the debi l i tated pati ent i s seen i n
cancrum ori s (noma) a condi ti on now vi rtual l y unknown i n Europe.
Diagnosis of ANUG
The cl i ni cal di agnosi s of ANUG may be confi rmed by the demonstrati on of a fusospi rochaetal
compl ex i n a Gram-stai ned deep gi ngi val smear. Bacteri a cul tured i n ANUG i ncl ude,
Treponema vi ncenti i , denti col a, and macrodenti um; Prevotel l a i ntermedi a; Porphyromonas
gi ngi val i s; and Fusobacteri um nucl eatum.
Management of ANUG
The i ni ti al treatment of ANUG compri ses supragi ngi val pl aque control , and the use of a
systemi c anti bi oti c, such as metroni dazol e. Smokers shoul d be advi sed to ref rai n from
smoki ng. Pati ents wi th ANUG present wi th varyi ng degrees of gi ngi val di scomfort, but i n
most cases i t i s not feasi bl e to carry out a scal e and pol i sh at the i ni ti al vi si t. Gentl e
debri dement of the gi ngi val ti ssues shoul d be done and the pati ent gi ven i nstructi ons to use
a chl orhexi di ne mouthri nse and attempt gentl e toothbrushi ng. ANUG responds rapi dl y to the
use of peni ci l l i n, and a number of other anti bi oti cs, but metroni dazol e i s the usual drug of
choi ce. A dose of 200 mg, three ti mes dai l y, for 3 days i s suff i ci ent i n most cases to reduce
the symptoms dramati cal l y i n 24 hours.

However, i f resol uti on does not occur, an underl yi ng condi ti on shoul d be suspected and
appropri ate i nvesti gati ons undertaken. When prescribi ng metroni dazol e pati ents shoul d be
warned to avoi d al cohol . Metroni dazol e i s si mi l ar in i ts ef fects to di sul fi ram and bl ocks the
normal metabol i c pathway of al cohol el i mi nati on. In some pati ents, si de-eff ects such as
nausea, hypotensi on, and fl ushi ng may fol l ow i f al cohol i s taken duri ng treatment. The
teratogeni c eff ects of metroni dazol e are uncertai n and i t i s theref ore not recommended i n
earl y pregnancy. Once the acute phase has resol ved, peri odontal treatment shoul d
commence, together wi th smoki ng cessati on advi ce.
Metroni dazol e i s the anti bi oti c of choi ce for the ini ti al management of ANUG.
Pati ents shoul d be warned not to take al cohol whi l st taki ng metroni dazol e.
Metroni dazol e i s best avoi ded duri ng pregnancy.
Syphilis
Syphi l i s i s a sexual l y transmi tted di sease and the causati ve bacteri al agent i s Treponema
pal l i dum. Thi s gai ns entry to the body vi a mucous membranes and mi nute abrasi ons i n the
ski n. There i s a popul ar mi sconcepti on that syphi l is i s a rare di sease but i t i s a common
i nfecti on i n Russi a and the Far East and i ts i nci dence worl dwi de i s i ncreasi ng. Outbreaks of
cases conti nue to be reported i n the UK and oral l esi ons of syphi l i s may be seen i n the oral
medi ci ne cl i ni c. Earl y di agnosi s i s essenti al as the l ong-term ef fects of untreated syphi l i s are
seri ous and potenti al l y l i f e-threateni ng.
P. 33
Clinical features of syphilis
Pri mary l esi ons of syphi l i s may appear on any part of the oral mucosa and must al ways be
consi dered i n the di ff erenti al di agnosi s of oral ulcerati on. As i n the case of the geni tal l esi on
the oral pri mary l esi on (chancre) appears f ol l owi ng a peri od of 23 weeks after i nfecti on.
Usual l y the chancre presents as a pai nl ess i ndurated swel l i ng, dark red i n col our and wi th a
gl azed surface (Fi g. 4. 2), from whi ch l arge numbers of Treponema pal l i dum can be i sol ated
and demonstrated on dark-ground mi croscopy. Chancres are most l i kel y to be found i n the
rel ati vel y soft and unrestri cted ti ssues of the tongue, cheeks, or l i ps, but where they occur
on the pal ate or gi ngi vae the morphol ogy of the l esi ons may be modi fi ed, and they may
appear as more di ff use structures. Whatever the si te, si ze, or shape of the chancre, however,
the heavy i nf ecti on of the surface i s consi stent, thus provi di ng both a conveni ent means of
di agnosi s and a consi derabl e hazard to the unsuspecti ng di agnosti ci an parti cul arl y i f gl oves
are not worn. At the ti me of the pri mary l esi on there i s a nontender enl argement of the
cervi cal l ymph nodes aff ecti ng the submental , submandi bul ar, pre- and post-auri cul ar, and
occi pi tal groupsthe so-cal l ed syphi l i ti c col l ar.
Thi s di sappearance of the pri mary l esi on, usual l y after a peri od of some 2 weeks, marks the
wi despread di ssemi nati on of the mi cro-organi sms and the onset of the second stage of the
di sease, whi ch may l ast f or many years. The oral symptoms most often descri bed at thi s
stage of the di sease are mucous patches (appeari ng as grey-whi te ul cers covered by a thi ck
sl ough) and snai l track ul cers. In vi ew, however, of the protean nature of the ski n
l esi ons produced duri ng thi s stage of the di sease, i t woul d seem at l east a reasonabl e
possi bi l i ty that there may be an equi val ent vari ation i n the form of oral l esi ons. If thi s i s so,
i t woul d seem more than l i kel y that secondary syphil i ti c ul cers may often pass unrecogni zed
or be mi staken f or some l ess si gni f i cant non-speci fi c l esi on and, for thi s reason, serol ogi cal
tests f or syphi l i s shoul d be part of the i nvesti gati on of oral ul cerati on of unknown ori gi n. In
thi s second stage l ymph nodes may agai n be pal pabl e as nontender, di screte structures.
In the terti ary stage of syphi l i s two major forms of oral i nvol vement may occur. The fi rst of
these, syphi l i ti c l eukopl aki a, wi l l be deal t wi th in detai l i n Chapter 10 together wi th other
l eukopl aki as. In the i nadequatel y treated or untreated cases, whi ch are now seen very rarel y,
l eukopl aki a of the whol e of the oral mucosa but especi al l y of the tongue i s an i mportant
compl i cati on, the more so because mal i gnant transformati on can occur. The second type of
Fig. 4.2 Pri mary syphi l i ti c l esi on (chancre) of the tongue.
oral i nvol vement i n the terti ary stage i s the development of a gumma. Thi s i s
essenti al l y a chroni c granul oma, of ten i n the pal atal ti ssues, that eventual l y breaks down
wi th the consequent producti on of a ti ssue defect. The untreated pati ent at thi s stage i s
l i kel y to have other more wi despread l esi ons, especi al l y of the nervous system, but the
mi cro-organi sms are by no means as readi l y demonstrated as i n the earl i er stages of the
di sease. As wel l as these most commonl y descri bed mani f estati ons of l ate syphi l i s, a number
of other oral changes have been descri bed, i ncl udi ng a fi brosi ng gl ossi ti s. Osteomyel i ti s
aff ecti ng the j aw i s a rare compl i cati on of syphi l i s. A wi de range of cl i ni cal presentati ons of
terti ary syphi l i s i s possi bl e and thi s fact emphasizes the wi sdom of i ncl udi ng serol ogi cal tests
for the di sease i n the i nvesti gati on of unusual oral condi ti ons. Even i f the pati ent i s of an
advanced age when the condi ti on i s di agnosed and although no systemi c effects are obvi ous,
treatment i s i ndi cated. Symptoms such as mental confusi on that mi ght be attri buted to

seni l e changes may be, i n f act, the resul t of the syphi l i ti c i nfecti on and may respond to
anti bi oti c treatment even at a l ate stage.
Treponema pal l i dum crosses the pl acental barri er and causes congeni tal syphi l i s i n the fetus.
The dental abnormal i ti es mai nl y aff ect the permanent denti ti on, as the deci duous teeth are
general l y wel l devel oped by the ti me thei r tooth germs are i nvaded by the spi rochaetes. The
fi rst permanent mol ars (Mul berry or Moon' s mol ars) and upper central
i nci sors (Hutchi nson' s i nci sors) are usual l y i nvol ved i n congeni tal syphi l i s (Fi g. 4. 3).
The diagnosis of syphilis
The di agnosi s of syphi l i s i s general l y based on the resul ts of serol ogi cal tests. Treponema
pal l i dum cannot be cul tured i n vi troi t i s propagated i n ani mal s to al l ow organi sms to be
prepared for serol ogi cal tests. Current serol ogi cal tests i ncl ude the Venereal Di sease
Reference Laboratory (VDRL) test, the Treponema pal l i dum haemaggl uti nati on assay (TPHA),
the fl uorescent Treponema anti body absorbed test (FTA (abs)), and the Treponema pal l i dum
i mmobi l i zati on (TPI) test (Tabl e 4. 2).
The treatment of syphilis
Peni ci l l i n i s the treatment of choi ce and i s gi ven i n hi gh doses. In pri mary syphi l i s the course
P. 34
Fig. 4.3 Hutchi nson' s i nci sors i n congeni tal syphi l i s.
of anti bi oti cs i s up to 1 month but i n l ate (or l atent) syphi l i s thi s i s for up to 12 weeks.
Pati ents who are al l ergi c to peni ci l l i n can be prescri bed erythromyci n or tetracycl i ne.
Gonorrhoea
Pri mary oral l esi ons of gonorrhoea are rel ati vel y rare. They are the resul t of transmi ssi on of
the organi sm (Nei sseri a gonorrhoea) by di rect mucosal contact.
Clinical features
Lesi ons appear predomi nantl y i n the pharynx and are i nvari abl y a resul t of orogeni tal sexual
contact.
Di ff use erythematous and ul cerati ve oral l esi ons, tonsi l l i ti s, a purul ent gi ngi vi ti s, and other
oral mani f estati ons have been descri bed. Pati ents demonstrated to have oral l esi ons are very
few rel ati ve to the very l arge number known to have geni tal gonorrhoea. The systemi c
di sturbances descri bed as bei ng associ ated wi th such oral l esi ons vary from mi l d to severe
febri l e symptoms, and the degree of oral di scomfort reported i s equal l y vari abl e, wi th some
pati ents compl ai ni ng of di ffi cul ty swal l owi ng. Submandi bul ar l ymphadenopathy may be
present. It i s l i kel y that, i n most cases, the nature of the i nf ecti on i s not i ni ti al l y suspected
on cl i ni cal grounds, but becomes cl ear onl y as a resul t of bacteri ol ogi cal exami nati on. The
true i nci dence of oral gonorrhoeal i nfecti ons probabl y remai ns unrecogni zed because of the
rel ati vel y non-speci f i c nature of the l esi ons.
Cl i ni cal features of oral gonorrhoea i ncl ude di ffuse vesi cul ar, erythematous, and ul cerati ve
oral l esi ons and a purul ent gi ngi vi ti s. Tonsi l l ar invol vement may al so be a feature.
A di f fuse f orm of gonorrhoea spread by haematogenous routes may very occasi onal l y aff ect
the oral mucosa, al though the predomi nant mani festati ons are on the ski n. Ul cers,
haemorrhagi c l esi ons, and other mani festati ons of hypersensi ti vi ty to the di ssemi nati ng
mi cro-organi sms have been descri bed. Occasi onal pati ents wi th gonococcal i nfecti ve arthri ti s
of the temporomandi bul ar joi nts have al so been reported. The symptoms are si mi l ar to those
of other types of i nfecti ve arthri ti spai n, swel l i ng, and tri smus. In most descri bed cases
the di agnosi s has depended on bacteri ol ogi cal study of fl ui d aspi rated from the af fected
joi nt.
Table 4.2 Serological tests currently used to diagnose
syphilis
Test Abbreviation
Venereal Di sease Ref erence Laboratory test VDRL
Treponema pal l i dum haemaggl uti nati on assay TPHA
Fl uorescent Treponema anti body absorbed test FTA
Treponema pal l i dum i mmobi l i zati on test TPI
Gonorrhoea i n the orofaci al area i s l i kel y to be underdi agnosed.
Diagnosis of gonorrhoea
Mi crobi ol ogi cal confi rmati on i s essenti al to conf i rm the cl i ni cal suspi ci on. Exami nati on of a
Gram-stai ned smear of the oral l esi ons may show Gram-negati ve di pl ococci . Mi crobi ol ogi cal
swabs shoul d al ways be taken for cul ture and sensi ti vi ty.
Treatment of gonorrhoea
As i n urogeni tal gonorrhoea, the treatment i s by high doses of anti bi oti cs. Varyi ng regi mes
have been descri bed, varyi ng from a si ngl e, hi gh-dose i ntramuscul ar i njecti on of procai ne
peni ci l l i n to oral amoxyci l l i n (or ampi ci l l i n) to short courses of oral tetracycl i ne or co-
tri moxazol e. The pati ent shoul d be ref erred to a speci al i st i n geni touri nary medi ci ne (GUM)
for ful l assessment. As i n al l sexual l y transmi tted di seases, i t shoul d be remembered that the
pati ent mi ght have acqui red more than one i nfecti on at the same ti me.

Non-specific urethritis
Non-speci f i c urethri ti s (NSU) i s probabl y the most common sexual l y transmi tted di sease and
i n most cases Chl amydi a speci es may be i sol ated f rom the urethra. The symptoms i ncl ude a
burni ng sensati on on mi cturi ti on and there may be a purul ent di scharge i n mal es. Chl amydi a
i nfecti on may be asymptomati c, parti cul arl y i n femal es. Mi crobi ol ogi cal tests are essenti al to
confi rm the di agnosi s and the majori ty of cases respond to tetracycl i ne therapy i n normal
doses over a week or two, even though no mi crobi al agent can be demonstrated. Rei ter' s
syndrome i s predomi nantl y seen i n young mal es and may fol l ow i nfecti on wi th gonorrhoea,
chl amydi a, or enteri c bacteri a. In thi s condi ti on there may be pol yarthri ti s, whi ch can aff ect
the temporomandi bul ar joi nt, urethri ti s, uvei ti s (or conjuncti vi ti s), and macul ar l esi ons on
the pal ms and sol es. Oral l esi ons tend to be erythematous and of ten resembl e erythema
mi grans wi th a whi ti sh border. Thi s appearance has been descri bed as a ci rci nate
stomati ti s.
Rei ter' s syndrome predomi nantl y affects young mal es and can present as a pol yarthri ti s,
urethri ti s, uvei ti s, and macul ar l esi ons on the palms and sol es. Oral l esi ons have al so been
reported and i ncl ude a ci rci nate stomati ti s.
Tuberculosis
The genus Mycobacteri um i ncl udes pathogeni c and non-pathogeni c speci es. M. tubercul osi s
and M. bovi s are equal l y pathogeni c f or man. Tubercul osi s i s a worl dwi de endemi c di sease
wi th up to one-thi rd of the worl d' s popul ati on bei ng affected. There i s a resurgence of
respi ratory pul monary di sease associ ated wi th i mmunodefi ci ency, mal nutri ti on, and non-
compl i ance wi th drug regi mes, parti cul arl y as an AIDS-rel ated phenomenon. The
pathogenesi s, cl i ni cal features, di agnosi s, and treatment of pul monary tubercul osi s are
outsi de the scope of thi s book.
Pri mary oral i nvol vement wi th tubercul osi s may, however, occur, al bei t rarel y, and shoul d
al ways be consi dered i n the di fferenti al di agnosi s of oral ul cerati on. The oral mucosa i s more
commonl y i nvol ved by becomi ng secondari l y i nf ected by the sputum i n cases of acti ve
pul monary di sease. Tubercul ous l ymphadeni ti s frequentl y aff ects the cervi cal l ymph nodes.
Ini ti al l y, a fi rm but mobi l e l ymphadenopathy i s pal pabl e. Si nus and abscess f ormati on occur
l ater wi th cervi cal l ymph nodes becomi ng fi xed (see Chapter 7).
Clinical features of oral tuberculosis
Oral l esi ons of tubercul osi s usual l y present as pai nful ul cerati on. The cl assi cal descri pti on of
P. 35
a tubercul ous ul cer i s of an i rregul ar l esi on wi th undermi ned borders and covered by a grey
sl ough. Ul cerati on commonl y af fects the tongue but other areas of the oral mucosa may be
i nvol ved, parti cul arl y towards the posteri or parts of the mouth. It shoul d be added that a f ew
l esi ons have been descri bed i n whi ch the presentati on of tubercul osi s of the oral mucosa i s
qui te di ff erent from the ul cers descri bed above. These l esi ons, presenti ng as whi te patches
or granul ati ng l esi ons, have been descri bed both as pri mary l esi ons and as l esi ons secondary
to pul monary i nf ecti on.
The diagnosis of oral tuberculosis
A tubercul ous ori gi n shoul d be consi dered i n the di fferenti al di agnosi s of persi stent oral
ul cerati on of unknown aeti ol ogy. Defi ni ti ve di agnosi s of such a l esi on i nvari abl y fol l ows
bi opsy, wi th hi stopathol ogi cal exami nati on of formal i n-fi xed ti ssue, showi ng non-caseati ng
granul omata. The number of aci dal cohol -fast baci l l i present i n the bi opsy speci men may,
however, be smal l and thei r demonstrati on by Zi ehl Ni el sen stai ni ng, or by
i mmunofl uorescent techni ques, can be di f fi cul t. Fresh ti ssue may be needed to cul ture
mycobacteri a on Lowestei n medi um, but thi s can take 68 weeks. If there i s cl i ni cal
suspi ci on of a tubercul ous ul cer when the pati ent fi rst presents then the i ni ti al bi opsy can be
di vi ded and hal f sent for cul ture.
Management
Pati ents wi th oral tubercul osi s shoul d be ful l y i nvesti gated for pul monary or other l esi ons and
i t woul d be expected that measures such as a chest radi ograph woul d be taken at the fi rst
moment of suspi ci on. The treatment of tubercul osi s i s outsi de the provi nce of oral medi ci ne
and the appropri ate acti on to be taken woul d i nvol ve referral of the pati ent to a chest
physi ci an (or speci al i st i n i nfecti ous di seases). It shoul d be rei terated that the i ncrease of
tubercul osi s i s cl osel y rel ated to geographi cal areas af fected by the HIV epi demi c.
Fungal infections
Oral Candidosis
Candi da spp are f ungi that have a wi de di stri buti on and that f requentl y form part of the
commensal fl ora of the human body. Swabs taken f rom the ski n, gut, vagi na, or mouth of an
apparentl y heal thy i ndi vi dual al l may show the presence of Candi da speci es and, i n
parti cul ar, Candi da al bi cans. The oral carri age rate of oral candi dal speci es is about 40 per
cent of the normal popul ati on. Tabl e 4. 3 shows candi dal speci es that have been i sol ated f rom
oral l esi ons. Of these, C. al bi cans i s the most frequent.

Predisposing factors for oral candidosis
It has l ong been recogni zed that di sease due to a prol i ferati on of thi s mi cro-organi sm i s a
mark of l owered resi stance or of metabol i c change i n the pati ent. The onset of candi dosi s
shoul d therefore l ead to a search for the underl yi ng cause. The si gni fi cance of pri mary and
secondary i mmune defi ci ency i n rel ati on to candi dal i nfecti ons i s descri bed i n Chapter 14. It
i s poi nted out that, al though pri mary i mmune defi ci enci es are rel ati vel y rare, secondary
def i ci enci es are much more common. Cl i ni cal si tuati ons commonl y l eadi ng to a l oweri ng of
the i mmune defences and hence to oral candi dosi s are shown i n Tabl e 4. 4.
It shoul d be remembered that the onset of candi dosis i n an adul t pati ent represents a change
i mpl yi ng a rel axati on of the normal i mmune defences. It does not i mpl y an i nfecti on by
forei gn candi dal strai ns. Al though, i n the present context, i t i s evi dent that the oral
condi ti ons i nvol vi ng Candi da are those more ful l y di scussed, i t must be remembered that
candi dal l esi ons may i nvol ve other areas and, i ndeed, may become wi despread and
di ssemi nated i n a few debi l i tated pati ents. The condi ti on septi caemi c candi daemi a may
i nvol ve the l ungs, myocardi um, and other vi tal organs, and i s al ways a di sease of very poor
prognosi s. Apart f rom such wi despread i nfecti ons, however, i nf ecti on of the ski n, hai r
fol l i cl es, and nai l s i s rel ati vel y common. Local tissue trauma may be a si gni fi cant
predi sposi ng f eature and, i n the case of candi dal paronychi a, ci rcul atory di sturbances and
di abetes are commonl y the preci pi tati ng factors. There i s some di fference of opi ni on as to
the i nci dence of Candi da i n the vagi na. Most i nvesti gati ons have shown a rel ati vel y l ow
i nci dence i n non-pregnant pati ents, but a hi gh one (of the order of 50 per cent) i n pati ents
duri ng l ate pregnancy. Not al l these pati ents by any means show evi dence of cl i ni cal
i nfecti on, but i t i s evi dent that thi s vagi nal popul ati on of Candi da must act as a reservoi r f or
i nfecti on, parti cul arl y of the new-born baby.
C. al bi cans normal l y exi sts i n the oral cavi ty i n the form of rather l arge yeast-l i ke cel l s
(bl astospores) that occasi onal l y el ongate to form germ tubes (pseudohyphae). In the i nacti ve
state the yeast f orm i s predomi nant, but when pathol ogi cal acti vi ty occurs the hyphal f orm i s
much more evi dent. These pseudohyphae can be seen, not onl y superf i ci al l y on the oral
mucosa, but al so penetrate the epi thel i um, as f ar as the stratum granul osum. The
mechani sm by whi ch Candi da exert a pathol ogi cal eff ect on ti ssues i s not known, al though i t
has been demonstrated that proteases and extracel l ul ar protei ns may be produced by the
mi cro-organi sms and that these can i nduce ski n l esions i n the absence of the organi sms
Table 4.3 Candidal species involved in oral candidosis
C. al bi cans*
C. tropi cal i s
C. pseudotropi cal i s
C. gl abrata
C. krusei
C. parapsi l osi s
* The most frequentl y i sol ated speci es.
P. 36
themsel ves.
Table 4.4 Predisposing factors for oral candidosis
Factor Description
Physi ol ogi cal Ol d age, i nf ancy, pregnancy
Local ti ssue trauma Mucosal i rri tati on, dental appl i ances, poor oral
hygi ene
Anti bi oti c therapy Broad spectrum (l ocal or systemi c)
Corti costeroi d therapy Topi cal , systemi c, and i nhal ers
Mal nutri ti on Haemati ni c defi ci enci eshi gh-carbohydrate di et
Immune defects AIDS
Endocri ne di sorders Di abetes mel l i tus, Addi son' s di sease, hypothyroi di sm
Mal i gnanci es Leukaemi as, aganul ocytosi s
Sal i vary gl and
hypofuncti on
Irradi ati on, Sjgren' s syndrome, xerogeni c drugs
Table 4.5 The laboratory diagnosis of oral candidosis
Oral lesion Investigation*
Swab Smear Oral
rinse
Biopsy
Pseudomembranous + + -
Erythematous + + -
Hyperpl asti c - +
Laboratory investigations for the diagnosis of oral candidosis
Appropri ate l aboratory tests are outl i ned i n Tabl e 4. 5 and have al ready been di scussed i n
Chapter 2. A swab, moi stened wi th steri l e sal i ne i f necessary, i s wi ped al ong the surface of
the l esi on and pl aced i n a sui tabl e transport medi um. The sampl e shoul d be sent promptl y to
the mi crobi ol ogy l aboratory for cul ture and sensi ti vi ty. A smear of the l esi on may al so be
useful and foam pads are used i n some centres to sampl e oral l esi ons for Candi da speci es. An
oral ri nse, wi th a phosphate-buffered sal i ne sol ution, wi l l determi ne the presence of Candi da
speci es and al so provi de the cl i ni ci an wi th a quanti tati ve assessment of the candi dal count.
Thi s hel ps to di f ferenti ate between commensal i sm and opportuni sti c i nfecti ons. Bi opsy of oral
l eukopl aki a i s essenti al and wi l l demonstrate Candi da-associ ated l esi ons, such as chroni c
hyperpl asti c candi dosi s (candi dal l eukopl aki a).
Haematol ogi cal tests i ncl udi ng a ful l bl ood count, esti mati ons of serum f erri ti n, vi tami n B
12
,
fol ate (and red cel l fol ate), and a bl ood gl ucose test shoul d be undertaken for al l cases of
persi sent oral candi dosi s, parti cul arl y i f an underl yi ng systemi c condi ti on i s suspected. Oral
candi dosi s associ ated wi th HIV i nfecti on i s di scussed l ater i n thi s chapter.
Clinical classification of oral candidosis
The cl i ni cal presentati on of oral candi dosi s i s vari abl e. The ori gi nal cl assi fi cati on of oral
candi dosi s by Lehner i n the 1960s recogni zed two major groups: acute (pseudomembranous
and

atrophi c) and chroni c (atrophi c and hyperpl asti c). It i s, however, now recogni zed that thi s
ori gi nal cl assi fi cati on had l i mi tati ons, and the currentl y accepted one (Tabl e 4. 6) i s based on
cl i ni cal l y rel evant termi nol ogy. Pseudomembranous candi dosi s can be acute but,
parti cul arl y i n i mmunocompromi sed pati ents, i s of ten chroni c. Atrophi c i s not
an enti rel y appropri ate term i n the context of oral candi dosi s as the mucosa may appear red
as a resul t of ei ther atrophy or i ncreased vascul ari ty, and thi s di fferentati on i s di ffi cul t to
make cl i ni cal l y. Angul ar chei l i ti s, denture stomati ti s, and medi an rhomboi d gl ossi ti s
may be a resul t of combi ned bacteri al and f ungal aeti ol ogy and are therefore cl assi f i ed as
Candi da-associ ated l esi ons. Systemi c mucocutaneous candi dosi s f requentl y has oral
mani festati ons and these are cl assi f i ed as secondary oral candi di ases (see Tabl e
4. 6).
Angul ar chei l i ti s + + - -
Candi da-associ ated denture stomati ti s
(pal ate and denture)
+ + + -
* +, Usef ul ; -, i nappropri ate; , may be hel pf ul .
P. 37
Table 4.6 The classification of oral candidosis
Antifungal agents used to treat oral candidosis
Topi cal and systemi c anti fungal agents sui tabl e for the treatment of oral candi dosi s are
summari zed i n Tabl e 4. 7.
The pol yene anti f ungal agents, nystati n and amphoteri ci n B, are wel l establ i shed and
rel ati vel y f ree from si de-eff ects when used l ocal l y. They are avai l abl e i n vari ous forms, such
as l ozenges, pasti l l es, creams, and suspensi ons. Unf ortunatel y, pati ent compl i ance i s often
poor wi th these preparati ons, whi ch may take a whi le to di ssol ve i n the mouth (f or exampl e,
pasti l l es and l ozenges) and have a di sti ncti ve taste. Mi crobi al resi stance to pol yene
anti f ungal s i s, however, uncommon. The newer azol es have very useful properti es, al though
resi stance i s rather more commonl y met and may be probl emati c i n the future, parti cul arl y i n
the i mmunocompromi sed pati ent. (C. krusei and and C. gl abrata are usual l y resi stant to
fl uconazol e. ) The l ocal l y acti ve agent, mi conazol e i s avai l abl e as an oral gel or cream. As
wel l as i ts anti fungal acti vi ty, i t has a l i mi ted anti bacteri al ef fect. The systemi cal l y acti ng
azol es, i traconazol e, f l uconazol e, and ketoconazol e, are of val ue i n general i zed and systemi c
candi dal i nfecti ons. There may be changes i n l i ver functi on, parti cul arl y wi th ketoconazol e,
and pati ents on these drugs must be careful l y moni tored. Systemi c therapy i s general l y not
necessary i n the majori ty of pati ents suff eri ng f rom oral candi dosi s but i n some condi ti ons,
such as chroni c hyperpl asti c candi dosi s, i ts use i s becomi ng wi despread and i s often very
eff ecti ve. Azol es have an unfavourabl e pharmacoki neti c i nteracti on wi th a number of drugs
i ncl udi ng anti -coagul ants (for exampl e, warfari n), terfenadi ne, ci cl ospori n, stati ns, ci sapri de,
and astemi zol e. Chl orhexi di ne sol uti on has anti bacteri al and anti candi dal eff ects.
Pri mary oral candi doses (group 1)
Acute: pseudomembranous, erythematous
Chroni c: pseudomembranous, erythematous, hyperpl asti c
Candi da-associ ated l esi ons: Candi da-associ ated denture-i nduced stomati ti s, angul ar
chei l i ti s, medi an rhomboi d gl ossi ti s
Secondary oral candi doses (group 2)
Oral mani f estati ons of systemi c mucocutaneous candi dosi s
Table 4.7 The treatment of primary oral candidoses in
immunocompetent patients
Topical therapy Systemic therapy
Pseudomembranous, erythematous hyperpl asti c candi dosi s
Amphoteri ci n l ozenges (10 mg) Fl uconazol e, 50100 mg dai l y for
23 weeks
Pseudomembranous candidosis
Unti l recentl y, thi s condi ti on, col l oqui al l y known as thrush, was al most al ways
descri bed as acute pseudomembranous candi dosi s, accordi ng to Lehner' s ori gi nal
cl assi fi cati on, even though many l esi ons were mani festl y chroni c i n nature. Reconsi derati on
of the oral l esi ons of candi dosi s i n HIV-rel ated condi ti ons and other

i mmunosuppressi ve states has l ed to the routi ne use of the term acute bei ng
abandoned. The pseudomembrane consi sts of a network of candi dal hyphae contai ni ng
desquamated cel l s, mi cro-organi sms, fi bri n, i nfl ammatory cel l s, and debri s. Thi s
or or
Nystati n pasti l l es (100 000 uni ts)
Di ssol ve sl owl y i n mouth, after meal s; use
4
ti mes dai l y; usual course i s14 weeks.
Itraconazol e 150 mg dai l y f or 2
weeks
Candi da-associ ated denture stomati ti s*
Amphoteri ci n or nystati n (as above)
remove dentures
If compl i ance poor:
Mi conazol e gel appl i ed to pal atal surf ace of
denture
4 ti mes dai l y for 14 weeks
Mi conazol e l acquer
Chl orhexi di ne 0. 2% ri nse, 4 ti mes dai l y (do
not use wi th nystati n)
Systemi c therapy i s occasi onal l y
requi red (as above)
Candi da-associ ated angul ar chei l i ti s*
Nystati n cream; appl y to corners of mouth
34
ti mes dai l y, unti l resol uti on
If mi crobi al report not avai l abl e or i n case
of mi xed i nfecti on:
Mi conazol e cream (or gel ); appl y 34
ti mes dai l y to angl es
Systemi c therapy may be requi red
* Intraoral reservoi rs of Candi da shoul d be el i mi nated from pati ents wi th angul ar
chei l i ti s and denture stomati ti s.
Mi conazol e l acquer (Dumi coat, 50 mg/g) can be appl i ed to f i tti ng surface of
upper denture after thorough cl eansi ng and dryi ngsee manuf acturers
i nstructi ons.
If Staphyl ococcus aureus onl y i sol ated, fusi di c aci d cream i s requi red (see Tabl e
6. 5 for f urther detai l s).
P. 38
pseudomembrane l i es on the surface of the ti ssue and candi dal hyphae penetrate
superfi ci al l y i nto the epi thel i um to provi de anchorage.
CLINICAL FEATURES
Cl i ni cal l y, thi s appears as a thi ck, whi te coati ng or seri es of patches on the af fected ti ssue
(Fi g. 4. 4). The pseudomembrane can be wi ped away and, si nce the more superfi ci al l ayers of
the epi thel i um may be i ncl uded, a red and bl eedi ng base i s l eft behi nd. Thi s may be
consi dered a reasonabl e prel i mi nary cl i ni cal test to di sti ngui sh thrush f rom other whi te
l esi ons of the mucosa and conf i rmati on may al so be obtai ned by taki ng a di rect smear from
the l esi on. Thi s may be fi xed by gentl e heat and i mmedi atel y stai ned, usi ng the peri odi c
aci dSchi f f (PAS) reagent. The hyphae are readi l y i denti f i ed under the mi croscope. A swab
shoul d be taken and sent to the mi crobi ol ogi cal l aboratory for cul ture and sensi ti vi ty.
Any of the mucosal surfaces of the mouth may be affected by thrush, as may the posteri or
pharyngeal wal l . In thi s l ast i nstance, the condi ti on must be taken parti cul arl y seri ousl y,
si nce extensi on i nto the oesophagus and trachea i s possi bl e and may prove f atal . Thi s i s
l i kel y to occur onl y i n the severel y debi l i tated pati ent, al though the pre-exi sti ng condi ti on
may not have been previ ousl y recogni zed. In a few pati ents l aryngeal candi dosi s of a l ess
acti ve f orm may be associ ated wi th oral l esi ons. Thi s has been noted i n pati ents taki ng
anti bi oti cs and i n those taki ng oral steroi d preparati ons. In general , the l aryngeal di scomfort
and hoarseness resol ve wi th treatment of the oral lesi ons.
MANAGEMENT
The treatment for pseudomembranous candi dosi s i s summari zed i n Tabl e 4. 7. It i s evi dent,
however, that treatment of symptoms al one i s i nsuf fi ci ent and that steps must be taken to
determi ne any predi sposi ng cause. In some pati ents the essenti al underl yi ng f actor i s
understood and under reasonabl e control , but i n others, i n parti cul ar those pati ents wi th
AIDS, the debi l i tati ng nature of the condi ti on may be expressed i n vi rtual l y conti nuous
candi dosi s of the mouth. In these ci rcumstances i t i s necessary to mai ntai n l ong-term
anti f ungal treatment. A parti cul ar di f fi cul ty ari ses i n the case of pati ents wi th l eukaemi a or
other neopl asti c di seases under treatment wi th cytotoxi c and other drugs. In these cases not
onl y the underl yi ng di sease process, but al so the therapy, tend to predi spose towards
Fig. 4.4 Pseudomembranous candi dosi s (thrush).
candi dosi s.
Erythematous candidosis
Thi s can be acute or chroni c dependi ng on the durati on of the oral l esi ons.
Acute erythematous candi dosi s was formerl y known as acute atrophi c candi dosi s or
anti bi oti c sore mouth and usual l y occurred as a resul t of medi cal treatment i nvol vi ng
anti bi oti cs or steroi d preparati ons. A si gni fi cant number of pati ents (adul ts and chi l dren) are
now usi ng steroi d i nhal ers for pul monary di sease and these can predi pose to the
devel opment of ei ther erythematous or pseudomembranous candi dosi s (Chapter 14).
CLINICAL FEATURES
Acute erythematous candi dosi s resembl es thrush wi thout the overl yi ng
pseudomembrane. Cl i ni cal l y, the mucosa i nvol ved i s red and pai nful . Thi s i s, i n fact, the onl y
vari ant of oral candi dosi s i n whi ch pai n and di scomfort i s marked. As woul d be expected from
thi s descri pti on, the epi thel i um i s thi n and atrophi c wi th candi dal hyphae embedded
superfi ci al l y i n the epi thel i um. The condi ti on may fol l ow or be concurrent wi th thrush, or may
occur as the onl y mani festati on of the i nf ecti on. As has been poi nted out above, thi s form of
candi dosi s i s common i n pati ents wi th the suppressed i mmune functi on of AIDS as wel l as
pati ents undergoi ng prol onged anti bi oti c or steroi d therapy. It i s not al ways possi bl e for
pati ents on steroi ds to di sconti nue treatment and the conti nuous l oweri ng of the resi stance
of the ti ssues to i nfecti on makes treatment by the usual means rel ati vel y i nef fecti ve.
MANAGEMENT
Topi cal anti f ungal therapy (Tabl e 4. 7) i s usual l y ef fecti ve. Erythematous candi dosi s as a
resul t of anti bi oti c therapy usual l y resol ves af ter cessati on of the course, al though thi s may
take some ti me. In a pati ent known to be suscepti ble to thi s condi ti on and for whom i t i s
necessary to prescri be further anti bi oti c treatment, i t woul d be wi se to add oral anti fungal
treatment to the regi me. In the authors experi ence, a mi nori ty of these pati ents appear
to devel op a chroni c gl ossodyni a (burni ng tongue) that persi sts despi te the l i ngual mucosa
appeari ng normal and af ter el i mi nati on of candi dal i nfecti on (see Chapter 17). Pati ents who
regul arl y requi re a steroi d i nhal er shoul d be i nstructed to ri nse thei r mouth wi th water after
use. Use of a spacer devi ce for the i nhal er may be necessary (Chapter 14, Fi g. 14. 2).
Hyperplastic candidosis
Chroni c hyperpl asti c candi dosi s i s al so known as candi dal l eukopl aki a and i s
associ ated wi th chroni c i nfecti on of the oral mucosa

wi th candi dal speci es, usual l y C. al bi cans. Thi s chroni c form of candi dosi s i s consi dered to be
a premal i gnant l esi on and i s more l i kel y to occur in pati ents who smoke. Currentl y, there i s a
great deal of controversy concerni ng the rol e of Candi da speci es i n the devel opment of
epi thel i al neopl asi a. The premal i gnant nature of candi dal l eukopl aki a i s di scussed f ul l y i n
Chapter 10.
CLINICAL FEATURES
Chroni c hyperpl asti c candi dosi s cl assi cal l y presents as a fi xed whi te patch at the
commi ssures of the mouth. Other areas of the mouth, parti cul arl y the pal ate, may be
aff ected but the tongue i s l ess commonl y i nvol ved. Cl i ni cal l y, the l esi on(s) appear as rai sed,
i rregul ar whi te pl aques, whi ch may be speckl ed or nodul ar i n appearance. There i s
frequentl y evi dence of oral candi dosi s el sewhere, parti cul arl y on the pal ate of pati ents wi th
ful l dentures and/or angul ar chei l i ti s.
MANAGEMENT
P. 39
Defi ni ti ve di agnosi s depends on hi stopathol ogi cal exami nati on of a bi opsy, whi ch i s essenti al
to confi rm the presence of Candi da speci es and to ascertai n the degree of epi thel i al
atypi ai f any. Management i nvol ves the eradi cati on of predi sposi ng factors, such as
smoki ng and i nsti tuti on of appropri ate anti f ungal therapyei ther topi cal or systemi c. An
i ni ti al course of f l uconazol e for 23 weeks can be combi ned wi th topi cal therapy (see Tabl e
4. 7), whi ch may be requi red on a l onger-term basi s. Attenti on must be gi ven to denture
hygi ene and other factors predi sposi ng to oral candi dosi s, ei ther l ocal or systemi c.
Haematol ogi cal i nvesti gati ons shoul d be undertaken to check for haemati ni c defi ci enci es such
as i ron defi ci ency, and a bl ood gl ucose test i s advi sabl e to excl ude di abetes. Exci si on of
persi stent l esi ons or those wi th a si gni fi cant degree of dyspl asi a i s preferabl y carri ed out
wi th a l aser. Long-term fol l ow-up i s essenti al .
Candida-associated, denture-induced stomatitis
Thi s i s by f ar the most common form of oral candi dosi s and i s al so referred to as chroni c
erythematous candi dosi s (or, more col l oqui al l y i n the past, as denture sore
moutha mi snomer because the condi ti on i s nearl y al ways pai nl ess). Thi s f orm of
denture stomati ti s represents the end-resul t of secondary candi dal i nfecti on of ti ssues,
traumati zed by a dental appl i ance. Thi s need not be i l l -fi tti ng, al though most of the dentures
i nvol ved are ol d. The appl i ance i s commonl y but not excl usi vel y a denturean orthodonti c
pl ate may produce a si mi l ar resul t.
CLINICAL FEATURES
The cl i ni cal pi cture i s of marked redness of the pal atal mucosa covered by the appl i ance (the
equi val ent mandi bul ar denture-beari ng area does not appear to become i nvol ved) of ten wi th
a sharpl y defi ned edge (Fi g. 4. 5). If a rel i ef area i s present on the denture, there may be a
correspondi ng area of spongy granul ar l ooki ng ti ssue, but otherwi se the af fected
mucosa i s smooth. Newton i ntroduced a cl assi fi cati on f or thi s condi ti on: type 1, characteri zed
by pi n-poi nt hyperaemi a of the pal atal mucosa; type 2 (the most common type), i n whi ch
there i s di ff use erythema l i mi ted to the area of the denture; type 3, i n whi ch there i s a
hyperpl asti c nodul ar reacti on of the pal atal mucosa (Tabl e 4. 8). There i s rarel y any compl ai nt
of soreness by the pati ent i n spi te of the i ntense erythematous appearance of the ti ssues.
Candi da-associ ated denture stomati ti ts, because of i ts restri cted di stri buti on to the denture-
beari ng area, i s someti mes mi staken for an al l ergi c reacti on to acryl i c resi n, a condi ti on that
i s rel ati vel y rare.
MANAGEMENT
By taki ng swabs or carryi ng out di rect i nocul ati on from the fi tti ng surface of the denture
i nvol ved i t i s practi cal l y al ways possi bl e to i sol ate a heavy growth of Candi da. Swabs from
the mucosal surface may al so provi de a prol i fi c growth. Bi opsy i s rarel y i ndi cated but may be
done i n persi stent or atypi cal cases to confi rm the di agnosi s. Hi stopathol ogy often shows few
candi dal hyphae wi thi n the epi thel i um.
Si nce there are two contri buti ng factors to thi s condi ti on (ti ssue trauma and i nfecti on),
treatment must be di rected at both i n order to obtai n rapi d resol uti on of symptoms. Denture
hygi ene i s an i mportant part of the treatment together wi th the el i mi nati on of trauma by the
adopti on of sui tabl e prostheti c techni ques, the use of ti ssue condi ti oners bei ng parti cul arl y
val uabl e as an i ni ti al treatment. The use of an anti f ungal cream or mi conazol e

gel on the fi tti ng surface of the appl i ance may prove a useful adjuvant to treatment and
hel ps to speed the resol uti on of the abnormal ti ssues (Tabl e 4. 7). Caref ul and regul ar
cl eani ng of al l surfaces of the denture i s al so of great i mportance. A l acquer contai ni ng
mi conazol e i s now avai l abl e and pai nted on to the fi tti ng surface of the upper denture.
Fig. 4.5 Candi da-associ ated, denture-i nduced stomati ti s (chroni c erythematous
candi dosi s) aff ecti ng the area of mucosa covered by a parti al denture.
P. 40
Table 4.8 Newton's classification of Candida-associated
denture stomatitis
Type Description
1 Pi n-poi nt hyperaemi a
2* Di ff use erythema, l i mi ted to the fi tti ng surfaces of denture
3 Nodul ar appearance of pal atal mucosa
* Type most commonl y seen.
Candida-associated angular cheilitis
Angul ar chei l i ti s presents as erythema and cracki ng at the angl es of the mouth and i s
commonl y a Candi da-associ ated l esi on (Fi g. 4. 6). The pathogenesi s, cl i ni cal presentati on,
and management of angul ar chei l i ti s are ful l y descri bed i n Chapter 6. The treatment of
Candi da-associ ated angul ar chei l i ti s i s summari zed i n Tables 6. 4 and 6. 5.
Median rhomboid glossitis
Medi an rhomboi d gl ossi ti s i s cl assi f i ed as a Candi da-associ ated l esi on and
characteri sti cal l y presents as an area of depapi l l ati on on the mi dl i ne of the dorsum of the
tongue, i mmedi atel y i n front of the ci rcumval l ate papi l l ae. The l esi on i s cl assi cal l y
rhomboi d-shapedhence the name. Its suface may be red, whi te, or yel l ow i n
appearance. Thi s l esi on i s further di scussed i n Chapter 6.
Secondary oral candidoses: chronic mucocutaneous candidosis
syndromes
Apart from the more common forms of oral candi dosi s, there i s a spectrum of condi ti ons i n
whi ch candi dosi s of the oral cavi ty, the ski n, and other structures such as the fi ngernai l s
may occur, wi th or wi thout associ ati on wi th other general i zed di sease processes. Thi s group
of condi ti ons i s general l y known as chroni c mucocutaneous candi dosi s (CMC). The oral
l esi ons i n CMC may be i ni ti al l y thrush-l i ke, but eventual l y resembl e l esi ons of chroni c
hyperpl asti c candi dosi s. The ski n l esi ons may i ncl ude wi despread and di sfi guri ng l esi ons of
the face and scal p. Granul omatous l esi ons of the l ips may al so occur, si mi l ar to those that
aff ect the ski n. A vari ant (fami l i al chroni c mucocutaneous candi dosi s) i s geneti cal l y
determi ned and i s transmi tted as an autosomal recessi ve condi ti on. In the
candi dosi sendocri nopathy syndrome whi te candi dal pl aques i n the mouth and candi dal
i nfecti ons of the nai l s are associ ated wi th di sorders of the parathyroi ds or adrenal s. A
cl assi fi cati on of chroni c mucocutaneous candi dosi s syndromes i s shown i n Tabl e 4. 9.
Fig. 4.6 Angul ar chei l i ti s wi th candi dal i nvol vement.
Table 4.9 Chronic mucocutaneous candidosis (CMC)
MANAGEMENT
Chroni c mucocutaneous candi dosi s i s di ffi cul t to treat and i n many cases reducti on, but not
eradi cati on, of Candi da speci es i s attai nabl e. Predi sposi ng factors for oral candi dosi s shoul d,
however, be el i mi nated (see above) wherever possi ble. Treatment i s wi th l ong-term systemi c
anti f ungal therapy such as f l uconazol e and may need to be prescri bed at doses hi gher than
the normal therapeuti c range. Regul ar moni tori ng of l i ver functi on i s essenti al .
Viral infections
A number of vi ral i nf ecti ons may occur i n and around the mouth and many of these present
as vesci ul ar l esi ons of the oral mucosa. The vesi cl es show a marked tendency to break down
wi th the producti on of ul cers.
Di agnosti c tests for vi ral i nfecti ons have al ready been di scussed i n Chapter 2. The i ni ti al
di agnosi s of vi ral i nf ecti on i s, however, usual l y on the basi s of cl i ni cal presentati on. Ti ssue
cul ture systems take at l east 24 hours to conf i rm the presence of a vi rus and serol ogi cal
tests take 1014 days. Tabl e 4. 10 shows the pri nci pal vi ruses that aff ect the oral and
peri oral regi on. Of these, three are human herpes vi rsuses (Tabl e 4. 11). There are currentl y
ei ght human herpes vi ruses (Tabl e 4. 11) and al l are envel oped, i cosahedral vi ral parti cl es,
wi th a doubl e-stranded DNA genome.
The human herpes vi ruses possess the property of l atency (that i s, they can remai n dormant
and become reacti vated l ater). The cl i ni cal di sease caused by the vi rus may di ff er dependi ng
on whether the i nf ecti on i s pri mary or secondary (that i s, a reacti vati on).
syndromes
Type Features
Fami l i al CMC Fi rst decadepersi stent candi dosi s: mouth, nai l s,
ski n. Iron defi ci ency.
Di ff use CMC
(Candi da granul oma)
Fi rst 5 yearschroni c candi dosi s: mouth, nai l s, ski n,
pharynx. Suscepti bl e to bacteri al i nf ecti ons
Candi dosi s-
endocri nopathy
syndrome
Hypothyroi di sm, hypoadrenocorti ci sm, and mi l d
chroni c hyperpl asti c candi dosi s i nvol vi ng the mouth
Candi dosi s-thymoma
syndrome
Haematol ogi cal di sorders.
Pseudomembranous or hyperpl asti c candi dosi s: mouth,
ski n, nai l s.
* Taken from Bagg, T. et al . (1999). Essenti al s of mi crobi ol ogy for dental students.
Oxf ord Uni versi ty Press, Oxford.
Table 4.10 Principal viruses that affect the oral and

Herpes simplex virus infections
Primary herpetic gingivostomatitis
The herpes si mpl ex vi rus (HSV) i s a DNA vi rus of whi ch two mai n groups are known i n man,
together wi th a number of sl i ghtl y modi fi ed transi ti onal types. Herpes si mpl ex, type 1,
aff ects the oral mucosa, pharynx, and ski n whereas Herpes si mpl ex, type 2, predomi nantl y
i nvol ves the geni tal i a. The l esi ons produced by these two types of the vi rus appear to be
i denti cal , but there are suggesti ons that the l ong-term consequences of i nfecti on may be
di f ferent si nce the type 2 vi rus has been i mpl i cated i n the producti on of cervi cal carci noma.
The type 1 vi rus i s i mpl i cated i n the majori ty of oral and faci al l esi ons.
Pri mary herpeti c gi ngi vostomati ti s i s the most common vi ral i nfecti on aff ecti ng the mouth
and i t aff ects pati ents i n two mai n age groupsyoung chi l dren and young adul ts. In earl y
chi l dhood the i nfecti on may be subcl i ni cal . The i ncubati on peri od i s about 5 days.
CLINICAL PRESENTATION
perioral region
Herpes si mpl ex vi rus
Vari cel l a zoster vi rus
Epstei nBarr vi rus
Group A Coxsacki evi ruses
Paramyxovi ruses
Human papi l l oma vi ruses
Table 4.11 Human herpes viruses
Herpes si mpl ex vi rus (type 1)
Herpes si mpl ex vi rus (type 2)
Vari cel l a-zoster vi rus (type 3)
Epstei n-Barr vi rus (type 4)
Cytomegal ovi rus (type 5)
Human herpes vi ruses 6, 7, and 8*
* Associ ated wi th Kaposi ' s sarcoma.
P. 41
The pati ent wi th pri mary herpeti c gi ngi vostomati ti s may gi ve a hi story of recent exposure to
a pati ent wi th a herpeti c l esi on. The i ni ti al symptoms are of mal ai se wi th ti redness,
general i zed muscl e aches, and, someti mes, a sore throat. At thi s earl y stage the
submandi bul ar l ymph nodes are often enl arged and tender. Thi s prodromal phase may be
expected to l ast f or a day or two and i s fol l owed by the appearance of oral and, someti mes,
ci rcumoral l esi ons (Fi g. 4. 7). Groups of vesi cl es form on the oral mucosa and rapi dl y break
down to produce shal l ow ul cers. Al though the vesi cles may be rel ati vel y smal l , the
breakdown of conf l uent groups may resul t i n the formati on of l arge areas of ul cerati on. The
di stri buti on of the l esi ons i s vari abl eany of the oral mucosal surfaces may be i nvol ved.
Apart from the ul cerated areas, the whol e of the oral mucosa may be bri ght-red and sore. In
young chi l dren, parti cul arl y, there may be a marked gi ngi vi ti s that cl osel y resembl es that of
acute l eukaemi a. It i s evi dent that such an appearance shoul d i ndi cate screeni ng for
haematol ogi cal changes. Apart from the i ntraoral l esi ons, there may be l esi ons of the l i ps
and ci rcumoral ski n, whi ch, because of the rel ati ve stabi l i ty of the ski n compared to the oral
mucosa, may retai n a much more obvi ousl y vesi cul ar appearance. In a few cases, the
pri mary i nfecti on may become wi despread and di sseminated throughout the body, so that
encephal i ti s, meni ngi ti s, and other l i fe-endangeri ng condi ti ons may fol l ow. Such potenti al l y
fatal cases have been mai nl y reported i n pati ents who are i mmunocompromi sed.
In the vast majori ty of untreated cases there i s a sl ow recovery f rom the symptoms over a
peri od of some 1014 days. Most cases of pri mary herpeti c gi ngi vostomati ti s are di agnosed
on cl i ni cal grounds. HSV can be grown i n ti ssue culture systems but thi s takes at l east 24
hours. Serol ogy can al so be used to detect ei ther an i ncrease i n the IgE anti body ti tre
between the acute and conval escent phase, or speci fi c IgM anti bodi es.
MANAGEMENT
Speci f i c therapy f or vi ral i nf ecti ons i s general l y unsati sf actory and management i s, therefore,
symptomati c wi th general supporti ve measures, such as rest, fl ui ds, and
anti pyreti cs/anal gesi cs. For oral i nfecti ons, the use of an anti septi c (for exampl e,
chl orhexi di ne gl uconate) or tetracycl i ne mouthwash may reduce secondary i nfecti on and an
anal gesi c mouthwash (for exampl e, benzydami ne) wi l l reduce di scomfort, parti cul arl y whi l st
the pati ent i s eati ng. Systemi c anal gesi cs and anti ypreti cs may al so be requi red. In most
Fig. 4.7 Pati ent sufferi ng from pri mary herpeti c gi ngi vostomati ti s wi th i nvol vement of
the l i ps and peri oral ski n.
i mmunocompetent pati ents, HSV i nf ecti ons are sel f-li mi ti ng and l ast 1014 days.

Management of most vi ral i nfecti ons aff ecti ng the mouth i s symptomati cgeneral
supporti ve measures i ncl ude:
rest
fl ui ds
anti pyreti cs/anal gesi cs (systemi c)
anti septi c/anal gesi c mouthwashes
Aci cl ovi r i s acti ve agai nst herpes vi rses but does not eradi cate them. It can be used f or the
systemi c and topi cal treatment of herpes si mpl ex i nfecti ons aff ecti ng the ski n and mucous
membranes. Prophyl acti c use of aci cl ovi r for preventi on and recurrence of HSV i nfecti ons i s
justi f i ed i n the i mmunocompromi sed.
Systemi c anti vi ral therapy i s essenti al i f the patient i s i mmunocompromi sed, as thi s group i s
more suscepti bl e to general di ssemi nati on of the HSV vi rus. In these cases, the cl i ni cal
presentati on of the i nfecti on may be atypi cal . An acute herpeti c stomati ti s represents the
pri mary i nfecti on and, al though subsequent i mmuni ty may not be compl ete, i t i s onl y very
rarel y that a second acute attack fol l ows, unl ess the pati ent i s i mmunocompri sed. Recurrent
i ntraoral herpes i nfecti ons are therefore rare i n the i mmunocompetent pati ent but crops of
vesi cl es restri cted to part of the oral mucosa, parti cul arl y the pal ate, have been descri bed.
If the si gns and symptoms of pri mary herpeti c gi ngi vostomati ti s fai l to i mprove after 2
weeks, then the pati ent shoul d be ref erred for a speci al i st opi ni on to excl ude bl ood
dyscrasi as and other underl yi ng systemi c condi ti ons.
Secondary (recurrent) herpes simplex infections
Fol l owi ng resol uti on of the pri mary herpeti c i nfecti on, there i s an approxi matel y 3040 per
cent l i kel i hood that recurrent l esi ons wi l l f ol l ow, regardl ess of the i ntensi ty of the pri mary
attack. The usual si te of the recurrent l esi on i s on or near the l i ps and the l esi on i s known as
herpes l abi al i s or a col d sore. Less commonl y, the ski n and mucosa of the
nose and nasal passages are i nvol ved, as may be, occasi onal l y, al most any si te on the face.
In an i ndi vi dual pati ent, however, the areas i nvol ved tend to remai n the same i n successi ve
epi sodes. The recurrences may be provoked by a wi de range of sti mul ae, i ncl udi ng sunl i ght,
mechani cal trauma, and, parti cul arl y, mi l d febri l e condi ti ons such as the common col d.
Emoti onal f actors al so pl ay a part i n preci pi tati ng recurrences i n many pati ents. HSV
parti cl es can be detected i n the sal i va of approxi matel y 30 per cent of pati ents who devel op
col d sores.
In the prodromal stages of a recurrence the pati ent may f eel a mi l d degree of ti redness and
mal ai se. Thi s i s qui ckl y fol l owed by a peri od of i rri tati on and i tchi ng over the area of the l i p
i nvol ved i n recurrence and, wi thi n a f ew hours, vesi cl es appear surrounded by a mi l dl y
erythematous area (at thi s stage the pati ent i s hi ghl y i nf ecti ous). In a short ti me the vesi cl es
burst and a scab i s f ormed. From thi s poi nt the process i s one of sl ow heal i ng over a peri od
of some 10 days, but secondary i nfecti on may occasi onal l y del ay the heal i ng process and
l ead to the producti on of smal l pustul es i n the area. Heal i ng i s wi thout scarri ng and the
aff ected area returns, af ter a short peri od of erythema, to an apparentl y normal stage unti l
the onset of the next recurrence. Thi s may be after a peri od of a few weeks or even days i n
some i ndi vi dual s, but general l y the i nterval s between recurrences are on the order of
months, thi s evi dentl y dependi ng to a l arge extent on the degree of exposure of the pati ent
P. 42
to the parti cul ar sti mul us i nvol ved.
MANAGEMENT
There i s no therapeuti c measure avai l abl e that provi des reasonabl y consi stent resul ts and
that enti rel y prevents further recurrences. Aci cl ovi r cream, used as earl y as possi bl e and at
l east fi ve ti mes a day i n the earl y stages, may prevent progressi on of the l esi on. Thi s i s now
perhaps the most eff ecti ve and acceptabl e form of treatment for recurrent herpes, al though
there i s no real suggesti on that a permanent cure may be obtai ned. A si mi l ar anti vi ral drug,
penci cl ovi r, can be used topi cal l y f or col d sores, (herpes l abi al i s). The prodrug of
penci cl ovi r i s famci cl ovi r, whi ch i s a systemi c anti vi ral agent recommended for HZV i nfecti ons
and geni tal herpes.
Topi cal therapy for col d sores (herpes l abi al i s) shoul d be appl i ed in the prodromal
phase of the l esi on. Aci cl ovi r and penci cl ovi r creams are avai l abl e for topi cal appl i cati on.
Varicella zoster virus infections
Vari cel l a zoster vi rus (VZV) i s a DNA vi rus morphologi cal l y si mi l ar to the HSV and apparentl y
responsi bl e for two compl etel y di ssi mi l ar di seases i n humans, namel y, chi ckenpox and herpes
zoster. There i s, however, l i ttl e evi dence that contact wi th one of these di seases i s
responsi bl e for the i ni ti ati on of the other. It seems more l i kel y that the zoster erupti on
represents the reacti vati on of the vi rus i n a previ ousl y i nf ected pati ent wi th onl y parti al
i mmuni ty, a si tuati on paral l el to that i n recurrent herpes si mpl ex. The zoster vi rus i s al so
thought to remai n l atent i n the rel evant sensory gangl i on and to pass down the nerve to the
ski n or mucous membrane on reacti vati on.
Chickenpox
Pri mary i nfecti on wi th VZV mani f ests as chi ckenpox, whi ch i s a common di sease of chi l dhood.
It has an i ncubati on peri od of 1421 days, duri ng whi ch pati ents are i nfecti ous. The
i nfecti on i s spread by di rect contact or dropl et i nfecti on and i s establ i shed f i rst i n the upper
respi ratory tract.
Just bef ore the rash, oral l esi ons may appear on the hard pal ate, pi l l ar of fauces, and uvul a
and appear as smal l ul cers, wi th a red hal o. The rash i ni ti al l y mani fests as pi nk,
macul opapul ar l esi ons that devel op i nto i tchy vesi cl es on the back, chest, face, and scal p.
Other cl i ni cal mani f estati ons i ncl ude mal ai se, fever, and l ymphadenopathy. Adul ts can be
qui te severel y af fected. Rare compl i cati ons of chi ckenpox i ncl ude pneumoni a and
encephal i ti s. Treatment i s symptomati c but i t i s i mportant f or the pati ent to avoi d contact
wi th any i mmunocompromi sed i ndi vi dual s.

Shingles
Most pati ents wi th herpes zoster are mi ddl e-aged or ol der (70 per cent over the age of 50),
but i t can occur i n much younger pati ents and a few neonatal cases have been descri bed.
Predi sposi ng factors that have been suggested i ncl ude a wi de range of debi l i tati ng di seases
and i mmunosupressi on, ei ther of i atrogeni c or natural l y occurri ng ori gi n.
The characteri sti c superfi ci al l esi on of herpes zoster i s a vesi cul ar erupti on i n an area of
di stri buti on of a sensory nerve. The band-l i ke di stri buti on of shi ngl es on the trunk i s wel l
known, havi ng gi ven the name to the vi rus (zoster, gi rdl e). When the erupti on affects the
P. 43
tri gemi nal nerve, the faci al ski n and oral mucosa in the sensory area may be af fected. The
tri gemi nal nerve i s i nvol ved i n about 15 per cent of cases. Of the three di vi si ons of the
tri gemi nal nerve the ophthal mi c i s the most f requentl y i nvol ved, but the other two di vi si ons
are al so not uncommonl y aff ected. Occasi onal l y, when the di sorder af fects one di vi si on of the
tri gemi nal nerve, the adjacent di vi si on becomes i nvol ved as wel l . Cervi cal nerves may al so
be af fected. The i ni ti al symptoms are of pai n and tenderness i n the affected areaa
di scomf ort much more severe than that experi enced in HSV i nfecti on. The prodromal pai n of
shi ngl es i s severe and i s occasi onal l y mi sdi agnosed as toothache. The prodromal phase may
l ast f or 2 or 3 days and i s succeeded by the appearance of vesi cl es i n a rash, whi ch may be
ei ther sparse or so dense as to be al most confl uent. Frequentl y, the vesi cl es appear over a
peri od of days rather than together, and these may become secondari l y i nf ected. When the
ophthal mi c di vi si on of the tri gemi nal nerve i s i nvol ved there may be corneal ul cerati on,
whi ch requi res most careful management to avoi d permanent scar f ormati on. Wi thi n the
mouth, the vesi cl es behave much as those of herpes si mpl ex i nfecti ons, rapi dl y breaki ng
down to form ul cers. The uni l ateral di stri buti on of the oral l esi ons, and conf i nement to the
area of a si ngl e branch of the tri gemi nal nerve, may gi ve a cl ue as to the nature of the
i nfecti on but thi s cannot be rel i ed upon. If untreated, the vesi cl es and oral ul cerati on f ade
over a vari abl e peri od from 2 to 4 weeks, or even l onger. The ski n vesi cl es may form fi rm
crusts and, parti cul arl y i f these are di sturbed, marked scarri ng may occur. Fol l owi ng the
fadi ng of the rash the major compl i cati on of the condi ti onpostherpeti c neural gi amay
appear. In thi s condi ti on anaesthesi a, paraesthesi a, and tri gemi nal neural gi a-l i ke pai ns may
aff ect the area and may persi st f or a peri od of years, occasi onal l y reappeari ng af ter a
prol onged absence. Thi s i s a hi ghl y refractory condi ti on that may fai l to respond to any form
of medi cal treatment (see Chapter 15). In a very f ew pati ents the faci al nerve may become
i nvol ved duri ng an epi sode of zoster reacti vati on, probabl y vi a the geni cul ate gangl i on. Faci al
weakness, l oss of taste sensati on, and symptoms such as di zzi ness resul ti ng from
l abyri nthi ne di sturbance may occur. Thi s i s the Ramsay Hunt syndrome (Tabl e 4. 12).
Vesi cul ar l esi ons i n thi s condi ti on are most often seen on the pal ate and around the external
audi tory meatus. In most cases, thi s i s a sel f -l i miti ng condi ti on that resol ves wi th restorati on
of functi on, but i n some pati ents there may be permanent faci al weakness.
MANAGEMENT
Treatment of shi ngl es i s wi th hi gh doses of systemic aci cl ovi r or famci cl ovi r. Thi s i s
parti cul arl y i mportant when the ophthal mi c di vi si on of the tri gemi nal nerve i s aff ected. There
i s some evi dence that earl y use of systemi c anti vi ral therapy may reduce the posti nfecti ve
Table 4.12 Ramsay Hunt syndrome
Invol vement of f aci al nerve wi th VZV
Faci al nerve pal sy (l ower motor neurone)
Vesi cl es i n external audi tory meatus
Vesi cl es on pal ate
Other symptoms, e.g. di zzi ness, l oss of taste
compl i cati on of postherpeti c neural gi a (Chapter 15). In the case of the oral l esi ons, topi cal
treatment as f or herpes si mpl ex stomati ti s (tetracycl i ne mouthwash) i s used, wi th the same
rati onal e and wi th si mi l ar, i f sl ower, resul ts. Perhaps one of the most i mportant functi ons of
the cl i ni ci an faced wi th a case of herpes zoster i s to be aware of the possi bi l i ty of corneal
i nvol vement and to pl ace the pati ent under appropriate care i f any symptoms, however mi l d,
appear.
Shi ngl es (parti cul arl y i f aff ecti ng the tri gemi nal nerve) shoul d be treated as soon as possi bl e
by systemi c anti vi ral therapy; such as aci cl ovi r or famci cl ovi r.
EpsteinBarr virus infection
The Epstei nBarr vi rus (EBV) pl ays a part i n the pathogenesi s of a number of condi ti ons
aff ecti ng the orof aci al regi on, i ncl udi ng i nf ecti ous mononucl eosi s (gl andul ar fever). The EBV
i s consi dered to be an oncogeni c vi rus and associ ated wi th the f ormati on of mal i gnant
l ymphomas (Burki tt' s l ymphoma) i n Afri ca and l i nked to nasopharyngeal carci noma,
parti cul arl y i n southern Chi na. The associ ati on of EBV wi th oral hai ry l eukopl aki a i n
i mmunocompromi sed pati ents, parti cul arl y those wi th HIV i nfecti on, i s di scussed i n the
secti on on AIDS.
Infectious mononucleosis
Thi s i s a rel ati vel y common i nfecti on, usual l y mani festi ng symptoms i n earl y adul thood.
Chi l dren may suf fer a subcl i ni cal i nfecti on. The diagnosi s i s confi rmed by l aboratory tests,
whi ch shoul d i ncl ude a bl ood f i l m and di fferenti al whi te cel l count. Duri ng the acute phases
of the i nfecti on, pati ents produce atypi cal mononucl ear cel l s (Dauncy cel l s) and there
wi l l be a l ymphocytosi s. In the past, the Paul Bunel l test was used to detect the
heterophi l e anti body i n pati ent' s serum, whi ch aggl uti nates sheep erythrocytes. In most
l aboratori es thi s test has been repl aced by a sl i de aggl uti nati on test cal l ed the
monospot.
The pati ent wi th gl andul ar fever feel s i l l and has a fever and enl argement of the l ymph nodes
that may extend over the whol e

of the body. The severi ty of these symptoms i s very wi del y vari abl e, rangi ng from a mi nor
attack that may pass al most unnoti ced to a condi ti on requi ri ng hospi tal i zati on.
In the earl y stages of thi s i nfecti on a sore throat and oral ul cerati on may be very
troubl esome. The ul cerati on i s qui te non-speci f i c, but may be wi despread. As i n si mi l ar
ci rcumstances symptomati c treatment of the oral ul cers wi th anti bi oti c mouthwashes i s the
onl y hel pful procedure. Petechi ae may be vi si bl e at the juncti on of the hard and soft pal ate.
Management of thi s condi ti on i s symptomati c but recovery can be qui te protracted i n severe
cases.
Coxsackievirus infections
Thi s group of vi ruses i s di vi ded i nto Coxsacki e group A and group B vi ruses. Group A vi ruses
are pri nci pal l y responsi bl e for two i nfecti ons that aff ect the oropharyngeal regi on: hand,
foot, and mouth di sease and herpangi na. These are rel ati vel y uncommon but tend to
occur as mi ni -epi demi cs because they are hi ghl y contagi ous. The putati ve vi ruses, cl i ni cal
presentati on, and treatment of these i nf ecti ons are summari zed i n Tabl e 4. 13. Treatment for
both these i nfecti ons i s symptomati c.
Hand, foot, and mouth disease
P. 44
Hand, foot, and-mouth di sease (not the same as foot and mouth di sease) i s caused
by a Coxsacki e A vi rus, usual l y type 16 but l ess commonl y types 5 and 10. A number of
mi nor epi demi cs of thi s condi ti on have occurred and been descri bed i n detai l , one i nvol vi ng
students and staff at a dental hospi tal . The oral lesi ons i n thi s outbreak consi sted of smal l
ul cers, resembl i ng mi nor aphthous ul cers, rel ati vely few i n number and di stri buted over the
oral mucosa. However, oral l esi ons, observed by one of the authors, al so i n dental hospi tal
personnel , and confi rmed by a f ul l range of i nvesti gati ons, have, i n fact, consi sted of bul l ae
that l ater ruptured to produce transi entl y pai nful erosi ons of the mucosa. The l esi ons of the
hands and feet consi st of a red macul ar rash, each macul e apparentl y surroundi ng a deep-
seated vesi cl e. The consti tuti onal symptoms experi enced are of a mi l d nature, a sl i ght
mal ai se wi th mi ni mal pai n and di scomfort. The symptoms resol ve i n about a week.
Herpangina
Herpangi na i s predomi nantl y caused by Coxsacki e A vi ruses. It i s a mi l d i nfecti on, seen
predomi nantl y (but not enti rel y) i n chi l dren, that tends to occur i n mi nor epi demi cs. The
pati ent compl ai ns of a moderate degree of mal ai se and of a sore throat, whi l e occasi onal l y
there i s al so a mi nor degree of muscl e weakness and pai n. Smal l vesi cul ar l esi ons appear i n
the posteri or part of the mouth, i n parti cul ar, on the soft pal ate. These l esi ons are not
parti cul arl y characteri sti c of the condi ti on, but resembl e other vi rus-i nduced l esi ons and are
recogni zabl e onl y by thei r typi cal di stri buti on. Thi s i s a sel f -l i mi ti ng condi ti on. The l esi ons
fade af ter some 35 days and compl i cati ons are extremel y rare.
Paramyxovirus infections
Paramyxovi ruses i ncl ude the mumps vi rus and the morbi l l i vi rus that causes measl es. These
i nfecti ons are i ncl uded i n thi s chapter because they both have orof aci al mani f estati ons.
Mumps
Mumps i s characteri zed by bi l ateral swel l i ng of the paroti d gl ands, al though uni l ateral
gl andul ar swel l i ng can occur. Invol vement of the submandi bul ar gl and i s l ess common. The
sal i vary gl and ducts usual l y appear red and i nfl amed and pati ents occasi onal l y report a dry
mouth. Tri smus may be present and the gl ands are extremel y pai nf ul and tender to touch.
Compl i cati ons of mumps i ncl ude pancreati ti s, encephal i ti s, orchi ti s, oophori ti s, and deafness.
The el derl y are at greater ri sk of devel opi ng compl i cati ons. Management i s symptomati c wi th
Table 4.13 Clinical features of Coxsackievirus infections
Hand, foot, and mouth di seaseCoxsacki e A16 (others: A5, A10)
Intraoral vesi cl es/ul cers
Macul ar rash wi th vesi cul ati on on
pal mar surf ace of hands
pl antar surace of feet
Mal ai se
Durati on: 7 days
Mi ni -epi demi cs
Herpangi naCoxsacki e A (most types)
Sore throat and mal ai se
Vesi cl es/ul cers on pal ate and pharynx
Durati on: 35 days
general supporti ve measures (see al so Chapter 8).
Mumps
Incubati on peri od: 1421 days
Transmi ssi on: respi ratory secreti ons and sal i va
Sal i vary gl and enl argement, usual l y bi l ateral paroti d i nvol vement
Tri smus
Di agnosi s: usual l y on cl i ni cal presentati on
Measles
Measl es usual l y presents as a systemi c, febri l e i l lness wi th an i ni ti al nasal di scharge,
(catarrhal stage). Kopl i k' s spots are bl ui sh-whi te, pi npoi nt spots, wi th dark-red aveol ae, that
appear on the buccal mucosae and di sappear af ter 34 days. A macul opapul ar ski n rash
then appears but resol ves af ter a few daysdi sappearance of the rash heral ds recovery. In
a f ew cases there are potenti al l y seri ous compl i cati ons resul ti ng from i nf ecti on wi th measl es,
i ncl udi ng encephal i ti s and pneumoni a. Management i s symptomati c wi th general supporti ve
measures. Anti bi oti cs may be requi red for compl i cati ons such as secondary oti ti s or
pneumoni a.

Human papillomavirus infections
There are over 100 di fferent human papi l l omavi ruses (HPV) and they can cause warty l esi ons
on the ski n and mucous membranes. The common wart (verruca vul gari s) i s commonl y seen
on the ski n and oral mucosa, where i t i s i ndi sti ngui shabl e from a squamous cel l papi l l oma
(Chapter 9). The cl i ni cal presentati on i s of a caul i fl ower-l i ke l esi on. Intraoral l esi ons
or those affecti ng the l i ps are often the resul t of autoi nocul ati on by chewi ng warts on the
hands. Heck' s di sease i s a rare oral mani festati on of HPV i nf ecti on i n the mouth and i s
di scussed i n Chapter 9. Venereal warts (condyl oma acumi natum) may occur i n the mouth, as
a resul t of orogeni tal sexual contact and appear as sof t, pi nk papi l l ary l esi ons, parti cul arl y
on the pal ate and tongue. Cancer of the cervi x has been associ ated wi th HPV i nfecti on.
Human immunodeficiency virus and AIDS
The human i mmunodefi ci ency vi rus (HIV) i s a retrovirus responsi bl e for the acqui red i mmune
def i ci ency syndrome (AIDS). The f i rst human retrovi rus, human T-cel l l ymphotrophi c vi rus
type I, was i sol ated i n1980 f rom a pati ent wi th T-cel l l eukaemi a. The cl i ni cal mani festati ons
of HIV i nfecti on were fi rst recogni zed i n 1981. The country of ori gi n of the vi rus i s unknown,
al though there i s f ai rl y strong presumpti ve evi dence that the i ni ti al centre of acti vi ty was i n
central Afri ca. HIV i nf ecti on i s, however, a rapi dly expandi ng worl dwi de probl em, al though,
qui te cl earl y, prognosti cati on i n these ci rcumstances i s a very di ffi cul t process and there can
be no degree of certai nty about the many projecti ons of future pati ent numbers that have
been made. Transmi ssi on of the vi rus i s currentl y thought to be predomi nantl y vi a bl ood and
bl ood products and semen. Peri natal and postnatal transmi ssi on occur i n about 20 per cent of
i nfants born to i nfected mothers.
The pathogenesi s of di sease caused by HIV i nfecti on i s compl ex and outsi de the scope of thi s
book. The most si gni fi cant ef fect of the vi rus i s i n the i nfecti on and consequent i nacti vati on
of CD4-expressi ng cel l s, that i s, the hel per and del ayed-type hypersensi ti vi ty T cel l s.
Inf ecti on wi th the vi rus i s a sl ow process, but eventual l y the host' s i mmune system i s
functi onal l y di sabl ed.
A cl assi fi cati on of the stages of HIV i nfecti on has been proposed and i s shown i n Tabl e 4. 14.
P. 45
The management of pati ents wi th HIV i nf ecti on i s compl ex but i nvol ves anti retrovi ral therapy
and the treatment of opportuni sti c i nfecti ons (f or exampl e, candi dosi s, herpes vi rus
i nfecti ons, and Pneumocysti s cari ni i ) and mal i gnanci es associ ated wi th AIDS. Azi dothymidi ne
(AZT) was the fi rst anti retrovi ral drug to be l i cenced for treati ng HIV and i s a protease
i nhi bi tor. The prognosi s for pati ents has i mproved wi th the i ntroducti on of mul ti pl e drug
therapi es. Tri pl e therapy consi sts of a combi nati on of drugs, i ncl udi ng a protease i nhi bi tor
(for exampl e, AZT), nucl eosi de reverse transcri ptase i nhi bi tors, and non-nucl eosi de reverse
transcri ptase i nhi bi tors. The cost of these drug regi mens i s hi gh and consi dered to be
unaff ordabl e by Thi rd Worl d countri es. Thei r l ong-term ef fecti veness has yet to be proven.
Oral manifestations of HIV infection
The oral mani f estati ons of HIV i nfecti on are consi dered to be of great si gni fi cance and have
been cl assi fi ed as two groups: group 1, l esi ons commonl y associ ated wi th HIV i nf ecti on; and
group 2, l esi ons l ess commonl y associ ated wi th HIV i nf ecti on (Tabl e 4. 15). The most
si gni f i cant consi derati on concerns the changes that may appear i n the oral cavi ty of a pati ent
wi th HIV i nf ecti on that has not, as yet, been di agnosed. The l i kel y oral changes are
dependent on the i ncreasi ng reducti on i n i mmune survei l l ance, presenti ng ei ther as i nfecti ons
or as neopl asms. The most l i kel y i nfecti on i s candi dosi s.
Table 4.14 Classification of the stages of HIV infection*
Group Description
I Seroconversi on i l l ness
II Asymptomati c
III Persi stent general i zed l ymphadenopathy
IVA Consti tuti onal di sease
IVB Neurol ogi cal di sease
IVC Secondary i nfecti ous di sease
IVD Secondary cancers
IVE Other condi ti ons
* As proposed by the Center for Di sease Control , USA.
Table 4.15 Oral manifestations of HIV infection

ORAL CANDIDOSIS IN HIV INFECTION
As has been poi nted out previ ousl y i n thi s chapter, oral candi dal i nfecti on shoul d al ways be
consi dered as an i ndi cator of general i zed i l l heal th and, i n the case of the earl y AIDS pati ent,
i t may be the earl i est presenti ng si gn. Other vi ral, f ungal , and bacteri al oral i nfecti ons are
much l ess l i kel y, but do occur. The otherwi se unexpl ai ned onset of an i nf ecti on, parti cul arl y
when associ ated wi th other persi stent si gns and symptoms, such as general i zed l ymph node
enl argement, mal ai se, i ntermi ttent fevers, and wei ght l oss, shoul d arouse suspi ci ons. Oral
candi dosi s has al ready been descri bed i n thi s chapter and angul ar chei l i ti s i s further
di scussed i n Chapter 6. Erythematous candi dosi s commonl y occurs i n HIV-i nfected i ndi vi dual s
and af fects the hard and soft pal ate, tongue, and buccal mucosae. Pseudomembranous
candi dosi s i s f requentl y chroni c and angul ar chei l iti s may al so be present.
HAIRY LEUKOPLAKIA AND HIV INFECTION
Hai ry l eukopl aki a has been descri bed i n a l arge number of pati ents who are HIV-
i nfected. It occurs on the l ateral margi ns of the tongue and has a fol ded, corrugated, or
hai ry appearance and general l y i s asymptomati c. Al though Candi da may be
associ ated wi th the l esi on, i t does not seem to be a candi dal l eukopl aki a or chroni c
hyperpl asti c candi dosi s, as descri bed earl i er i n thi s chapter. The l esi on appears to be
associ ated wi th the Epstei nBarr vi rus (herpes, type 4), as demonstrated both by
hi stochemi cal and i mmunol ogi cal studi es. It i s suggested that hai ry l eukopl aki a i s a powerful
cl i ni cal i ndi cator of i mmunosuppressi on i n HIV-i nfected pati ents and that i t i s a predi ctor of
the eventual onset of AIDS. Thi s does not seem to be a l esi on wi th si gni fi cant premal i gnant
potenti al . No cases of mal i gnant transf ormati on i n hai ry l eukopl aki a have as yet been
descri bed.
PERIODONTAL DISEASE IN HIV INFECTION
Three peri odontal mani f estati ons of HIV have been descri bed: a l i near gi ngi val erythema that
Di seases strongl y associ ated wi th HIV i nfecti on
Candi dosi s
Erythematous
Pseudomembranous
Hai ry l eukopl aki a
Peri odontal di sease
Li near gi ngi val erythema
Necroti zi ng ul cerati ve gi ngi vi ti s
Necroti zi ng ul cerati ve peri odonti ti s
Kaposi ' s sarcoma
Lymphoma
Lesi ons l ess commonl y associ ated wi th HIV i nfecti on
Mycobacteri al i nfecti ons
Mel anoti c pi gmentati on
Necroti zi ng (ul cerati ve) stomati ti s
Cysti c sal i vary gl and di sease
Thrombocytopeni c purpura
Non-speci f i c ul cerati on
Vi ral i nf ecti ons i ncl udi ng Herpes si mpl ex, Herpes zoster, and human papi l l oma vi rus
i nfecti on
P. 46
appears as a red band on the margi nal gi ngi va and is not parti cul arl y associ ated wi th poor
oral hygi ene; a necroti zi ng ul cerati ve gi ngi vi ti s, associ ated wi th pai n and spontaneous
bl eedi ng; and a necroti zi ng ul cerati ve peri odonti ti s that i s destructi ve and rapi dl y
progressi ve.
KAPOSI'S SARCOMA
The neopl asm that i s most l i kel y to occur i n the AIDS pati ent i s Kaposi ' s sarcoma (Fi g. 4. 8).
The mouth and, i n parti cul ar, the mucosa of the hard pal ate i s a common si te for thi s l esi on,
whi ch i s, i n f act, a form of di ffuse l ymphoma rather than a di screte neopl asm. The l esi on i s
descri bed as a pi gmented, nonpai nful , sl i ghtl y nodul ar l esi on of the mucosa wi th a
characteri sti c hi stol ogi cal appearance. Before the recogni ti on of AIDS thi s was consi dered to
be a rare l esi on, confi ned to el derl y pati ents of several restri cted raci al groups (Bantu,
Jewi sh, and Ital i an) or to pati ents on i mmunosuppressi ve therapy. Its appearance i n pati ents
of other ki nds i s now sai d to be vi rtual l y pathognomoni c of acti ve AIDS. Homosexual men are
consi dered to be more at ri sk from Kaposi ' s sarcoma than other groups wi th HIV i nfecti on.
The herpes vi rus 8 (HHV-8) i s now consi dered to have a rol e i n the aeti ol ogy of thi s sarcoma.
LYMPHOMA
Non-Hodgki n' s l ymphoma i s commonl y associ ated wi th HIV i nfecti on and may present as a
swel l i ng or ul cerati ve l esi on i n the mouth.
Group 2 l esi ons i ncl ude a wi de range of opportuni sti c i nfecti ons (Tabl e 4. 12) i ncl udi ng HPV-
associ ated l esi ons that may af fect the oral cavi ty. Swel l i ng of the sal i vary gl ands, xerostomi a
(Chapter 8), and mel anoti c pi gmentati on has al so been associ ated wi th HIV i nfecti on.
Discussion of problem cases
Case 4.1 Discussion
The pati ent can be advi sed to use a topi cal anti vi ral preparati on, such as aci cl ovi r or
Fig. 4.8 Kaposi ' sarcoma of the gi ngi vate
Q1 What advi ce woul d you gi ve thi s l ady?
penci cl ovi r cream. Thi s shoul d be appl i ed fi ve ti mes dai l y starti ng i n the prodromal stage of
the l esi on, that i s, when she f i rst feel s a ti ngl i ng or pri cki ng sensati on of the
l i ps. In resi stant cases, or when the l esi ons recur at regul ar i nterval s, then i t woul d be
justi f i ed to prescri be a prophyl acti c course of systemi c aci cl ovi r (for dose, see
manuf acturer' s data sheet). Exposure to sun appears to preci pi tate thi s l ady' s l esi ons and
she shoul d use a sunbl ock l i p preparati on wi th a hi gh sun protecti on factor (SPF). It i s
advi sabl e to remi nd the pati ent that col d sores are hi ghl y i nfecti ous and she shoul d be
caref ul not to share towel s, cups, or cutl ery wi th other f ami l y members. Orogeni tal
transmi ssi on i s al so a ri sk duri ng sexual acti vi ty.
Herpeti c whi tl ows were consi dered to be an occupati onal hazard for dental heal th-care
workers, pri or to the use of protecti ve gl oves, and resul ted f rom contact wi th a pati ent
presenti ng wi th a herpeti c l esi on on thei r l i ps. Pati ents who bi te thei r nai l s may
autoi nocul ate thei r HSV and devel op a herpeti c whi tl ow, whi ch i s extemel y pai nful and
di f fi cul t to treat.

Case 4.2 Discussion
You shoul d i nform the pati ent about hi s candi dal i nfecti on and expl ai n that i t i s i mportant to
check for an underl yi ng cause or preci pati ng f actor. Detai l ed questi oni ng about hi s medi cal
hi story and general heal th i s essenti al , wi th parti cul ar emphasi s on recent anti bi oti c or
steroi d therapy (i ncl udi ng steroi d i nhal ers for asthma). There may be a fami l y hi story of
di abetes and the pati ent shoul d be asked speci f i c questi ons, rel ati ng to symptoms of
di abetes. At thi s stage, the pati ent can be asked if he knows any reason why he mi ght be
suscepti bl e to i nfecti ons. He may then vol unteer that he i s i n a ri sk group for HIV i nfecti on.
It i s not al ways appropri ate i n the dental surgery to ask pati ents about thei r l i festyl e or
sexual i ty, but the pati ent may wi sh to di vul ge thi s i nformati on. Pati ents shoul d be abl e to
di scuss thei r medi cal hi story i n pri vacy and i t must al ways be consi dered conf i denti al , by al l
dental staff.
The pati ent shoul d be prescri bed topi cal or systemi c anti f ungal s (for exampl e, fl uconazol e),
whi l e awai ti ng the resul ts of mi crobi ol ogi cal sampl i ng, and a topi cal anal gesi c mouthwash to
reduce oral di scomfort. He shoul d be advi sed to maintai n hi s fl ui d i ntake and eat soft f oods.
Further i nvesti gati ons, i ncl udi ng a ful l range of bl ood tests, and fol l ow-up need to be carri ed
out at a speci al i st oral medi ci ne (or oral surgery) uni t. The pati ent may request HIV testi ng
but thi s shoul d onl y be done after prof essi onal counsel l i ngthi s i s usual l y avai l abl e i n a
department of geni touri nary medi ci ne.
Projects
1. Wi despread and often i nappropri ate use of anti bi oti cs has resul ted i n the emergence of
bacteri a that are resi stant to therapy. It i s wel l recogni zed that resi stance to anti bi oti cs
i s transferred f rom one bacteri um to another. Fi nd out about the di fferent methods of
geneti c exchange i n bacteri a.
2. What l aboratory tests are currentl y used for the di agnosi s of HIV i nfecti on? Why i s
professi onal counsel l i ng recommended for pati ents pri or to HIV testi ng?
Q2 Does the l esi on on her l i p pose a hazard to members of the dental team?
P. 47
> Tabl e of Cont ent s > 5 - Oral ul cer at i on
5
Oral ulceration
Problem cases
Case 5.1
A 45-year-ol d ex-smoker i s referred to you i n the oral medi ci ne cl i ni c by a cardi ol ogi st. The
gentl eman has a hi story of severe, recurrent oral ul cerati on aff ecti ng the l ateral borders of
tongue, l abi al mucosae, and soft pal ate. One or two ul cers occur at a ti me, they usual l y l ast
for about 8 weeks and he i s rarel y ul cer-free. Thi s pati ent fi rst devel oped ul cers 9 months
pri or to the consul tati on, af ter bei ng di scharged from hospi tal fol l owi ng a severe myocardi al
i nfarcti on. He i s currentl y on medi cati on, i ncl udi ng a potassi um-channel acti vator (ni corandi l )
for unstabl e angi na and, si nce the myocardi al i nfarcti on, has been on aspi ri n 75 mg/day. The
pati ent reports di ffi cul ty wi th eati ng and swal l owing. He has no eye, ski n, or geni tal l esi ons.
Al l previ ous attempts to control hi s oral ul cerati on wi th topi cal therapy have fai l ed. On
exami nati on he has l arge aphthousl i ke ul cers wi th evi dence of mucosal scarri ng.
Two days after your consul tati on thi s pati ent presents wi th severe toothache and requi res
the extracti on of a mandi bul ar fi rst mol ar. He i s extremel y anxi ous about dental treatment.
Case 5.2
A 20-year-ol d teacher attends your dental practi ce, wi th a 5-year hi story of recurrent
aphthous stomati ti s that i s becomi ng a nui sance. The ul cers occur mai nl y i nsi de her cheeks
and l i ps. They usual l y l ast 10 days and two or three occur at a ti me. Over-the-counter
remedi es have been tri ed but are not very hel pful . She does not want to be ref erred to the
nearest speci al i st uni t, whi ch i s 50 mi l es away.
Introduction
Ul cerati on of the oral mucosa i s a common reason for pati ents to seek medi cal or dental
advi ce. Pati ents may report recurrent epi sodes of oral ul cerati on, si ngl e epi sodes of
ul cerati on, or persi stent oral ul cerati on. Causes of oral ul cerati on (Tabl e 5. 1) i ncl ude trauma,
recurrent aphthous stomati ti s (RAS), mi crobi al i nfecti ons (Chapter 4), mucocutaneous
di seases (Chapter 11), drug therapy (Chapter 14), and squamous cel l carci noma (Chapter
10). Ul cerati on due to trauma, RAS, and Behet' s di sease wi l l be di scussed i n thi s chapter.
Q1 How woul d you manage thi s gentl eman' s severe RAS and what therapeuti c opti ons
are avai l abl e?
Q2 How woul d you manage thi s dental emergency?
Q1 How woul d you manage thi s pati ent i n your practi ce?
Table 5.1 Principal causes of oral ulceration
Traumatic ulceration
Aetiology
Traumati c ul cers can be due to physi cal (mechani cal , thermal , el ectri cal ) or chemi cal trauma.
Common causes of mechani cal trauma are sharp, broken down teeth, orthodonti c and
prostheti c appl i ances, and numb l i ps or tongue bei ng bi tten after a l ocal anaestheti c
i njecti on.
Chemi cal trauma can be caused by aspi ri n or toothache remedi es bei ng pl aced
di rectl y on the oral mucosa.
Aspi ri n pl aced di rectl y on the oral mucosa, as a remedy for toothache, can cause a chemi cal
burn.
Clinical features
Traumati c ul cers are sore, pai nful to touch, and tend to have an i rregul ar border wi th
erythematous margi ns and a yel l ow base. Duri ng the heal i ng phase they f requentl y devel op a
keratoti c hal o.
Management
Management i nvol ves the el i mi nati on of the suspected cause and use of an anti septi c
mouthwash (for exampl e, 0. 2 per cent chl orhexi di ne) or a si mpl e coveri ng agent such as
Orabase

duri ng the heal i ng phase of ul cerati on. Al l traumati c ul cers shoul d be revi ewed. If they
persi st then a bi opsy shoul d be carri ed out to exclude squamous cel l carci noma.
If a putati ve traumati c ul cer persi sts for more than 1014 days after el i mi nati on of the
aeti ol ogi cal factor, then the pati ent shoul d be referred for a speci al i st opi ni on and possi bl e
bi opsy to excl ude the possi bi l i ty of an oral carci noma.
Facti ti ous (sel f-i nfl i cted) oral ul cerati on can al so occur (see Chapter 17) and may be di ffi cul t
to di agnose and manage. Psychi atri c advi ce i s required i n some cases.
Recurrent aphthous stomatitis
Recurrent aphthous stomati ti s (RAS) i s the most common oral mucosal di sease aff ecti ng
Trauma
Recurrent aphthous stomati ti s (RAS)
Mi crobi al i nfecti ons
Mucocutaneous di seases
Systemi c di sorders
Drug therapy
Squamous cel l carci noma
P. 52
humans and has been reported as af fecti ng 2025 per cent of the general popul ati on at
any ti me. A hi gher preval ence has been reported i n North Ameri can students, parti cul arl y at
exami nati on ti mes, and i n upper soci o-economi c groups. Interesti ngl y, RAS i s i nfrequentl y
found i n Bedoui n Arabs.
Clinical features
The cl i ni cal f eatures of RAS consi st of recurrent bouts of one or several , shal l ow, ovoi d,
pai nful ul cers, occurri ng at i nterval s of a f ew days or up to 23 months. Three cl i ni cal
presentati ons of RAS are recogni zed: mi nor recurrent aphthous stomati ti s (Mi RAS), major
recurrent aphthous stomati ti s (MjRAS); and herpeti form ul cerati on (HU). The cl i ni cal
presentati on of these types of RAS are shown i n Tabl e 5. 2. Pati ents may someti mes present
wi th a mi xed pattern of RAS but thi s i s rel ati vel y uncommon.
Minor recurrent aphthous stomatitis (MiRAS)
Thi s i s the most common form of RAS and approxi matel y 80 per cent of pati ents have l esi ons
of thi s type. It i s reported that 56 per cent of the pati ents are femal es and that the peak age
of onset of the ul cers i s i n the second decade (1019 years). However, many pati ents
experi ence thei r fi rst ul cers at an age wel l outsi de these l i mi ts. Indeed, i t i s by no means
uncommon to fi nd thi s form of ul cerati on i n much younger chi l dren.
In i ts most characteri sti c form Mi RAS presents the pi cture of a number of smal l ul cers (one
to fi ve) appeari ng on the buccal mucosa, the l abi al mucosa, the f l oor of the mouth, or the
tongue. Moreover, the ul cers are usual l y concentrated i n the anteri or part of the mouth. The
pharynx and tonsi l l ar f auces are rarel y i mpl i cated i n thi s form of ul cerati on. The prodromal
stage of ul cerati on i s vari abl e, but there i s usual l y a sensati on descri bed as burni ng
or pri cki ng for a short peri od before the ul cers appear. Fol l owi ng thi s phase,
ul cerati on occurs di rectl y by l oss of the epi thel i um. The ul cers are usual l y l ess than 1 cm i n
di ameter and, i n most i nstances, thei r si ze i s approxi matel y 4 or 5 mm i n di ameter. However,
the cl assi fi cati on of mi nor RAS does not depend on the di mensi ons of the l esi ons
al one, but on a number of cl i ni cal features. It i s qui te possi bl e to have l arge mi nor ul cers
and smal l major ones. The appearance of the ul cer base i s grey-yel l ow, often wi th a red and
sl i ghtl y rai sed margi n, and, unl ess i nfl uenced by the si te (as i n the depth of the buccal
sul cus where they appear el ongated), they are usual l y oval i n shape (Fi g. 5. 1). The ul cers
are pai nful , parti cul arl y i f the tongue i s i nvol ved, and may make eati ng or speaki ng di ffi cul t.
If the l i ps are i mpl i cated, there may be a mi nor degree of oedema i n the surroundi ng area
but thi s i s not common. Lymph node enl argement i s seen onl y as a response to secondary
i nfecti on i n severel y af fected pati ents. The course of these ul cers vari es f rom a few days to a
l i ttl e over 2 weeks, but usual l y thei r durati on i s of the order of 10 days. After thi s peri od the
ul cerated areas re-epi thel i al i ze and heal over i n an i nterval of some days, but resol uti on may
or may not be si mul taneous i n al l the ul cers of a group. Mi nor aphthae heal wi thout scar
formati on. Thus, i f scars shoul d form, i t i s probabl e that the condi ti on i s not Mi RAS, but the
major type. Fol l owi ng heal i ng of the ul cers, there i s a vari abl e ul cer-free i nterval 34
weeks i s most commonbut many pati ents are abl e to predi ct wi th some degree of
accuracy the peri odi ci ty of the condi ti on. In a f ew pati ents, however, the recurrence of the
ul cers appears to be enti rel y random and, i n some cases, there may not be an ul cer-free
peri od between attacks, wi th new aphthae devel opi ng before exi sti ng ones have heal ed.
Table 5.2 Clinical features of RAS
Feature Type of RAS

Major recurrent aphthous stomatitis (MjRAS)
Major RAS vari es f rom the mi nor form i n a number of i mportant cl i ni cal features. The ul cers
are general l y l arger than those of Mi RAS (Fi g. 5. 2) and they are of greater durati on, up to a
peri od of months i n some cases. As a resul t of the l ong peri ods of ti me i nvol ved, there i s
probabl y a marked tendency to the producti on of a heaped-up margi n whi ch, when a si ngl e
ul cer i s seen i n i sol ati on, may l ead to the suspi cion that the l esi on i s mal i gnant. On eventual
heal i ng, the ul cers may l eave a substanti al scar and thi s, together wi th the ti ssue destructi on
that may occur duri ng the acti ve phase of ul cerati on, may l ead to gross di storti on of the
i nvol ved ti ssues (Fi g. 5. 3). Mj RAS may produce l esi ons throughout the enti re oral cavi ty,
i ncl udi ng the sof t pal ate and tonsi l l ar areas, and ul cerati on of ten extends to the oropharynx
(Fi g. 5. 4). The i nvol vement of the posteri or oral ti ssues i s so characteri sti c of MjRAS as to be
di agnosti c, even though the ul cers may i ni ti al l y be smal l .
Minor (MiRAS) Major (MjRAS) Herpetiform (HU)
Peak age of
onset
(decade)
Second Fi rst and second Thi rd
Number of
ul cers/bout
15 13 520 (up to 100)
Si ze of
ul cers (mm)
< 10 >10 12
Durati on 714 days 2 weeks3
months
714 days
Heal wi th
scarri ng
No Yes No*
Si te Non-kerati ni zed
mucosa especi al l y
l abi al /buccal
mucosa.
Dorsum and
l ateral borders of
tongue
Kerati ni zed pl us
non-kerati ni zed
mucosa
parti cul arl y sof t
pal ate
Non-kerati ni zed
mucosa, but
parti cul arl y fl oor of
mouth and ventral
surface of tongue
* Unl ess a number of ul cers coal esce.
P. 53
Fig. 5.1 Aphthous stomati ti s (mi nor type) on tongue.
Fig. 5.2 Aphthous stomati ti s (major type), on l abi al mucosa, of 6 weeks durati on.
Fig. 5.3 Scarri ng of the l ateral margi n of the tongue fol l owi ng recurrent major aphthous
stomati ti s.

The number of ul cers present at one ti me vari es from one to 10 i n MjRAS. Frequentl y, a
si ngl e ul cer wi l l persi st for a l ong peri od, whi l e other (usual l y smal l er) ul cers fade. Affected
pati ents show a sl i ght mal e preponderance and there i s a wi de vari ati on i n the age of
i nci dence, al though most pati ents experi ence thei r f i rst ul cers duri ng the fi rst two decades of
l i f e. Unl i ke Mi RAS, there does not appear to be a cycl i cal pattern i n MjRAS and the ul cers are
usual l y unpredi ctabl e i n thei r onset. Long peri ods of remi ssi on may be f ol l owed by i nterval s
of i ntense ul cer acti vi ty, wi thout any obvi ous preci pi tati ng f actor. The prol onged and pai nful
ul cerati on may present si gni f i cant probl ems to the pati ent. In parti cul ar, eati ng may become
extremel y di ffi cul t and the general heal th of the pati ent may suf fer as a consequence.
Herpetiform ulceration (HU)
Thi s di sti ncti ve form of RAS di ffers i n many ways from both Mi RAS and MjRAS and i t i s l ess
common. The term herpeti f orm i s not unl i ke that of dermati ti s herpeti formi s i n
dermatol ogi cal practi ce, i n that i t refers to a morphol ogi cal resembl ance to l esi ons caused by
herpes vi rus i nfecti ons. However, i t i s an unf ortunate one, causi ng conf usi on wi th vi ral
i nfecti ons. In HU the ul cers are smal l (12 mm) and mul ti pl e (as many as 100 ul cers may
be present at the same ti me). Al though any non-kerati ni sed oral mucosa may be i nvol ved,
characteri sti cal l y the aff ected si tes are the l ateral margi ns and ventral surface of the tongue
and the f l oor of the mouth (Fi g. 5. 5). Indi vi dual ul cers are grey and wi thout a del i neati ng
erythematous border, maki ng them qui te di ffi cul t to vi sual i ze. In spi te of thei r smal l si ze,
these ul cers are very pai nf ul and may make eati ng and speaki ng di ffi cul t. A si ngl e crop of
ul cers may l ast for approxi matel y 714 days, and the peri od of remi ssi on between attacks
i s vari abl e. In some i nstances there may be repeated epi sodes of ul cerati on wi thout any
i nterval of remi ssi on. Where many ul cers are present they may coal esce to f orm l arger
confl uent areas of ul cer, usual l y wi th marked erythema. The pati ents af fected are
predomi nantl y f emal e (2. 6:1), the most common age of onset bei ng i n the 2029-year-ol d
group. Fortunatel y, thi s f orm of recurrent ul ceration i s rel ati vel y short-l i ved, most pati ents
experi enci ng spontaneous remi ssi on wi thi n 5 years of onset. Heal i ng wi th scar formati on has
been descri bed i n HU but thi s i s probabl y a resul t of coal escence. Nothi ng resembl i ng the
gross ti ssue di storti on caused by the scars of MjRAS i s seen as an af termath of HU.
Fig. 5.4 Aphthous stomati ti s (major) on the sof t pal ate.
P. 54
Recurrent aphthous stomatitis (RAS) in children
RAS i s not, by any means, a condi ti on restri cted to adul ts. The authors revi ewed over 100
pati ents wi th wel l -establ i shed patterns of RAS f rom the age of 7 years onwards. The i ni ti al
appearance of the ul cerati on was, i n the case of a number of pati ents, bel ow the age of 3
years, al though these di d not i ncl ude cases of major aphthous ul cerati on. The fi rst
appearance of major ul cerati on i n thi s group was at the age of 7 years. The di stri buti on of
types of ul cerati on and the preval ence of haematol ogi cal abnormal i ti es was enti rel y
equi val ent to that descri bed i n mi xed age and adul t groups. The onset of ul cerati on, contrary
to frequentl y expressed i deas, di d not seem to be, i n any way, associ ated wi th puberty.
Wi thi n thi s group there was onl y one pati ent wi th an abnormal jejeunal mucosa typi cal of
coel i ac di sease.
Aetiology of RAS
The causati ve f actors of RAS are unknown but are probabl y mul ti factori al , wi th preci pi tati ng
systemi c, l ocal , mi crobi al , and geneti c factors al l bei ng i mpl i cated (Tabl e 5. 3). Aphthous-
l i ke ul cers can be associ ated wi th a number of systemi c condi ti ons, but these may not
exhi bi t the cl assi cal behavi our or pattern of RAS.
Genetic factors
It i s hi ghl y l i kel y that geneti c factors predi spose to RAS, wi th the preval ence of the condi ti on
bei ng modi fi ed by envi ronmental factors. Approxi matel y 4050 per cent of RAS pati ents
gi ve a f ami l i al hi story of RAS. The probabi l i ty of a si bl i ng devel opi ng RAS i s i nfl uenced by
thei r parents RAS status and there i s a hi gh correl ati on of RAS i n i denti cal twi ns. A
number of studi es have attempted to correl ate associ ati ons between parti cul ar human
l eukocyte anti gen (HLA) hapl otypes and RAS but the evi dence i s equi vocal .
Fig. 5.5 Aphthous stomati ti s (herpeti form type) under the ti p of the tonguemul ti pl e,
very smal l , and, as i s often the case, di ffi cul t to see. The pati ent was eventual l y found
to have coel i ac di sease and the ul cerati on was el i mi nated by a gl uten-free di et.
Table 5.3 Possible aetiological associations with RAS

Trauma
RAS pati ents often report aphthous ul cers at si tes of trauma, parti cul arl y due to
toothbrushi ng, or at the si te of a l ocal anaestheti c i njecti on.
Smoking cessation
There have been several reports of a negati ve associ ati on between smoki ng and RAS. A
si gni f i cant number of pati ents report that ul ceration appeared at the ti me they stopped
smoki ng. Tobacco may i ncrease mucosal kerati ni zati on, whi ch makes the mucosa l ess
suscepti bl e to ul cerati on. In suscepti bl e pati ents, ni coti ne repl acement therapy (for exampl e,
wi th transdermal patches) has been tri ed wi th vari abl e outcomes. Al ternati vel y, stress, after
stoppi ng smoki ng, may pl ay a rol e i n the i ncreased suscepti bi l i ty to RAS.
Stress and menstruation
These factors have both been i mpl i cated i n pl ayi ng a si gni fi cant rol e i n the aeti ol ogy of RAS.
A recent detai l ed revi ew of the l i terature was unabl e to establ i sh a defi ni te associ ati on
between RAS and the menstrual cycl e, al though there are many anecdotal reports of women
who rel ate thei r RAS to the l uteal phase of thei r cycl e. There i s al so no convi nci ng evi dence
that psychol ogi cal i l l ness or stress i s rel ated to RAS.
Microbial factors
There have been numerous studi es to i nvesti gate the rol e of mi cro-organi sms i n RAS. Li nks
have been proposed wi th oral streptococci , herpes si mpl ex vi rus, vari cel l a zoster vi rus, and
cytomegal ovi ruses but f urther studi es are requi red to prove or refute such associ ati ons.
Food hypersensitivity
There i s no proven associ ati on between hypersensi ti vi ty to certai n f oods and RAS, other than
i n gl uten-sensi ti ve enteropathy (coel i ac di sease).
Histopathology and immunopathogenesis of RAS
There are three phases i n the devel opment of an aphthous ul cer, the preul cerati ve,
ul cerati ve, and heal i ng phases. Hi stol ogi cal l y, aphthae are mucosal ul cerati ons wi th a mi xed
i nfl ammatory i nfi l trate. CD4
+
T l ymphocytes and l arge granul ar l ymphocytes predomi nate i n
the preul cerati ve and heal i ng phases and CD8
+
cel l s predomi nate i n the ul cerati ve phase.
Numerous studi es have been conducted on the i mmunopathogenesi s of RAS and there i s good
Trauma
Smoki ng cessati on
Mi crobi al agents
Geneti c factors
Stress
Menstruati on
Food hypersensi ti vi ty
P. 55
evi dence that pati ents wi th RAS show evi dence of i mmune dysregul ati on. It has been
postul ated that, i n the presence of a l ocal or systemi c sti mul us, the epi thel i al cel l s become
targets for the cytotoxi c acti on of l ymphocytes and monocytes and are subsequentl y
destroyed. Current evi dence woul d suggest that RAS i s not an autoi mmune di sease. The
i mmunopathogenesi s of thi s common di sorder, however, i s yet to be el uci dated.
Systemic conditions and RAS-like lesions
Tabl e 5. 4 gi ves a l i st of systemi c condi ti ons associ ated wi th RAS. Behet' s di sease wi l l be
di scussed i n the next secti on of thi s chapter.
Nutritional deficiencies
Haemati ni c (i ron, f ol i c aci d, or vi tami n B
12
) defi ci enci es have been reported to be twi ce as
common i n RAS pati ents as i n control s and up to 20 per cent of RAS pati ents may have a
haemati ni c def i ci ency. B-compl ex defi ci ency (B
1
, B
2
, and B
6
) has been reported i n a Scotti sh
cohort of RAS pati ents and zi nc defi ci ency has been i mpl i cated i n a few pati ents.
Repl acement therapy i n RAS pati ents wi th nutri ti onal def i ci enci es has not, however, met wi th
uni form success.
Up to 20 per cent of RAS pati ents may have a haemati ni c defi ci ency.
Gastrointestinal disorders
The associ ati on of RAS wi th coel i ac di sease and Crohn' s di sease i s ful l y di scussed i n Chapter
12. The preval ence of coel i ac di sease i n pati ents who present wi th RAS i s now thought to be
l ess than 5 per cent. Pati ents wi th establ i shed Crohn' s di sease frequentl y report oral
ul cerati on that i s aphthous-l i ke.
Neutropenia
A l arge percentage of pati ents wi th cycl i cal neutropeni a present wi th aphthous-l i ke
ul cerati on that occurs at i nterval s (often monthl y), refl ecti ng thei r neutropeni c status. Other
mani festati ons of neutropeni a i ncl ude f ever, mal ai se, and suscepti bi l i ty to bacteri al and
fungal i nfecti ons. Pati ents who are f uncti onal l y neutropeni c (for exampl e, those wi th chroni c
granul omatous di sease or beni gn fami l i al neutropenia) are al so suscepti bl e to aphthous-
Table 5.4 Systemic conditions and aphthous-like
lesions
Behet' s di sease
Nutri ti onal defi ci enci es
Gastroi ntesti nal di sorders
Cycl i cal neutropeni a
HIV i nfecti on
MAGIC syndrome
FAPA syndrome
Drug reacti ons
type ul cerati on.
HIV-associated aphthous stomatitis
These ul cers tend to occur i n crops of fi ve or fewer ul cers, on non-kerati ni zed mucosa and
may resembl e mi nor or major aphthae. They are frequentl y very pai nf ul and l ast for several
months. Thi s type of ul cerati on can be extremel y debi l i tati ng i n these pati ents and can cause
probl ems wi th eati ng. Care must be taken to excl ude HSV or cytomegal ovi rus i nf ecti on i n
HIV-i nfected pati ents. In addi ti on, some of the drugs used to

treat HIV i nf ecti on may predi spose pati ents to oral ul cerati on ei ther di rectl y or i ndi rectl y, by
causi ng myel osuppressi on. Thal i domi de has successf ul l y been used to treat HIV-associ ated
RAS.
Other systemic conditions and factors
Other systemi c condi ti ons associ ated wi th RAS-l i ke ul cerati on i ncl ude MAGIC (mouth
and geni tal ul cers wi th i nfl amed carti l age) syndrome, FAPA (peri odi c f ever, aphthous ul cers,
pharyngi ti s, and cervi cal adeni ti s) syndrome, and rel ati ve IgA defi ci ency. Drugs such as
nonsteroi dal anti -i nfl ammatory drugs (NSAIDs), ni corandi l , a potassi um-channel acti vator,
and methotrexate have been i mpl i cated i n RAS-l i ke ul cerati on.
Management of RAS
History and examination
The correct di agnosi s of a pati ent wi th RAS i s dependent on a detai l ed and accurate cl i ni cal
hi story and exami nati on of the ul cer(s). Important factors to be el i ci ted i n the hi story are
shown i n Tabl e 5. 5.
When exami ni ng pati ents wi th oral ul cerati on i t i s essenti al to carry out an extraoral
exami nati on i ncl udi ng pal pati on of the cervi cal l ymph nodes (see Chapter 7). The oral
mucosa, i ncl udi ng the area of ul cerati on, shoul d then be systemati cal l y exami ned. Tabl e 5. 6
l i sts the i mportant features to be noted when exami ni ng a pati ent wi th oral ul cerati on.
Cl i ni cal eval uati on of an ul cer i ncl udes i nspecti on and pal pati on, whi ch compl ement each
other. The base of the ul cer can be necroti c, granul ar purul ent, or covered wi th mucus. The
consi stency of the base (soft, fi rm, or hard) and fi xati on to underl yi ng structures can be
ascertai ned by pal pati on. The edges of the ul cer can be i rregul ar, strai ght, or undermi ned
and may feel hard i n contrast to the surroundi ng tissue. Thi s i s the characteri sti c i ndurati on,
associ ated wi th neopl asti c i nfi l trati on. Another feature of a carci noma i s i ts rol l ed border.
The ti ssue surroundi ng the ul cer may be whi te, speckl ed, erythematous, or normal i n
appearance.
The di agnosi s of RAS i s not usual l y di ffi cul t and may be deduced, i n most cases, from the
hi story and characteri sti c cl i ni cal appearance. If there i s any doubt about the di agnosi s,
appropri ate di agnosti c tests shoul d be arranged to excl ude other causes or oral ul cerati on.
Further investigations of RAS
Pati ents wi th persi stent and troubl esome RAS shoul d undergo screeni ng to check for an
underl yi ng haemati ni c defi ci ency (Tabl e 5. 7). Thi s i ncl udes a f ul l bl ood count and fi l m and
measurement of i nf l ammatory markers and haemati ni cs (serum ferri ti n, serum B
12
, serum
and red cel l fol ate) (Chapters 2 and 13). Screeni ng f or defi ci enci es of vi tami n B compl exes or
zi nc defi ci enci es i s not routi nel y carri ed out, but may be i ndi cated i n certai n groups of
pati ents. Pati ents wi th RAS associ ated wi th a systemi c condi ti on shoul d be ref erred to the
appropri ate speci al i st for f urther i nvesti gati ons and management. If there i s any suspi ci on of
coel i ac di sease, ei ther due to the pati ent' s hi story or evi dence of mal absorpti on on routi ne
testi ng, then serol ogi cal testi ng f or appropri ate IgA autoanti bodi es shoul d be carri ed out (see
P. 56
Chapter 12) and the pati ent ref erred to a gastroenterol ogi st f or endoscopy and bi opsy of the
smal l i ntesti ne.
Table 5.5 RASimportant points in the history
Age of onset
Fami l y hi story
Frequency of ul cerati on
Durati on of ul cerati on
Number of ul cers
Si te of ul cers (non-kerati ni zed or kerati ni zed)
Si ze and shape of ul cers
Associ ated medi cal condi ti on(s)
Geni tal ul cerati on
Ski n probl ems
Gastroi ntesti nal di sturbances
Drug hi story
Table 5.6 Oral ulcerationimportant features to be
noted
Number of ul cers
Si ze and shape of ul cer(s)
Si te of ul cerati on
Edge of ul cer
Base of ul cer
Surroundi ng ti ssue
Scarri ng
Consi stency
Table 5.7 Investigations for patients with persistent RAS
Haemogl obi n and ful l bl ood count
ESR/CRP
Serum B
12

Serum/red cel l f ol ate
Anti -gl i adi n and anti -endomysi al autoanti bodi es*
Therapy for RAS
Tabl e 5. 8 shows some of the therapeuti c opti ons avai l abl e f or the management of pati ents
wi th RAS. Some of these medi cati ons are avai l abl e over the counter and others requi re a
prescri pti on. Pati ents requi ri ng systemi c i mmunomodul ators are best managed i n a hospi tal
setti ng. The choi ce of therapy for RAS depends on the severi ty and frequency of ul cerati on
but the objecti ves of treatment are to rel i eve di scomfort, reduce secondary

i nfecti on, promote heal i ng of exi sti ng ul cerati on, and prevent new ul cers occurri ng.
* If gl uten-sensi ti ve enteropathy (coel i ac di sease) i s suspected (see Chapter 12).
P. 57
Table 5.8 Therapeutic options for RAS*
Type Therapy
Topi cal anti septi c Chl orhexi di ne gl uconate (mouthwash)
Topi cal anal gesi cs Benzydami ne hydrochl ori de (mouthwash)
Li gnocai ne ri nse
Topi cal corti costeroi ds Hydrocorti sone hemi succi nate (pel l ets)
Tri amci nol one acetoni de (i n adhesi ve paste)
Betamethasone val erate (mouthwash)
Becl omethasone di propi onate (spray)
Budesoni de (spray)
Tri amci nol one (wi th or wi thout chl ortetracycl i ne)
mouthwash
Topi cal anti bi oti c Chl ortetracycl i ne mouthwash
Systemi c
i mmunomodul ators
Predni sol one
Azathi opri ne
Col chi ci ne
ANALGESIC AND ANAESTHETIC PREPARATIONS
Topi cal anal gesi c sprays or ri nses such as benzydami ne hydrochl ori de (Di f fl am) can be
used to reduce di scomfort. However, 2 per cent l i gnocai ne gel , used di rectl y or di l uted as a
ri nse, i s more ef fecti ve for severe cases of RAS. Care must be taken i f used i n the posteri or
part of the mouth as stronger anal gesi c preparati ons can aff ect the l aryngeal refl exes. Long-
term use of topi cal l i gnocai ne i s not advi sabl e, as i t may be absorbed and cause systemi c
eff ects. Over-the-counter throat l ozenges contai ni ng a l ocal anaestheti c, often
combi ned wi th an anti septi c, can be used to rel i eve di scomfort f rom RAS but most
preparati ons contai n sugar and shoul d not be used conti nuousl y i n dentate pati ents.
Some pati ents may al so requi re systemi c anal gesi cs such as i buprofen or paracetamol ,
al though i t i s i mportant to remember that there i s a possi bi l i ty, al bei t rare, that NSAIDs can
preci pate RAS.
COVERING AGENTS
Several pastes and gel s can be used to coat the surface of the ul cers and to form a
protecti ve barri er agai nst secondary i nfecti on and further mechani cal i rri tati on (Chapter 3).
Some di f fi cul ty may be experi enced i n appl yi ng certai n of these preparati ons, parti cul arl y to
l arge ul cers and to those at the back of the mouth. It i s al so awkward to keep these
coveri ngs i n pl ace on the tongue and l i ps where constant movement tends to wi pe
them off. In spi te of these di sadvantages, however, such si mpl e remedi es are often hel pful i n
control l i ng the symptoms of mi nor aphthous ul cerati on.
ANTISEPTIC AGENTS
Anti septi c substances that may be hel pful i n temporari l y reduci ng secondary i nfecti on are
avai l abl e i n wi de vari ety and sui tabl y formul ated as mouthwashes, gel s, and pasti l l es
(Chapter 3). The response i s vari abl e, as mi ght be expected i n vi ew of the wi de range of
organi sms i nvol ved, but i n many cases some degree of rel i ef i s achi eved. Chl orhexi di ne
mouthwashes are wi del y used for the symptomati c treatment of RAS and are consi dered
hel pful by many pati ents, parti cul arl y i f oral hygi ene i s di f fi cul t to mai ntai n because of oral
ul cerati on. Extri nsi c stai ni ng of teeth associ ated wi th l ong-term use of chl orhexi di ne may be
a probl em.
TOPICAL ANTIBIOTICS
A more ef fecti ve measure i n the rel i ef of symptoms caused by secondary i nf ecti on i s the
Ci cl ospori n
Thal i domi de
Mi scel l aneous Ci metedi ne
Carbenoxol one (mouthwash and systemi c)
5 ami no-sal i cyl i c aci d
Dapsone
Pentoxphyl l i ne
Low-energy l aser
Levami sol e
*See Chapter 3 for f urther detai l s of topi cal therapy.
appl i cati on of topi cal anti bi oti cs. A mouthwash contai ni ng 2 per cent tetracycl i ne or
chl ortetracycl i ne i s often hi ghl y eff ecti ve i n reduci ng the pai n caused by severe ul cerati on
and, as a resul t of the much l ess heavi l y col oni zed envi ronment, the ul cers often heal more
rapi dl y than otherwi se (Chapter 3). The treatment of HU depends l argel y on thi s therapy, and
response to the anti bi oti c mouthwash i s often rapi d and compl ete. There are obvi ous
di sadvantages, however, i n the use of broad-spectrum anti bi oti cs f or thi s purpose, the ri sk of
hypersensi ti vi ty reacti ons and the encouragement of growth of resi stant organi sms bei ng the
most i mportant. Local secondary i nfecti on by opportuni sts such as Candi da seems to be much
l ess of a probl em, but, as been poi nted out i n Chapter 4, an anti bi oti c sore tongue
(acute erythematous candi dosi s) may very occasi onal l y devel op. In vi ew of the desi rabi l i ty of
l i mi ti ng the l ocal use of anti bi oti cs, thi s i s a form of treatment that shoul d be used spari ngl y
and, i n the majori ty of cases, as a si ngl e course of treatment for an i sol ated attack of
ul cerati on.
The response of HU to a 2 per cent chl ortetracycl i ne mouthwash i s often rapi d.
TOPICAL STEROIDS
Topi cal steroi ds can be effecti ve drugs i n the treatment of RAS. Pati ent response i s vari abl e
and there are some i ndi vi dual s who gai n l i ttl e or no rel i ef from thei r use. Steroi ds used i n
thi s manner have two modes of acti on. Thei r anti -i nfl ammatory acti on modi fi es, i n a mi nor
way, the progress of the ul cerati on at al l stages and, to some extent, reduces the di scomf ort
experi enced. The second effect of steroi ds, namel y, the speci fi c bl ocki ng effect of the T
l ymphocyteepi thel i al cel l i nteracti on, i s much more i mportant i n the present context.
Si nce the concentrati on of sensi ti zed l ymphocytes occurs bef ore and duri ng the earl y stages
of

ul cerati on, i t f ol l ows that the drugs exert thei r maxi mum eff ect at thi s ti me. Wi th the
establ i shment of the ul cerati on and the fal l i n sensi ti zed l ymphocyte concentrati on, the
speci f i c bl ocki ng ef fect of the steroi d becomes l ess i mportant, and onl y the anti -i nfl ammatory
acti on of the drug operates. For thi s reason i t i s i mportant that the pati ent shoul d
understand that, whatever preparati on i s used, i t is of maxi mum val ue at the ti me that the
earl i est prodromal si gns of ul cerati on are noti ced.
If usi ng a topi cal steroi d for RAS, ai m to use a preparati on that i s l ess l i kel y to gi ve si de-
eff ects because of systemi c absorpti on.
The drugs most commonl y adopted for l ocal oral appl i cati on i n RAS are hydrocorti sone
hemi succi nate (as pel l ets of 2. 5 mg) and tri amci nol one acetoni de (i n an adhesi ve paste
contai ni ng 0. 1 per cent of the steroi d). There i s l i ttl e ri sk of adrenal suppressi on provi ded
that the recommended dose (four ti mes dai l y) i s adhered to. Li ttl e i s known about the l ong-
term ef fect of smal l dosages of topi cal steroi d therapy i n chi l dren but prescri bi ng of these
shoul d be approached wi th cauti on, i n thi s group of pati ents.
Hydrocorti sone succi nate (2.5 mg) tabl ets (Corl an) are di ssol ved f our ti mes dai l y i n
vi ci ni ty of aphthous ul cer. Start i n the prodromal phase of RAS.
Tri amci nol one (Adcortyl ) i n orabase paste shoul d be appl i ed on a moi st f i nger to dri ed
ul cers, four ti mes dai l y. Many pati ents fi nd thi s preparati on di f fi cul t to use but i t appears to
be retai ned better, i f appl i ed at ni ght.
In Mi RAS unresponsi ve to these preprati ons and i n MjRAS i t may be necessary to use a more
potent steroi d preparati on, such as a betnesol (or tri amci nol one) ri nse or steroi d spray (see
Chapter 3).
P. 58
SYSTEMIC THERAPY FOR RAS
In severe cases of RAS, parti cul arl y MjRAS, i t may be necessary to use some f orm of
systemi c therapy. However, al l drugs have si de-eff ects and ri sks that must be wei ghed
agai nst thei r benef i ts. Apart from predni sol one, a number of systemi c drug therapi es have
been advocated for the treatment of MjRAS (Tabl e 5.8) and, i n some cases, Behet' s
di sease (see next secti on). Thal i domi de has been used successf ul l y for severe RAS that has
fai l ed to respond to other treatment modal i ti es. It has al so proved useful i n HIV-associ ated
oral ul cerati on. Thal i domi de i s a TNF i nhi bi tor that has anti -i nfl ammatory eff ects. Its use
i s l i mi ted because of i ts teratogeni c eff ects. Thal i domi de must not be used for women of
chi l d-beari ng age. Peri pheral neuropathy i s a si de-eff ect of therapy and regul ar nerve
conducti on tests (el ectromyography, EMG) must be carri ed out. Pati ents shoul d recei ve
counsel l i ng, pri or to starti ng drug treatment. Col chi ci ne affects the functi on of pol ymorphs
by i nhi bi ti ng thei r mi grati on to si tes of i nfl ammati on. The use of ci meti di ne (H
2
-receptor
bl ocker), carbenoxol one sodi um, and many other treatment modal i ti es have al so been tri ed
for RAS (Tabl e 5. 8) wi th l i ttl e success.
Thal i domi de can be used f or severe RAS and Behet's syndrome but pati ents must recei ve
counsel l i ng. Thal i domi de must not be used f or women of chi l d-beari ng age. Peri pheral
neuropathy i s a recogni zed si de-eff ect of therapy and pati ents must have nerve conducti on
studi es on a regul ar basi s.
Behet's disease
Behet' s (pronounced Betchet' s) syndrome was fi rst recogni zed i n Turkey and was
ori gi nal l y thought to be a di sease of Medi terranean ori gi n. However, a l arge number of cases
have si nce been reported i n Japan and other countri es. Women are more commonl y af fected
than men. Behet' s syndrome i s a rare di sease characteri zed by a cl assi cal tri ad of RAS
(any of the three cl i ni cal vari ants), geni tal ul cerati on, and i nfl ammatory eye l esi ons. Other
mani festati ons i ncl ude ski n, joi nt, neurol ogi cal , vascul ar, and i ntesti nal di sorders. Up to 90
per cent of af fected pati ents have RAS. Geni tal ul cers are si mi l ar to those of the oral mucosa
and heal i ng may take pl ace wi th scar formati on and l ead to resi dual ti ssue l oss and deformi ty
si mi l ar to that i n the oral cavi ty. The ocul ar i nvol vement i ni ti al l y takes the f orm of anteri or
uvei ti s, a superf i ci al i nfl ammatory l esi on of the anteri or part of the eye, whi ch may become
more severe i n l ater epi sodes and, perhaps, progress to i nvol ve other structures of the eye.
Thi s may l ead to permanent damage by scar f ormati on or, even, to bl i ndness.
The sequence of i nvol vement of the oral and geni tal mucosa i s vari abl e, and the two may not
be i nvol ved si mul taneousl y. Indeed, there may be a consi derabl e i nterval between the
i nvol vements. Ocul ar i nvol vement, however, i s usuall y l ate, occurri ng someti mes af ter many
years of i ntermi ttent oral and geni tal ul cerati on. In the general i zed form of the di sease, ski n
l esi ons of vari ous ki nds may appear, the most characteri sti c bei ng papul es that proceed to
pustul e formati on. It i s i nteresti ng to note that in some pati ents wi th ski n l esi ons there i s a
marked ski n reacti on to trauma. The tendency of steri l e bl i sters to devel op at venepuncture
si tes i s known as pathergy, and i t may be usef ul as a di agnosti c indi cator. Pathergy
i s l ess commonl y reported i n UK pati ents wi th Behet' s di sease.
The neurol ogi cal di sease that may occur i n these pati ents i s the resul t of the appearance of
centres of i nfl ammati on and necrosi s wi thi n the central nervous system. The symptoms are
vari abl e, dependi ng upon the l ocati on of the l esi ons, but i n the earl y stages they may
resembl e those of mul ti pl e scl erosi s. There i s a vascul i ti s, perhaps compl i cated by thromboti c
epi sodes, that may be ei ther l ocal i zed and mi nor or i nvol ve major vessel s. The eff ect on the
joi nts i s that of a non-speci f i c arthropathy. It has been suggested that major and mi nor
cri teri a shoul d be used to arri ve at a di agnosi s of Behet' s di sease. The major cri teri a are
oral ul cerati on, geni tal ul cerati on, eye l esi ons, and ski n l esi ons. The mi nor cri teri a i ncl ude
l esi ons of the nervous system, vascul ar system, joints, gastroi ntesti nal tract, and pul monary
system. However, there i s no agreement

as to the number or types of l esi on necessary to arri ve at the di agnosi s. An i nternati onal
worki ng party has redef i ned the cri teri a for the diagnosi s of Behet' s as the presence of
RAS pl us any two of: recurrent geni tal ul cerati on, eye l esi ons, ski n l esi ons or a posi ti ve
pathergy test (Tabl e 5. 9). It i s the authors vi ewpoi nt that, as thi s i s a progressi ve
condi ti on, often begi nni ng as RAS wi thout any other system i nvol vement, i t i s i mpossi bl e at
any gi ven ti me to di fferenti ate wi th any degree of accuracy between uncompl i cated RAS and
RAS that mi ght eventual l y proceed to orogeni tal ul cerati on or Behet' s di sease.
Management
The management of Behet' s syndrome usual l y requi res a mul ti di sci pl i nary approach.
However, the l ocal management of oral aphthae i n Behet' s syndrome i s exactl y that for al l
other forms of RAS and i s si mi l arl y l i mi ted i n eff ect. Systemi c therapy i s theref ore requi red
i n most cases and drugs used i ncl ude: systemi c steroi ds, azathi opri ne, cycl ophosphami de,
col chi ci ne, ci cl ospori n, and, more recentl y, anti -TNF therapy and mycophenol ate.
Thal i domi de appears to be successful i n some cases wi th mucocutaneous i nvol vement but i ts
use i s restri cted because of i ts teratogeni ci ty and si de-eff ects (see above).
Case 5.1 Discussion
The hi story and cl i ni cal exami nati on of the ul cers i n thi s case are consi stent wi th major RAS.
It i s i mportant speci fi cal l y to enqui re about geni tal ul cerati on and other symptoms, whi ch
mi ght be suggesti ve of Behets di sease. The short hi story of ul cerati on i s, however,
suggesti ve of a recent preci pi tati ng cause or factor, whi ch shoul d be sought. It i s i mportant
to establ i sh whether the pati ent stopped smoki ng after hi s myocardi al i nfarcti on and then
devel oped RAS, as smoki ng cessati on can preci pi tate RAS i n some i ndi vi dual s. Stress may
al so have been a contri butory factor. The possi bi l ity of HIV i nfecti on must al so be
consi dered, but i s unl i kel y i n thi s case. Oral ul cerati on has been reported i n pati ents on
ni corandi l , a potassi um-channel acti vator that i s used f or unstabl e angi na, and i t i s i mportant
to rul e thi s out as a cause of thi s pati ent' s oral ul cerati on. (Thi s must not be stopped before
l i ai si ng wi th the cardi ol ogi sts. ) Bl ood tests shoul d be arranged to check f or rai sed
P. 59
Table 5.9 Criteria for the diagnosis of Behet's disease
RAS pl us any two of:
Recurrent geni tal ul cerati on
Eye l esi ons (anteri or uvei ti s, posteri or uvei ti s, reti nal vascul i ti s)
Ski n l esi ons (erythema nodusum, papul opustul ar lesi ons)
Posi ti ve pathergy test (read by physi ci an 2448 h)
Q1 How woul d you manage thi s gentl eman and what therapeuti c opti ons are avai l abl e?
i nfl ammatory markers, haemati ni c defi ci enci es, and anti -endomysi al autoanti bodi es. A f ul l
bi ochemi cal and i mmunol ogi cal profi l e i s al so advi sabl e.
In vi ew of the severi ty of the RAS and thi s pati ent' s poor qual i ty of l i fe, some form of
systemi c therapy needs to be consi dered and the opti ons i ncl ude systemi c steroi ds,
azathi opri ne, ci cl ospori n, thal i domi de, and col chi ci ne. In the case of a young pati ent wi th a
hi story of coronary artery di sease, l ong-term predni sol one i s contrai ndi cated al though a
short, reduci ng course of predni sol one may gi ve short-term rel i ef of ul cerati on. RAS does,
however, eventual l y recur after stoppi ng the steroi d. Topi cal anal gesi cs may be requi red and
an anti fungal shoul d al so be consi dered, parti cul arl y i f there i s a hi gh oral carri age rate of
Candi da al bi cans.
Col chi ci ne has been reported as successful therapy for RAS and azathi opri ne may be worth
consi deri ng, ei ther al one or as a steroi d-spari ng agent. Another treatment opti on for thi s
mal e pati ent i s thal i domi de, al though thi s i s associ ated wi th a number of si gni fi cant si de-
eff ects and the pati ent wi l l need counsel l i ng and cl ose moni tori ng.
There are a number of factors to be consi dered when arrangi ng extracti on of thi s man' s
tooth, i ncl udi ng hi s medi cal and drug hi story (previ ous myocardi al i nfarcti on, aspi ri n) and
anxi ety about dental treatment. He i s probabl y best treated under l ocal anaesthesi a, wi th
sedati on i f requi red. The aspi ri n may predi spose to postextracti on haemorrhage but thi s i s
unl i kel y to be si gni fi cant and there i s no i ndi cation to stop the medi cati on. It i s i mportant to
check for haemostasi s after the extracti on and, i f necessary, suture and/or pack the socket.
If the pati ent devel ops angi na then he shoul d use hi s gl yceryl tri ni trate spray subl i ngual l y. If
there are more severe cardi ac compl i cati ons, then the pati ent must be treated accordi ngl y
(see Chapter 19 on Medi cal emergenci es).
Case 5.2 Discussion
Al though the pati ent appears f i t and wel l , she shoul d be questi oned about any gut, eye, or
ski n probl ems and asked i f she has suf fered from geni tal ul cerati on. Her doctor can arrange
for bl ood tests but i t i s i mportant to di pl omati cal l y poi nt out that these shoul d i ncl ude
esti mati on of ferri ti n, fol ate, and B
12
l evel s, as a f ul l bl ood count and fi l m are i nsuffi ci ent. (It
i s i mportant to establ i sh a good worki ng rel ati onshi p wi th general medi cal practi ti oners i n
your area. )
If thi s pati ent' s cl i ni cal exami nati on i s consi stent wi th a di agnosi s of Mi RAS and there i s no
i ncl i nati on of systemi c di sease, then there are a number of treatment opti ons avai l abl e that
the denti st can prescri be. Anal gesi c ri nses can be hel pful , parti cul arl y bef ore meal s, and an
anti septi c ri nse wi l l reduce secondary i nfecti on and ai d pl aque control , parti cul arl y i f
toothbrushi ng i s pai nful . Hydrocorti sone pel l ets can be used topi cal l y i n the prodromal phase
of ul cerati on and may speed up resol uti on of the ulcers.

Tri amci nol one paste i s di ffi cul t to appl y but may be useful , i f appl i ed l ast thi ng at ni ght. Any
obvi ous cause of mechani cal trauma due to broken teeth or dental appl i ances shoul d be
el i mi nated, i n case these are preci pi tati ng the aphthous ul cers. More potent topi cal steroi ds,
i n the form of ri nses or i nhal ers (see Tabl e 5. 8) may be requi red. In most cases, these
si mpl e measures can reduce the di scomfort and durati on of RAS. It does, however, need to
be poi nted out to thi s pati ent that there i s not, at the present ti me, any sati sfactory
cure for thi s condi ti on and the ri sks of systemic therapy probabl y outwei gh the
benefi ts i n her parti cul ar case.
Project
Q2 How woul d you manage thi s dental emergency?
Q1 How woul d you manage the pati ent i n your practi ce?
P. 60
1. Fi nd out whi ch systemi c drugs have been reported as causi ng oral ul cerati on, i ncl udi ng
RAS.
> Tabl e of Cont ent s > 6 - Di seases of t he l i ps and tongue and di st ur bances of tast e and hal i t osi s
6
Diseases of the lips and tongue and disturbances
of taste and halitosis
Problem cases
Case 6.1
A 20-year-ol d femal e student attends your surgery compl ai ni ng of red patches on her tongue
that move from one area to another. These have been present f or a number of years but
previ ousl y gave no symptoms, provi ded she avoi ded spi cy f oods. Recentl y, she has noti ced
that her tongue i s permanentl y sore. Thi s student al so reports feel i ng ti red al l the ti me and
l ooks rather pal e.
Case 6.2
A 7-year-ol d gi rl , sufferi ng from Down' s syndrome is brought to your surgery for routi ne
dental care. Her mother i s concerned because the gi rl has a persi stent crack i n the centre of
her l ower l i p. She has al so noti ced that her daughter' s tongue l ooks too bi g f or her
mouth. The mother wants to know i f these probl ems are associ ated wi th her Down' s
syndrome and asks whether they can be treated by surgery.
Case 6.3
A 60-year-ol d reti red gardener attends your practi ce requesti ng new dentures. On
exami nati on there i s crusti ng of hi s l ower l i p. Palpati on reveal s areas of thi ckeni ng and some
i ndurati on. On cl oser questi oni ng thi s gentl eman reports that hi s l i p has been l i ke thi s for the
l ast 45 years and i t gets worse i n the col d weather. He has been appl yi ng Vasel i ne to
the l ower l i p.
Introduction
Some l esi ons that affect the l i ps and tongue meri t separate descri pti on. Al though many
condi ti ons that i nvol ve the oral mucosa i n general may al so affect the l i ps and tongue, there
are others i n whi ch these structures are speci f i cal l y i nvol ved. Pati ents wi th these compl ai nts
may be di ffi cul t to di agnose and, i n parti cul ar, i t may be di ff i cul t to di fferenti ate between
Q1 How woul d you manage thi s case?
Q1 What advi ce woul d you gi ve to thi s gi rl ' s mother?
Q2 What other factors must be consi dered when provi di ng dental treatment f or a pati ent
wi th Down' s syndrome?
Q2 What i s the di f ferenti al di agnosi s of the l i p condi ti on?
symptoms that are essenti al l y physi cal i n nature and those that are psychol ogi cal l y i nduced.
Diseases of the lips
The l i ps represent a transi ti onal area between the oral mucous membrane and the ski n of the
face and may, therefore, be i nvol ved i n pathol ogi cal processes aff ecti ng both structures.
Swelling of the lips
There i s consi derabl e i ndi vi dual and raci al vari ation i n the si ze of l i ps. Abnormal swel l i ngs of
the l i ps can ei ther be di ffuse or l ocal i zed to a parti cul ar area of the l i p. Tabl e 6. 1 hi ghl i ghts
some of the causes of both l ocal and di ffuse l i p swel l i ng (readers are encouraged to consul t a
textbook of oral pathol ogy for further i nformati on concerni ng l ocal i zed l esi ons). Pati ents wi th
orof aci al granul omatosi s (OFG) f requentl y present to oral medi ci ne cl i ni cs wi th l i p swel l i ng
that i s cosmeti cal l y di sfi guri ng (Chapter 12). Angioedema, ei ther al l ergi c or non-al l ergi c,
may present i ni ti al l y wi th l i p swel l i ng and be mi staken for a l ocal oral probl em, such as
i nfecti on. Angi oedema i s di scussed f urther i n Chapter 14.
Angular cheilitis (angular stomatitis, cheilosis, perlche)
Angul ar chei l i ti s, the i nfl ammati on of one (or both) of the corners of the mouth, i s a
rel ati vel y common condi ti on. Its rel ati onshi p to candi dal i nfecti ons has been f ul l y di scussed
i n Chapter 4.

Angul ar chei l i ti s i s a mul ti factori al condi ti on wi th a number of l ocal and systemi c
predi sposi ng factors (Fi g. 6. 1).
Angul ar chei l i ti s i s a mul ti factori al condi ti on wi th a number of l ocal and systemi c
predi sposi ng f actors.
In most cases, deep fol ds at the angl es of the mouth become traumati zed as a resul t of
conti nual wetti ng by sal i va. These angul ar f ol ds are the resul t ei ther of anatomi cal
P. 64
Table 6.1 Causes of lip swelling
Diffuse Localized
Angi oedema (al l ergi c/non-al l ergi c) Mucocoel e
Oedema (trauma or i nfecti on) Abscess
Orofaci al granul omatosi s (OFG) Haematoma
Crohn' s di sease Sal i vary adenoma
Haemangi oma Basal cel l carci noma
Lymphangi oma Squamous cel l carci noma
confi gurati ons or, i n many cases, unsati sfactory dentures, parti cul arl y those wi th a
decreased verti cal di mensi on. Secondary i nf ecti on by C. al bi cans (Fi g. 6. 2), S. aureus (or
both), and other bacteri a, i ncl udi ng B-haemol yti c streptococci , fol l ows. In some cases
angul ar chei l i ti s i s the resul t of an underl yi ng systemi c condi ti on or defi ci ency that resul ts i n
the host' s def ences, bei ng compromi sed (Tabl e 6. 2). A systemati c di agnosti c approach must
be used i n these pati ents (Tabl e 6. 3) and management i s di ctated by the resul ts of
i nvesti gati ons (Tabl e 6. 4). Anti mi crobi al therapy for angul ar chei l i ti s i s summari zed i n Tabl e
6. 5. Anti fungal therapy has been ful l y di scussed i n Chapter 4.
A few other condi ti ons may resul t i n a more speci fi c form of angul ar chei l i ti s. For i nstance,
faci al eczema may present at the angl es of the mouth and mi mi c a si mpl e angul ar chei l i ti s.
In thi s si tuati on the di agnosi s l argel y depends on the hi story of the

pati ent. It i s unl i kel y that a pati ent woul d i ni ti al l y present wi th eczema aff ecti ng the angl es
of the mouth onl y. The rapi d response to l ocal steroi d appl i cati ons (such as 1 per cent
hydrocorti sone cream) hel ps to conf i rm the di agnosis. In Crohn' s di sease angul ar chei l i ti s i s
a si gni f i cant di agnosti c i ndi cator when associ ated wi th other markers of the di sease (Chapter
12). Thi s i s a paral l el to the anal f i ssures seen in thi s condi ti on and, si mi l arl y, responds
readi l y to l ocal steroi d therapy.
Fig. 6.1 Factors i nvol ved i n the pathogenesi s of angul ar chei l i ti s. (Adapted wi th
permi ssi on f rom Fi g. 26. 13 i n Bagg, J. , et al . (1999). Essenti al s of mi crobi ol ogy for
dental students. Oxford Uni versi ty Press, Oxford. )
P. 65
Fig. 6.2 Angul ar chei l i ti s wi th Candi da present.
Table 6.2 Angular cheilitis as a marker of systemic
disease
Anaemi a
Iron, B
12
, or fol ate defi ci ency
OFG (i ncl udi ng Crohn' s di sease)
HIV i nfecti on
Di abetes mel l i tus
Sj gren' s syndrome (and other causes of sal i vary gl and hypof uncti on)
Table 6.3 The investigation of patients presenting with
angular cheilitis
Hi story: key questi ons
General i zed i l l heal th?
Known systemi c di sease?
Xerogeni c medi cati on?
Anti bi oti c or steroi d medi cati on?
Soci al hi story, i ncl udi ng tobacco use?
Il l -fi tti ng dentures?
Dentures worn at ni ght?
Therapeuti c radi ati on?
Cytotoxi c drugs?
Exami nati on: check for
Si gns of anaemi a
Lymphadenopathy
Sal i vary gl and swel l i ng
Intraoral candi dosi s
Denture-i nduced erythematous candi dosi s
Commi ssural l eukopl aki a (chroni c hyperpl asti c candidosi s)
Oral dryness
Mani festati ons of OFG
Il l -fi tti ng dentures/reduced verti cal di mensi on
Speci al i nvesti gati ons
Mi crobi ol ogy
Sampl i ngtechni que (smear, swab, and oral ri nse, i f avai l abl e).
Sampl i ngsi te (angl es, pal ate, fi tti ng-surface of denture, anteri or nares)
Bl ood tests
Indi cated i f suspi ci on of underl yi ng systemi c factor or l ocal therapeuti c measures
fai l to resol ve angul ar chei l i ti s
Ful l bl ood count
Serum B
12
, ferri ti n, serum and red cel l fol ate (or whol e bl ood f ol ate)
Bl ood gl ucose
Other tests
As i ndi cated by cl i ni cal fi ndi ngs
Table 6.4 The management of patients with angular
cheilitis
El i mi nati on of predi sposi ng l ocal factors
Check dentures and denture hygi ene
Instruct pati ent to l eave out dentures at ni ght
Referral for treatment of underl yi ng defi ci enci es or systemi c di sease
Provi si on of appropri ate anti mi crobi al therapy (topi cal or systemi c)as i ndi cated
by mi crobi ol ogy (see Tabl e 6. 5)
Table 6.5 Antimicrobial therapy for angular cheilitis*
Lip fissures
Verti cal cracks i n the l i ps are l ess commonl y seen than angul ar chei l i ti s and the most
common si te i s i n the mi dl i ne of the l ower l i p. Such fi ssures are often remarkabl y resi stant to
conservati ve treatment and surgi cal exci si on has been occasi onal l y advocated for the more
resi stant l esi ons. The majori ty of these f i ssures are i nfected by Staphyl ococcus aureus and
the most sati sfactory form of treatment consi sts of the el i mi nati on of the secondary i nf ecti on
(by the use of sui tabl e anti bi oti c creams) fol l owed by the use of steroi d creams. Wi th such a
regi me most of these cracks can be persuaded to heal , at l east temporari l y. A short course of
a systemi c anti bi oti c may be requi red i n some persistent casesthe drug of choi ce i s
i ndi cated by cul ture and sensi ti vi ty of the putati ve bacteri a. Shoul d the predomi nant
organi sm i nvol ved not be a Staphyl ococcus, but Candi da, then the treatment shoul d be
modi fi ed sui tabl y. The basi c pri nci pl e, however, of the el i mi nati on of secondary i nf ecti on
fol l owed by the use of topi cal steroi d i s the one most l i kel y to be successful . Li p cracks of
thi s nature show a marked tendency to

reoccur and a permanent cure i s unl i kel y. Li p fi ssures are commonl y seen i n pati ents wi th
Down' s syndrome, together wi th angul ar chei l i ti spossi bl y as a resul t of mouth breathi ng.
Li p fi ssuri ng i s al so a feature of OFG (Chapter 12).
Chroni c l i p fi ssures are often resi stant to conservati ve treatment and may requi re surgi cal
management.
Allergic cheilitis
Irri tati on and scal i ng of the l i ps may resul t from a contact al l ergy to a wi de vari ety of
substances, i n parti cul ar, to the consti tuents of li psti cks. However, other topi cal preparati ons
used on the l i ps, such as l i p oi ntments or moi sturi zers, toothpastes, and, occasi onal l y, foods,
may cause an al l ergi c chei l i ti s. In general , the i rri tati on i s conf i ned to the vermi l i on of the
l i p, but may extend beyond i t i n an eczema-l i ke i rri tati on of the peri oral ski n. Thi s form of
contact sensi ti vi ty i s of ten di ff i cul t to di agnose and requi res detai l ed hi story taki ng. For
Candi da i sol ated
Intraoral

: nystati n pasti l l es or amphoteri ci n l ozenges (el i mi nate oral reservoi r
of i nfecti on)
Angl es: nystati n oi ntment
S. aureus i sol ated
Angl es: fusi di c aci d cream
Anteri or nares: mupi roci n cream or fusi di c aci d cream.
Mi xed i nfecti ons
Mi conazol e ge1 or cream
* Systemi c fl uconazol e may be requi red to treat angul ar chei l i ti s resi stant to topi cal
therapy or for i mmunocompromi sed pati ents.
Chl orhexi di ne sol uti on has anti bacteri al and anti candi dal acti vi ty and can be
used as a ri nse. Chl orhexi di ne may parti al l y i nhi bit the acti on of nystati n.
Mi conazol e can al so be used i f i nfecti ng mi cro-organi sms are unknowni t has
anti -staphyl ococcal acti vi ty. Mi conazol e i s not the drug of choi ce for Candi da
i nfecti ons because of vari abl e sensi ti vi ty and possi bl e devel opment of azol e
resi stance.
P. 66
i nstance, a l i psti ck al l ergy may be caused by the mi ni mal amounts transferred i n ki ssi ng and
so may remai n qui te unsuspected. In some i nstances the i rri tati on may fol l ow the acti on of
sunl i ght on the l i ps. Thi s may not be a pri mary acti ni c reacti on, but may be secondary to a
photosensi ti zi ng ef fect of substances, such as those i n l i psti cks, whi ch mi ght i n other
subjects cause di rect contact chei l i ti s. Thi s i s rather di f ferent from the acti ni c chei l i ti s that
may occur i n some pati ents who work i n ful l sunl i ght for many years (see bel ow). In the case
of a si mpl e contact chei l i ti s, treatment consi sts of traci ng the sensi ti zi ng substance and
el i mi nati ng i t. Patch-testi ng may be necessary to conf i rm the putati ve al l ergen. Temporary
rel i ef may be gi ven by use of topi cal steroi ds, but these shoul d not be conti nued on a l ong-
term basi s.
Actinic cheilitis (solar keratosis)
In a number of predomi nantl y mal e pati ents, l ong exposure to sunl i ght, often the resul t of
outdoor work i n a sunny cl i mate, i s f ol l owed by the onset of a chei l i ti s i n whi ch epi thel i al
atypi a may occur wi th progressi on to carci noma i n some cases. Thi s i s al most al ways most
marked i n the l ower l i pthe so-cal l ed l i p at ri sk. Crusti ng and i ndurati on of the
vermi l i on margi n occurs, the i ndurati on bei ng due, i n part, to a fi broti c reacti on of the
connecti ve ti ssues (Fi g. 6. 3). Bi opsy i s necessary for a ful l assessmentthe cl i ni cal
appearance al one i s an i nsuffi ci ent gui de to the presence of worryi ng changes i n the
epi thel i um. If , on bi opsy, si gni fi cant dyspl asi a i s found, then exci si on of the vermi l i on area
by a l i p shave operati on or l aser treatment shoul d be consi dered. Chemi cal exfol i ants can
al so be used.
Prol onged exposure to sunl i ght, ei ther occupati onal or recreati onal , can resul t i n the
devel opment of an acti ni c chei l i ti s.
Exfoliative cheilitis
Exfol i ati ve chei l i ti s i s an uncommon condi ti on affecti ng onl y the vermi l i on borders of the l i ps
and characteri zed by the producti on of excessi ve amounts of kerati n. Thi s forms brown scal es
that may be spontaneousl y shed or may be removed by the pati ent (Fi g. 6. 4). It has been
suggested that thi s condi ti on i s excl usi vel y seen i n femal e pati ents, but several cases have
recentl y been reported i n mal es. The hi stol ogy of the l esi on i s of a si mpl e hyperparakeratosi s
and i t i s not consi dered to be a premal i gnant l esi on. There i s no apparent systemi c
background to thi s condi ti on but some pati ents report an associ ati on wi th stress, the degree
of kerati n formati on apparentl y i ncreasi ng at ti mes of i ncreased anxi ety. Al l systemi c
i nvesti gati ons i n these pati ents prove negati ve and al l forms of treatment have as yet proved
unhel pf ul . Local and systemi c steroi ds, cautery, cryosurgery, and many other forms of
treatment have been attempted wi thout success. In those wi th an apparent anxi ety-rel ated
condi ti on, mi l d tranqui l l i zati on has been reported as hel pful , al though the mechani sm for thi s
i s far f rom cl ear. Anti depressant medi cati on has also been prescri bed for a few of these
cases, wi th some degree of success. The authors experi ence wi th thi s condi ti on i s that
aff ected i ndi vi dual s of ten appear to have a personal i ty di sorder. The extent to whi ch there i s
a f i cti ti ous el ement i nvol ved i s di ffi cul t to quanti f y. Most pati ents tend to peel of f the
exf ol i ati ng ski n, but thi s coul d be a normal response under the ci rcumstances. Eventual l y,
thi s condi ti on appears to resol ve spontaneousl y but, whi l e present, i t i s suffi ci ent to cause
the pati ent consi derabl e di stress.

Perioral dermatitis
Thi s rel ati vel y uncommon condi ti on i s often a considerabl e di agnosti c probl em and the
pati ents are from ti me to ti me seen i n the oral medi ci ne cl i ni c f or i nvesti gati on. The pati ents
are al most al ways young adul t femal es and compl ai n of an erythematous rash on the f aci al
ski n around the mouth (Fi g. 6. 5). In some cases, but not al l , there are papul es present. In
some pati ents there i s a hi story of the use of steroi d creams on the face, but thi s i s not by
any means i nvari abl e. Peri oral dermati ti s can al so be due to a contact al l ergy (see al l ergi c
chei l i ti s).
Fig. 6.3 Sol ar keratosi s of the l ower l i p.
Fig. 6.4 Exfol i ati ve chei l i ti s.
P. 67
The di agnosi s i s essenti al l y cl i ni cal there are no systemi c changes detectabl e. Many of
those pati ents wi th papul es present respond to a l ong-term l ow-dose regi me of tetracycl i nes,
much as i n acne. Paradoxi cal l y, some pati ents respond wel l to l ow-potency steroi d creams,
such as 1 per cent hydrocorti sone. As i n al l cases where the f aci al ski n i s i nvol ved, cauti on
must be exerci sed i n the use of more potent steroi d preparati ons because of the possi bi l i ty of
atrophi c changes. In vi ew of the wi de range of presentati on and reacti on to therapy, i t i s
possi bl e that peri oral dermati ti s represents a reacti on pattern rather than a si ngl e enti ty,
and that more than one basi c aeti ol ogi cal process may be i nvol ved.
Lick eczema
Thi s i s a f urther peri oral condi ti on that often causes di agnosti c conf usi on. The pati ents are
youngof ten chi l drenand compl ai n of a sharpl y del i neated zone of i rri tabl e, scal y ski n
around the mouth. Thi s condi ti on, as i ts name i mpl ies, i s si mpl y the resul t of a l i cki ng habi t
that the pati ent may strongl y deny, even when actual l y performi ng the l i cki ng acti on duri ng
the exami nati on! The treatment i s to stop the habi t. If thi s i s done, the l esi on rapi dl y f ades.
Such a wel l -i ngrai ned habi t (of whi ch the pati ent may be enti rel y unconsci ous) may,
however, be di ffi cul t to eradi cate. A removabl e appl i ance wi th a rough or sharp edge to
i nterf ere wi th the tongue acti on i s often successf ul .
Cheilocandidosis
There have been several reported cases i n whi ch the l i ps become the si te for a heavy
candi dal i nfecti on. These l esi ons are reported as occurri ng bi l ateral l y, predomi nantl y on the
l ower l i p, and appear as ul cerated granul ar areas from whi ch C. al bi cans can be freel y
cul tured. Thi s i s reported to occur i n general l y heal thy pati ents, but there are strong
suggesti ons of pri or l ocal abnormal i ty that mi ght lead to a secondary candi dal i nf ecti on, for
exampl e, sol ar i rri tati on i n some Austral i an pati ents. In some cases the chei l i ti s has been
associ ated wi th chroni c i ntraoral candi dosi s. These l esi ons have been consi dered by several
authors to represent candi dal i nfecti on affecti ng i ntri nsi cal l y unstabl e epi thel i um and i t i s
suggested that, as i n chroni c hyperpl asti c candi dosi s (candi dal l eukopl aki a), earl y treatment
by anti f ungal s mi ght l ead to resol uti on, whereas del ay mi ght l ead to i ncreasi ng epi thel i al
dyspl asi a.
Fig. 6.5 Peri oral dermati ti s i n a young femal e pati ent.
Diseases of the tongue
The tongue may be i nvol ved i n a wi de range of oral mucosal di seases. Apart from thi s
i nvol vement i n general i zed oral condi ti ons, several l esi ons are qui te speci fi c to the tongue.
Many of these depend on al terati ons i n the speci al ized epi thel i al coveri ng of the tongue and,
i n parti cul ar, i n the f i l i form papi l l ae. These structures appear to be parti cul arl y suscepti bl e to
changes brought about by systemi c abnormal i ti es. For i nstance, i n anaemi as (i n whi ch the
oral mucosa as a whol e may undergo some change), the predomi nant oral abnormal i ty may
be of the tongue. When a l esi on of any ki nd i s present on the tongue, i t may be brought
rapi dl y to the noti ce of the pati ent because of the mobi l i ty of the organ and i ts ri ch suppl y of
sensory nerve endi ngs. Thi s, however, i s by no means i nvari abl y the case and there are
many exampl es of l esi ons (for exampl e, the wi despread ul cerati on of major erosi ve l i chen
pl anus) i n whi ch the pati ent compl ai ns of far l ess pai n than mi ght be expected. Of greater
si gni f i cance i s the fact that neopl asms of the tongue may grow to a consi derabl e si ze bef ore
secondary i nf ecti on l eads to symptoms of pai n (an earl y carci noma of the tongue may be
qui te pai nl ess).
Developmental abnormalities and morphological variations
Si gni f i cant devel opmental abnormal i ti es of the tongue are rare. Ankyl ogl ossi a (tongue ti e) i s
usual l y congeni tal and may be associ ated wi th mi crogl ossi a. Contrary to popul ar opi ni on,
there i s no evi dence that ankyl ogl ossi a i nterferes wi th speech, al though pati ents may fi nd
that the restri cted mobi l i ty i nterferes wi th the mechani cal cl eansi ng acti on of the tongue.
Macrogl ossi a can occur i n a number of systemi c condi ti ons, such as congeni tal
hypothyroi di sm, Down' s syndrome, acromegal y, and amyl oi dosi s.
Tongue fissures
The normal gross structure of the tongue i ncl udes fi ssures, whi ch may show vari ati on i n
number, depth, and arrangement. There i s consi derabl e doubt as to the extent to whi ch the
pattern of fi ssures may change duri ng di sease processes, al though i t does

seem that i n a f ew condi ti ons, for exampl e, chroni c mucocutaneous candi dosi s, the fi ssures
appear so exaggerated and of such abnormal form that they must be consi dered as part of
the pathol ogi cal change (Fi g. 6. 6). In general , however, there i s l i ttl e evi dence of changes i n
fi ssure pattern due to l ess exaggerated pathol ogi es. There i s no doubt, however, that
fi ssures of normal f orm and di stri buti on may pl ay a part i n the modi fi cati on, or even the
i ni ti ati on, of pathol ogi cal processes, si nce condi ti ons of anaerobi c stasi s are present i n the
depths of these structures and present opportuni ti es for sel ecti ve bacteri al growth. Thi s
bei ng so, i t i s common to f i nd superf i ci al i nfecti ons and si mi l ar condi ti ons concentrated at
and al ongsi de the fi ssures. Si mi l arl y, i n a si tuation that may l ead to a sore and i rri tated
tongue, the fi ssures may be the fi rst and the most severel y aff ected part. The use of a
chl orhexi di ne mouthwash may be hel pful for pati ents wi th a symptomati c fi ssured tongue.
P. 68
The so-cal l ed scrotal tongue i s a normal morphol ogi cal vari ant wi th mul ti pl e fi ssures
on the dorsum of the tongue. The appearance of an irregul ar border of the tongue (of ten sai d
to resembl e a pi e-crust edge) i s known as a crenated tongue and can be one
of the features of bruxi sm i n some pati ents.
Coated tongue
The tongues of al l normal i ndi vi dual s have a coati ng consi sti ng of a l ayer of mucus,
desquamated epi thel i al cel l s, organi sms, and debri s. In the heal thy i ndi vi dual the tongue i s
mobi l e, there i s a rapi d fl ow of sal i va, and thi s coati ng i s kept to a mi ni mum. Wi th the
sl i ghtest di sturbance to the heal th of the i ndi vi dual , however, the bal ance i s upset and the
coati ng may qui ckl y become very much thi cker. A l ack of mobi l i ty of the tongue, whi ch may
be caused by the most mi nor pai nful l esi ons, a di sturbance i n sal i va fl ow, an excess of
tobacco or of al cohol , a gastri c or respi ratory upset, or a febri l e condi ti on, may resul t i n a
bui l d-up of the tongue coati ng suffi ci ent to produce a whi te or col oured pl aque. The col our of
such a coati ng depends on a vari ety of factors, such as tobacco usage and di etary habi ts,
and i s of very l i ttl e di agnosti c si gni f i cance. Such a coati ng may, however, be unpl easant to
the pati ent and i ts removal may be qui te di ffi cul t si nce very often i t i s fi rml y adherent to the
tongue. Pati ents frequentl y become overl y aware and sel f -consci ous about thei r coated
tongue and need reassurance. In a few pati ents their coated tongue becomes an obsessi on.
Vi gorous brushi ng of the tongue i s often advocated and tongue scrapers are now
commerci al l y avai l abl e. Use of these i s someti mes found to be most unpl easant by the
pati ent. Eff ervescent mouthwashes, such as those contai ni ng ascorbi c aci d, may be hel pf ul ,
parti cul arl y i f combi ned wi th brushi ng of the tongue.
Hairy tongue
In hai ry tongue the l esi on does not consi st si mpl y of a coati ng on the surf ace of the tongue
but represents an el ongati on of the fi l i form papi l l ae, often to many ti mes thei r ori gi nal
l ength. Wi th thi s el ongati on the papi l l ae often take on a dark col our, bl ack or brown bei ng
common (Fi g. 6. 7). The mechani sm for the formati on of these col oured hai ry tongues i s qui te
unknown, there apparentl y bei ng many i ni ti ati ng factors. For i nstance, hai ry tongue
Fig. 6.6 Deep fi ssures of the tongue i n a pati ent wi th chroni c mucocutaneous
candi dosi s.
frequentl y fol l ows a course of anti bi oti c therapy and may resol ve qui te rapi dl y on compl eti on
of treatment. Other hai ry tongues apparentl y appear compl etel y spontaneousl y and no cause
i s ever found f or them. Equal l y doubtful i s the source of the pi gment i nvol ved. It i s usual to
rel ate thi s to pi gment-produci ng organi sms entrapped wi thi n the papi l l ae but, i n fact, no
such organi sms have ever been demonstrated. In the past, the presence of hai ry tongue was
of ten ascri bed to candi dal i nfecti on but, agai n, i t has never been shown that there i s any
true associ ati on between candi dosi s and the producti on of the el ongated papi l l ae.
Treatment of hai ry tongue i s remarkabl y di ff i cul t. Those cases associ ated wi th anti bi oti c
therapy frequentl y, but not i nvari abl y, resol ve when the medi cati on i s f i ni shed. The use of
eff ervescent and mucus-sol vent mouthwashes may be hel pful i n reduci ng secondary i rri tati on
and thereby produci ng sui tabl e condi ti ons for the resol uti on of the abnormal i ty but, agai n,
the resul ts are vari abl e. The authors have used chemi cal cauteri zati on, wi th tri chl oraceti c
aci d, to treat hai ry tongue, but onl y smal l areas can be managed at a ti me, because of
di scomf ort af terwards. Long-term

resul ts of thi s chemi cal counteri zati on were di sappoi nti ng. Sucki ng a dry peach stone
has been advocated for the management of hai ry tongue but thi s approach does seem to be
potenti al l y hazardous! Some pati ents wi th thi s condi ti on use tongue scrapers or brush thei r
tongue vi gorousl y but these measures are rarel y effecti ve.
Atrophy of the lingual epithelium
At the opposi te extreme from the prol i ferati on of the fi l i f orm papi l l ae seen i n hai ry tongue
are the atrophi c changes i n the papi l l ae f ound i n a number of general i zed di seases but, i n
parti cul ar, i n haematol ogi cal and nutri ti onal abnormal i ti es. Atrophi c change i n the oral
epi thel i um as a whol e may be the consequence of a wi de range of abnormal i ti es (f or
i nstance, i ron defi ci ency, megal obl asti c anaemi as, and nutri ti onal def i ci enci es of vari ous
ki nds). These atrophi c changes are someti mes, but not i nvari abl y, found predomi nantl y i n
the fi l i form papi l l ae, and the surface of the tongue may become red, shi ny, and pai nf ul . It i s
now accepted that a wi de spectrum of general i zed abnormal i ti es may be responsi bl e for the
producti on of si mi l ar epi thel i al changes. Conversel y, a wi de range of oral si gns and
symptoms may occur i n di ff erent pati ents wi th si mi lar condi ti ons. It i s certai nl y true,
however, that al l pati ents wi th pai nf ul , depapi l l ated, or reddened tongues shoul d undergo
bl ood tests i ncl udi ng esti mati ons of serum, ferri ti n, B
12
, fol ate, and gl ucose to el i mi nate the
P. 69
Fig. 6.7 Bl ack hai ry tongue.
possi bi l i ty of an underl yi ng haemati ni c def i ci ency or undi agnosed di abetes.
A sore tongue may be f ound i n associ ati on wi th many general i zed condi ti ons, parti cul arl y the
anaemi as and sal i vary gl and hypofuncti on. For i nstance, i n rheumatoi d arthri ti s, where there
may be an anaemi a of chroni c di sease, i t i s not unusual to f i nd a sore tongue. Si mi l arl y, i n
Sjgren' s syndrome, i n whi ch the sal i vary f l ow i s reduced, the tongue may become dry,
reddened, or pai nf ul (Chapter 8). There are, however, some pati ents i n whom no cause can
be found for the atrophi c changes. In ol der pati ents the atrophy may be regarded as an age
change, but i t i s not cl ear to what extent thi s may represent subcl i ni cal systemi c changes. In
some condi ti ons i n whi ch depapi l l ati on of the tongue has taken pl ace, the ori gi nal
morphol ogy of the l i ngual epi thel i um may not return on heal i ng. For i nstance, i n major
erosi ve l i chen pl anus a l arge area of the tongue may be ul cerated. On heal i ng, the epi thel i um
i s often vi rtual l y devoi d of f i l i form papi l l ae appeari ng as a fl at, featurel ess ti ssue.
In addi ti on to those pati ents i n whom a recogni zable pathol ogi cal condi ti on exi sts, there i s a
group, l argel y of ol der femal e pati ents, who compl ai n of tenderness and soreness of the
tongue, and who show no obvi ous si gns to account for these symptoms. Al l tests may show
negati ve resul ts and i t i s tempti ng to cl assi fy such symptoms as bei ng psychogeni c i n ori gi n.
There i s no doubt that i n some pati ents thi s may be so, but the possi bi l i ty of habi t-i nduced
i rri tati on must al ways be consi dered i n such cases. Gl ossodyni a and burni ng mouth syndrome
(BMS) are consi dered ful l y i n Chapter 17.
Traumatic irritation of the tongue
The mobi l i ty of the tongue makes i t parti cul arl y suscepti bl e to trauma, whi ch may be acute
(as that fol l owi ng the f racture of a cari ous tooth) or chroni c (as that caused, for exampl e, by
the adopti on of a habi tual acti vi ty such as rubbi ng the tongue over the edges of the anteri or
teeth or a denture). The l esi ons produced vary greatl y, from mi l dl y erythematous patches
of ten seen on the ti p of the tongue to frank ul cerati on. These traumati c ul cers are often
extremel y pai nf ul and, al though they may be evi dentl y rel ated to the sharp edge i n questi on,
they may show some degree of i ndurati on due to l ocal i zed i nfl ammatory changes together
wi th whi teness of the surroundi ng epi thel i umchanges that arouse i mmedi ate suspi ci on of
mal i gnancy. It has been ampl y demonstrated, however, that superfi ci al ul cers of the tongue,
surrounded by a whi te margi n, are vi rtual l y al ways beni gn and of traumati c ori gi n. The
easi est way to al l ay these suspi ci ons i s to remove the off endi ng tooth or appl i ance, or to
deal wi th i t i n some other sui tabl e way i n order to el i mi nate the trauma compl etel y.
Traumati c ul cers resol ve very rapi dl y and vi rtual l y al l trace may be expected to have
di sappeared wi thi n a week of removi ng the i rri tant. If thi s resol uti on has not occurred i n the
expected ti me, then the l esi on must be vi ewed wi th suspi ci on and a bi opsy taken. In the
tongue, as i n other areas, there i s l i ttl e evi dence of a rel ati onshi p between physi cal trauma
and mal i gnancy, but the possi bi l i ty must obvi ousl y be consi dered.
More di ff i cul t to di agnose are the more chroni c forms of l i ngual i rri tati on resul ti ng f rom
habi ts of vari ous ki nds (for exampl e, tongue bi ti ng). These often resul t i n no obvi ous oral
l esi on, but frequentl y cause the pati ent a great deal of concern. In these cases i t may be
di f fi cul t to deci de whether any systemi c factor i s i nvol ved i n the producti on of the symptoms
descri bed by the pati ent and i t may be necessary to carry out a ful l seri es of haematol ogi cal
i nvesti gati ons before comi ng to the concl usi on that a habi t i s the onl y f actor i nvol ved. In
parti cul ar, there are a number of pati ents i n whom the i rri tati on of the ti p of the tongue and,
of ten al so the l ower l abi al mucosa, fol l ows the most mi nor change i n morphol ogy of the
anteri or denti ti on, for exampl e, by the repl acement of a crown or the i nserti on of a
repl acement parti al denture. Frequentl y, there i s no di scerni bl e abnormal i ty i n the
restorati on to account for thi s. Nonethel ess, i t i s evi dent from the mi l d erythema of both the
ti p of the tongue and the mucosa of the l ower l i p that trauma i s occurri ng. It i s of ten di ffi cul t
to hel p such pati ents i n the absence of any di scerni bl e faul t i n the restorati on.
Enlargement of the foliate papillae
The f ol i ate papi l l ae appear as bi l ateral , pi nk nodul es on the si de of the tongue, at the
juncti on of the anteri or two-thi rds and posteri or thi rd. They can become enl arged and
i nfl amed (f ol i ate papi l l i ti s) and cause soreness to the pati ent. The appearance of these may
cause concern to pati ents, who shoul d be adequatel y reassured.

Geographic tongue (erythema migrans, benign
migratory glossitis)
Depapi l l ati on of the tongue i s al so a marked feature of the condi ti on usual l y descri bed as
geographi c tongue or erythema mi grans. The pattern of depapi l l ati on i s qui te
characteri sti c wi th the af fected areas occurri ng as red patches surrounded by whi te borders.
The patches are di stri buted over the surf ace of the tongue i n a map-l i ke fashi on
(hence the name geographi c tongue) and tend to vary posi ti on, apparentl y movi ng
about the surface of the tongue, hence the name erythema mi grans (or beni gn mi gratory
gl ossi ti s) (Fi g. 6. 8). The appearance of the l esi ons may be the pati ent' s onl y compl ai nt but
some pati ents compl ai n of tenderness of the tongue, parti cul arl y when eati ng hi ghl y
fl avoured f ood. Thi s condi ti on of ten proves extremel y worryi ng to the pati ent i n vi ew of i ts
of ten qui te spectacul ar appearance. The age range of the pati ents i nvol ved i s very wi de.
Al though geographi c tongue i s general l y consi dered to be a condi ti on af fecti ng adul ts, the
authors have seen pati ents from the age of 3 years upwards. The associ ati on of geographi c
tongue wi th psori asi s has been reported i n a number of studi es. A f urther suggested
associ ati on i s between geographi c tongue and deep fi ssuri ng. Agai n, there i s no cl ear
evi dence that thi s i s other than a random associ ation of two rel ati vel y common condi ti ons.
Erythema mi grans can occur el sewhere on the oral mucosa and has been reported aff ecti ng
the pal ate and l abi al mucosa.
The aeti ol ogy of erythema mi grans i s unknown. It i s rarel y associ ated wi th any underl yi ng
systemi c di sorder al though, shoul d there be a haemati ni c defi ci ency such as anaemi a, there
may be an i ncrease i n the tenderness of the tongue. Di agnosi s i s i n general si mpl e, bei ng
based on the characteri sti c appearance and behavi our of l esi ons, al though i t may someti mes
be necessary to see the pati ent on several occasi ons to conf i rm the mi gratory nature of the
condi ti on. If the tongue i s symptomati c, then appropri ate bl ood tests shoul d be carri ed out to
el i mi nate any underl yi ng haemati ni c defi ci ency. In some pati ents the l esi ons may be
somewhat l ess def i ned and wi th a marked whi te border, al though, i n most cases, at l east a
smal l area of whi te demarcati on may be seen. In other pati ents the l esi ons may occur i n a
more stati c manner or i n a si ngl e si te and thi s may cause confusi on wi th a traumati c l esi on.
There are, however, f ew cases of geographi c tongue that cannot eventual l y be di agnosed on
a cl i ni cal basi s al one. Bi opsy i s rarel y necessary to conf i rm di agnosi s, but shoul d be
consi dered i f there i s a possi bi l i ty of si ni ster pathol ogy. No successful treatment i s known f or
a pai nful geographi c tongue, but an anal gesi c mouthwash may provi de symptomati c rel i ef.
Most pati ents l earn to avoi d foodstuff s that i rri tate thei r tongue. Some authori ti es have
suggested the use of zi nc suppl ements for geographi c tongue but the evi dence for thi s bei ng
eff i caci ous i s mai nl y anecdotal .
P. 70
Median rhomboid glossitis
The tongue may be i nvol ved i n candi dal i nfecti on (Chapter 4). Thi s i s parti cul arl y so i n the
chroni c i nfecti ons consequent to i mmunol ogi cal def ects (such as chroni c mucocutaneous
candi dosi s) or i n debi l i tati ng chroni c di seases. In such cases the tongue may not onl y be
covered by pl aques of candi dal pseudomembrane, but may al so become deepl y fi ssured and
apparentl y f i brosed. It shoul d be stressed that candi dosi s of the tongue, as i n the oral cavi ty
i n general , may wel l si gnal underl yi ng systemi c di sease, such as di abetes or i ron def i ci ency.
Lesi ons of superf i ci al mi dl i ne gl ossi ti s are usual ly seen i n apparentl y heal thy pati ents. These
are, by defi ni ti on, i n the mi dl i ne of the tongue i n si tes varyi ng from i mmedi atel y i n front of
the val l ate papi l l ae (where the condi ti on i s known as medi an rhomboi d gl ossi ti s
because of the characteri sti c shape of the l esi on in that si te) towards the anteri or dorsal
surface of the tongue. Medi an rhomboi d gl ossi ti s has f or l ong been consi dered to be a
devel opmental abnormal i ty i n some way connected wi th the si te of the embryoni c tubercul um
i mpar. It has now been recogni zed that these l esi ons are often associ ated wi th the presence
of Candi da.
Fig. 6.8 Geographi c tongue (erythema mi grans).
Medi an rhomboi d gl ossi ti s appears as an area of depapi l l ati on on the dorsum of the tongue
and may have a red, whi te, or yel l ow appearance (Fi g. 6. 9). Long-term therapy wi th topi cal
anti f ungal agents, f or exampl e, nystati n pasti l l es, may be effecti ve i n reduci ng the si ze of
the l esi on, and recent success has been reported with systemi c anti f ungal agents (for
exampl e, fl uconazol e). Not al l l esi ons di sappear ful l y wi th thi s treatment. Any doubt as to
the nature of the condi ti on shoul d be resol ved by bi opsy and hi stopathol ogi cal exami nati on.
In general , however, the characteri sti c appearance, si te, and texture of the l esi on i s such as
to enabl e a confi dent i ni ti al di agnosi s to be made on cl i ni cal

grounds. Pati ents presenti ng wi th thi s l esi on should undergo bl ood tests to excl ude an
underl yi ng haemati ni c defi ci ency or di abetes. Medi an rhomboi d gl ossi ti s may al so be seen i n
pati ents i nfected wi th HIV.
Disturbances of taste and halitosis
Disturbances of taste
Hypogeusi a i s a reduced taste sensati on and ageusi a i s a compl ete l oss of
taste. An unpl easant or al tered taste sensati on i s known as dysgeusi a. Pati ents
compl ai ni ng of di sturbance of taste sensati on are among the most di ff i cul t to manage. There
i s often nothi ng other than the pati ent' s own descri pti on to characteri ze the condi ti on and
objecti ve tests are often not hel pful . Physi ol ogi cal tests to determi ne the pati ent' s abi l i ty to
di f ferenti ate the basi c taste sensati ons of sweet, sour, bi tter, and sal t may be done, but
these i n themsel ves may gi ve l i ttl e i nformati on, si nce the sense of taste as a whol e depends
on a mi xture of associ ati ons of taste and scent.
Disturbances of taste
Dysgeusi aunpl easant or al tered taste sensati on
Ageusi acompl ete l oss of taste
Hypogeusi areduced taste sensati on
P. 71
Fig. 6.9 Medi an gl ossi ti s.
True neurol ogi cal di sturbance of the sense of taste i s extremel y rare, but may occur i n some
general i zed abnormal i ti es of the central nervous system. A somewhat more common
(al though sti l l rare) neurol ogi cal di sturbance i s that due to surgi cal trauma to the chorda
tympani fol l owi ng operati ons on the mi ddl e ear. If the nerve i s di sturbed, ei ther duri ng
operati on or by postoperati ve oedema, an area of taste di sturbance may devel op on the si de
of the tongue correspondi ng to the termi nal di stri buti on of the chorda tympani wi th the
l i ngual nerve. In a f ew pati ents wi th Bel l ' s pal sy (Chapter 15) there i s a si mi l ar l oss of taste
sensati on due to i nvol vement of the chorda tympani on the af fected si de.
Medi cati on can af fect taste, and drugs commonl y i mpl i cated i ncl ude the anti rheumati c (for
exampl e, al l opuri nol and phenyl butazone) and anti metabol i te (for exampl e, methotrexate)
drugs. Peni ci l l ami ne and captopri l (an angi otensi n-converti ng enzyme (ACE) i nhi bi tor) have
been reported as causi ng taste di sorders. Metroni dazol e can al so cause an unpl easant taste.
Any drugs causi ng xerostomi a can al so i ndi rectl y affect taste percepti on.
The most common cause of a di sturbance of taste sensati on or of a f oul taste i n the mouth i s
the presence of a pyogeni c i nfecti on, most usual l y the resul t of peri odontal di sease, i nfecti on
i n the nose or si nuses, or a di scharge f rom a l esi on such as an i nfected dental cyst. An
i nfecti ve focus may be very di ff i cul t to detect, parti cul arl y when present i n a rel ati vel y
i naccessi bl e area such as a sal i vary gl and. The cl ini cal exami nati on of such a pati ent shoul d
al ways i ncl ude a careful search f or pus-contami nated sal i va f rom any of the major sal i vary
gl ands as wel l as radi ographi c assessment for the presence of possi bl e i ntrabony i nfected
l esi ons, si nusi ti s, and si mi l ar possi bl e i nfecti ve foci . Cl earl y, such pati ents are suf feri ng not
from a true di sturbance of taste sensati on, but f rom the chroni c i mposi ti on of unpl easantl y
tasti ng materi al i nto the oral cavi ty.
Tabl e 6. 6 l i sts some condi ti ons that may gi ve ri se to al terati ons or l oss of taste. Anosmi a
(absence of smel l ) can ei ther be transi ent due to upper respi ratory tract i nf ecti ons or
permanent as a resul t of teari ng of the ol factory nerve fol l owi ng maxi l l ofaci al or head
i njuri es and wi l l resul t i n some di mi nuti on of taste. The underl yi ng cause f or the al terati on i n
taste may be el i ci ted f rom the pati ent' s hi story and exami nati on of the oral cavi ty, but i t i s
advi sabl e to check for zi nc defi ci ency.
Pati ents wi th evi dence of crani al nerve def i ci ts or reporti ng neurol ogi cal symptoms shoul d be
sent for speci al i st eval uati on

and appropri ate i magi ng. When al l such exami nati ons have been perf ormed, there remai ns a
group of pati ents i n whom no abnormal i ty of any ki nd i s detected, but who conti nue to
compl ai n of an unpl easant taste. These pati ents are often obsessi onal , but i t i s very di ff i cul t
to determi ne whether thi s i s the cause or the consequence of thei r compl ai nt (see Chapter
17). Al terati ons i n taste can be one of the symptoms of oral dysaesthesi a. Some of the most
di f fi cul t pati ents to treat among those wi th di sturbances of taste sensati on are those who are
weari ng rel ati vel y recentl y acqui red dentures. Few taste buds are covered by the denture and
the ol f actory nerve endi ngs are i n no way affected but, nonethel ess, some such pati ents
compl ai n of an abnormal i ty or even compl ete l oss of taste sensati on. The expl anati ons for
thi s are not convi nci ng, but i t woul d seem that vari ati ons i n texture between the uncovered
pal ate and the denture may pl ay a part.
P. 72
Table 6.6 Conditions associated with alterations in taste
Dental conditions
Peri odontal di seases
Cari ous l esi ons
The denti st' s responsi bi l i ty i n such cases as those descri bed above i s to el i mi nate, by
detai l ed exami nati on, any of the l ocal causes. The pati ent may then need to be referred
appropri atel y to el i mi nate non-orodental causes. If thi s exami nati on i s negati ve and i f al l
concerned are sure that there i s no nasal or si mi l ar aeti ol ogy, then there i s l i ttl e poi nt i n
attempti ng treatment. Mouthwashes may occasi onal l y hel p but, i n general , the pati ent who i s
obsessi onal l y concerned wi th a persi stent taste i s compl etel y resi stant to l ocal treatment.
Halitosis
A cl osel y rel ated probl em i s that of hal i tosi sbad breath. Thi s may occur i n the heal thy, or
i n those wi th si gni f i cant oral or general i zed di sease. It i s of ten a transi ent probl em, but, i f
not, warrants i nvesti gati on. The causes of hal i tosi s may be di vi ded i nto l ocal (Tabl e 6. 7),
Di schargi ng dental si nus
Restorati ons wi th margi nal
def i ci enci es
Dry socket
Associ ated structures
Sal i vary gl ands
Sal i vary gl and hypof ucti on
Si al adeni ti s

Si nuses
Si nusi ti s
Lungs
Respi ratory di sease

Stomach
Gastro-oesophageal

refl ux di sorder
Systemic disease
Uraemi a
Neurol ogi cal di sorders (e.g. Bel l ' s pal sy, brai n tumours, damage to chorda tympani )
Anaemi a
Deficiencies
e. g. zi nc
Drugs
ACE i nhi bi tors, l i thi um sal ts, gol d, carbi mazol e, metroni dazol e, peni ci l l ami ne,
xerogeni c drugs
Smoking
Psychogenic
Oral dysaesthesi a
Psychosi s (del usi ons, hypochondri asi s)
systemi c (Tabl e 6. 8), and drug-i nduced (Tabl e 6. 9) causes. Pati ents who smoke or eat a
great deal of garl i c and hi ghl y spi ced f oods wi l l have a characteri sti c odour on thei r breath.
Local causes of hal i tosi s i ncl ude poor oral hygi ene, chroni c peri odontal or dental di sease, and
ear, nose, and throat (ENT) probl ems, such as si nusi ti s or chroni c tonsi l l i ti s (Tabl e 6. 7).
Systemi c di sease, as i n renal or hepati c fai l ure, respi ratory di sease, and uncontrol l ed
di abetes l eadi ng to ketosi s, i s a rare cause of hal i tosi s but shoul d be consi dered (Tabl e 6. 8).
Some drugs are reported as causi ng hal i tosi s (Tabl e 6. 9). The most common drug-i nduced
form of hal i tosi s i s probabl y that due to the l ack of oral hygi ene resul ti ng from i atrogeni c dry
mouth (di scussed i n Chapter 8).
The probl em of del usi onal hal i tosi s i s f urther di scussed i n Chapter 17.
Case 6.1 Discussion
The hi story i s suggesti ve of erythema mi grans (geographi c tongue). Exami nati on of the
tongue wi l l confi rm the di agnosi s and excl ude other pathol ogi cal condi ti ons.
The recent hi story of i ncreasi ng soreness suggests a possi bl e underl yi ng factor, such as
anaemi a, due to i ron defi ci encyparti cul arl y i n vi ew of her reported ti redness. Thi s gi rl
shoul d be asked f urther questi ons about her general heal th, i ncl udi ng any gastroi ntesti nal
symptoms and drug hi story. Si gns of i ron defi ci ency anaemi a i ncl ude pal l or of the mucous
membranes, angul ar chei l i ti s, aphthous stomati ti s, gl ossi ti s, oral candi dosi s, and koi l onychi a.
Table 6.7 Local causes of halitosis
Mouth
Poor oral hygi ene
Food packi ng and stagnati on
Chroni c peri odontal di sease
Acute necroti zi ng ul cerati ve gi ngi vi ti s
Dry socket
Peri coroni ti s
Chroni c dental sepsi s
Inf ecti ons
Mal i gnant tumours
Haemorrhage
Nose and pharynx
Pharyngi ti s
Tonsi l l i ti s
Si nusi ti s (postnasal dri p)
Mal i gnant tumours
Forei gn bodi es
Table 6.8 Systemic diseases causing halitosis
Bl ood tests shoul d be undertaken i ncl udi ng a f ul l bl ood count (FBC), serum B
12
, red cel l and
serum fol ate, and serum ferri ti n esti mati ons. It may al so be worthwhi l e to carry out a serum
zi nc assay. Iron defi ci ency anaemi a i n a femal e student wi th no

obvi ous gastroi ntesti nal di sease i s most l i kel y to be due to excess menstrual l oss and/or poor
di et. The geographi c tongue can be treated symptomati cal l y wi th an anal gesi c ri nse
(for exampl e, benzydami ne) and avoi dance of i rri tati ng foods. Any haemati ni c
def i ci ency shoul d be i nvesti gated and appropri atel y managed.
Case 6.2 Discussion
Enl argement of the tongue (macrogl ossi a) i s a wel l recogni zed feature of Down' s syndrome.
The tongue may become ul cerated as a resul t of occlusal trauma. An enl arged tongue and
constant dri bbl i ng may predi spose to the devel opment of angul ar chei l i ti s i n Down' s
syndrome, parti cul arl y i f there i s mouth breathi ng. Mi crobi ol ogi cal sampl i ng shoul d be carri ed
Lower respi ratory tract i nfecti ons
Di abeti c ketoaci dosi s
Ki dney fai l ure
Li ver fai l ure
Febri l e i l l ness
Table 6.9 Drugs that may produce halitosis
Paral dehyde
Isosorbi de di ni trate
Di sul fi ram
Al cohol
Cytotoxi c drugs (i ndi rectl y)
Xerogeni c drugs (i ndi rectl y)
P. 73
Q1 What advi ce woul d you gi ve to thi s gi rl ' s mother?
Q2 What other factors must be consi dered when provi di ng dental treatment for a pati ent
wi th Down' s syndrome?
out and an appropri ate anti mi crobi al agent appl i ed. Staphyl ococcus aureus i s commonl y
i sol ated from l i p fi ssures, whi ch may respond to topi cal fuci di n wi th the addi ti on of a l ow-
potency steroi d preparati on. Nasal carri age of S. aureus needs to be addressed, i f rei nfecti on
i s to be avoi ded. Persi stent l i p fi ssures of ten requi re surgi cal exci si on, al though cryosurgery
has al so been advocated. When consi deri ng el ecti ve surgi cal i nterventi on i n a pati ent wi th
Down' s syndrome, a ri sk assessment shoul d be undertaken i n rel ati on to anaesthesi a and
suscepti bi l i ty to i nf ecti ve endocardi ti s (see bel ow).
There are a number of factors that need to be considered when provi di ng dental treatment
for pati ents wi th Down' s syndrome. Congeni tal cardiac anomal i es are present i n about 40 per
cent of i nfants wi th Down' s syndrome but most can be successful l y corrected by surgery.
Anti bi oti c prophyl axi s may, therefore, be needed for dental procedures l i kel y to create a
bacteraemi a. Other potenti al probl ems for the dentist i ncl ude i ncreased suscepti bi l i ty to
i nfecti on and peri odontal di sease. There may be reduced cooperati on and understandi ng of
the need for oral hygi ene, but the majori ty of patients wi th Down' s syndrome can be
successful l y treated i n routi ne dental practi ce. The carri age rate of hepati ti s B i s reportedl y
hi gher i n pati ents who are i nsti tuti onal i zed.
Case 6.3 Discussion
The most l i kel y di agnosi s of thi s pati ent' s chroni c l i p condi ti on i s acti ni c chei l i ti s due to
sunl i ght damage, as a resul t of hi s outdoor occupati on. Sol ar UVB radi ati on i s most damagi ng
and responsi bl e for premal i gnant and agei ng damage. It i s i mportant to check the face,
scal p, and other exposed ski n for acti ni c keratoses, parti cul arl y i n fai r-hai red i ndi vi dual s.
Other condi ti ons to be consi dered i n the di f ferential di agnosi s are l i chen pl anus and l upus
erythematosus. An al l ergi c chei l i ti s i s al so possi bl e but l ess l i kel y unl ess i t i s superi mposed
on an al ready sun-damaged l i p. Bi opsy i s essenti al and management depends on the
hi stopathol ogy and degree of epi thel i al dyspl asi a.
Acti ni c chei l i ti s i s consi dered to be a premal i gnant condi ti on and can transform i nto
squamous cel l carci noma, whi ch can be qui te aggressi ve compared to others at thi s si te. In
cases where there i s si gni f i cant dyspl asi a but no frank carci noma, one f orm of treatment i s a
vermi l i onectomy, i n whi ch the vermi l i on border i s compl etel y exci sed and the l abi al mucosa
advanced to joi n the ski n. Laser abl ati on can al so produce good cosmeti c resul ts wi th fewer
postoperati ve compl i cati ons. Chemi cal exfol i ati on wi th 5-fl uorouraci l can be used but
someti mes causes qui te si gni fi cant di scomfort and inf l ammati on unti l heal i ng takes pl ace. Al l
pati ents wi th acti ni c chei l i ti s or evi dence of sun-damage el sewhere on the ski n must be gi ven
advi ce about sun avoi dance (i f possi bl e) and the use of sunscreens (l i p sal ves or creams)
wi th a hi gh sun protecti on f actor. Ol der men i n parti cul ar can be encouraged to wear a hat
outdoors, especi al l y i f they have thi nni ng hai r or are bal d. Pati ents di agnosed wi th acti ni c
chei l i ti s shoul d be fol l owed up on a regul ar basi s.
Project
1. There i s a wi de range of mouthwashes commerci al l y avai l abl e f or the management of
hal i tosi s (bad breath). What are the consti tuents of these preparati ons? Is there any
evi dence that they are effecti ve i n treati ng hal i tosi s?
Q2 What i s the di fferenti al di agnosi s of the l i p condi ti on?
> Tabl e of Cont ent s > 7 - Swel l ings of t he f ace and neck
7
Swellings of the face and neck
Problem case
Case 7.1
A 65-year-ol d man attends your surgery f or the provi si on of new dentures. The pati ent has
smoked (30 ci garettes per day) si nce he was a teenager. When questi oni ng thi s gentl eman
about hi s medi cal hi story you noti ce that he has a croaky voi ce. He reports a 2-
month hi story of i ncreasi ng hoarseness. On exami nati on you can pal pate a hard, fi xed,
nontender jugul o-omohyoi d l ymph node.
Facial swelling
Differential diagnosis
Al though dental i nf ecti ons are by far the most common cause of swel l i ngs about the face
seen by the dental surgeon, there are many other possi bi l i ti es that must be consi dered i n a
di f ferenti al di agnosi s. These i ncl ude f aci al swel l ings due to al l ergy or angi oedema (often
mi sdi agnosed as a purel y dental probl em) and the swel l i ngs i n oral Crohn' s di sease (orof aci al
granul omatosi s), whi ch are equal l y suscepti bl e to a mi staken i ni ti al di agnosi s. Si mi l arl y,
faci al swel l i ng due to i nf ecti ons of non-odontogeni c ori gi nsuch as that, f or i nstance, i n
acute si al adeni ti si s commonl y assumed to be of dental ori gi n. Masseteri c hypertrophy can
gi ve ri se to some di agnosti c probl ems i n pati ents presenti ng wi th faci al swel l i ng and i s
di scussed i n Chapter 16 (Temporomandi bul ar di sorders).
There are a l arge number of condi ti ons, both l ocal ized and general i zed, that can cause faci al
swel l i ng (Tabl e 7. 1). Some of these condi ti ons are consi dered el sewhere i n thi s book.
Readers are, however, advi sed to consul t textbooks of pathol ogy, medi ci ne, and surgery f or
detai l s of other condi ti ons.
Swellings in the neck
Cervi cal swel l i ngs general l y present ei ther at the si de or the mi ddl e of the neck and thei r
di f ferenti al di agnosi s i s outl i ned i n Tabl e 7. 2. Cervi cal l ymphadenopathy wi l l be di scussed i n
more detai l .
Q1 What other questi ons woul d you ask thi s gentl eman?
Table 7.1 A surgical sieve for the differential
diagnosis of facial swelling
Cervical lymphadenopathy
Differential diagnosis
Normal l ymph nodes are not pal pabl e. Enl argement of a node or a change i n texture, so that
i t may be pal pated through the ski n, i ndi cates that i t i s ei ther the pri mary si te of a
pathol ogi cal process (for exampl e, l ymphoma) or secondari l y i nvol ved (for exampl e, i n an
i nfecti ve or neopl asti c process el sewhere i n the body). Di ff erenti ati on between these two
si tuati ons i s i mportant. The

vast majori ty of cervi cal l ymph node enl argements are due to ei ther i nfecti on or neopl asi a
(Tabl e 7. 3). However, l ymph nodes can be enl arged in some connecti ve ti ssue di sorders (for
exampl e, sarcoi dosi s) and (rarel y) as a si de-eff ect of drug therapy (for exampl e, phenytoi n).
Congeni tal (e. g. l ymphangi oma, haemangi oma)
Inf ecti ous
Orodental i nfecti ons
Sal i vary gl and i nfecti ons (Chapter 8)
Cutaneous i nfecti ons
Neopl asti c
Sarcomas
Carci noma (Chapter 10)
Traumati c
Post-i njury
Postoperati ve
Surgi cal emphysema
Immunol ogi cal
Al l ergi c
Heredi tary (Chapter 14)
Endocri ne and metabol i c (Chapters 13 and 18)
Cushi ng' s syndrome
Acromegal y
Myxoedema
Nephroti c syndrome
Others, e. g. ;
Orofaci al granul omatosi s (Chapter 12)
Idi opathi c or drug-i nduced angi oedema (Chapter 14)
Corti costeroi d therapy (Chapter 3)
Masseteri c hypertrophy (Chapter 16)
P. 78
Table 7.2 The differential diagnosis of swellings in the
neck
Si de of neck
Cervi cal l ymphadenopathy
Sal i vary gl and enl argement (Chapter 8)
Acti nomycosi s
Examination of lymph nodes
Lymph nodes may be exami ned by pal pati on, ul trasound-gui ded fi ne needl e aspi rati on (USG-
FNA) and appropri ate i magi ng techni ques. Hi stopathol ogi cal exami nati on of an exci sed node
i s onl y i ndi cated i n speci fi c si tuati ons, such as occul t pri mary head and neck tumours and
l ymphoma.
When exami ni ng the l ymph nodes of the head and neck (Fi g. 7. 1), each of the mai n groups
must be pal pated i n turn, usi ng a systemati c approach. From a posi ti on behi nd the pati ent,
the pre-auri cul ar, paroti d, f aci al , submandi bul ar, submental , deep, cervi cal (upper, mi d, and
l ower), supracl avi cul ar, posteri or tri angl e, and occi pi tal groups of nodes are pal pated i n turn
on each si de. When exami ni ng the cervi cal nodes i t i s hel pful to rel ax the surroundi ng ti ssue
Caroti d body tumours
Branchi al cyst
Pharyngeal pouch
Cysti c hygroma
Mi ddl e of the neck
Thyroi d enl argement (mul ti nodul ar goi treneopl asia, ectopi c)
Thyrogl ossal cyst
Dermoi d cyst
Pl ungi ng ranul a
Ludwi g' s angi na
Table 7.3 Cervical lymphadenopathy: important infective
and neoplastic causes
Inf ecti ve causes
Lymphadeni ti s
Orodental
Antral
Tonsi l l ar
Aural
Nasopharyngeal
Scal p
Faci al
Tubercul osi s
Brucel l osi s
Inf ecti ous mononucl eosi s (gl andul ar fever)
HIV i nfecti on
Toxopl asmosi s (cat scratch f ever)
Syphi l i s
Neopl asti c causes
Lymphoma
Leukaemi a
Secondary neopl asi a
by bendi ng the pati ent' s head forward and l ateral l y towards the si de exami ned. If a pal pabl e
node i s found, i ts texture i s noted and i t i s moved between two fi ngers to di scover any
attachment to ski n or underl yi ng ti ssue.
In addi ti on to a detai l ed extra- and i ntraoral exami nati on, the exami ner shoul d check the
ski n of the scal p, face, and neck. Pl ai n radi ographs of the hard ti ssues may be requi red to
i denti fy any odontogeni c i nfl ammati on rel ated to the pati ent' s denti ti on. Secondary
neopl asms i n bone usual l y present as an i l l -def i ned radi ol ucency. If there are no foci of
i nfecti on or evi dence of mucosal l esi ons responsi ble for the cervi cal l ymphadenopathy, then
the pati ent shoul d be ref erred to a maxi l l of aci al (or ENT) surgeon for f urther exami nati on of
the ear, nose, and throat. Fl exi bl e nasoendoscopy or ri gi d oesophagol aryngoscopy may be
requi red, together wi th speci al i zed i magi ng techni ques such as MRI and ul trasonography (see
Chapter 2).
Inflammatory causes
Acute infections
Lymphadeni ti s ari si ng from an acute i nf ecti on such as a peri api cal abscess or a peri coroni ti s
i s usual l y uni l ateral . The appearance of the nodes i s rapi d, and they are soft and are pai nf ul
when touched. There may be oedema of the soft ti ssue surroundi ng the nodes gi vi ng the
vi sual i mpressi on of greater enl argement than i s, i n fact, the case. The faci al l ymph node,
l yi ng just anteri or to the anteri or border of masseter at the l evel of the occl usal pl ane, i s
commonl y i nvol ved i n chi l dren.
Chronic lymphadenitis
In chroni c i nfecti ons the af fected nodes are fi rm but may not be tender. In tubercul osi s the
i nvol ved nodes become attached to the ski n and thi s produces the so-cal l ed col l ar stud
abscess. In l ongstandi ng cases of chroni c i nfecti on, cal ci f i cati on of a node may present
as a sol i tary hard, nonfi xed swel l i ng. More wi despread smal l er cal ci fi cati ons i n the cervi cal
nodes are more common. They are usual l y di scovered i nci dental l y duri ng radi ography, are
asymptomati c, and cannot be pal pated. Wi despread l ymph node enl argement may be the fi rst
cl i ni cal si gn of i nfecti on wi th the HIV vi rusthe submandi bul ar nodes are often promi nentl y
aff ected (Chapter 4).

Neoplastic causes of lymph node enlargement
Lymphoma
The l ymph nodes of the neck are of ten i nvol ved early i n l ymphomas, both of the Hodgki n' s
and non-Hodgki n' s types. Affected nodes are i ni ti al l y di screte, rubbery i n consi stency, and
pai nl ess. Di agnosi s i s by hi stopathol ogi cal exami nati on. Burki tt' s l ymphoma more commonl y
ari ses as a central l esi on of the jaws, al though the cervi cal l ymph nodes may be af fected f i rst
i n a few pati ents.
P. 79
Leukaemia
The orof aci al mani f estati ons of l eukaemi a are f ul l y di scussed i n Chapter 13. Pati ents wi th an
underl yi ng di agnosi s of l eukaemi a rarel y present wi th a neck mass. It i s, however, i mportant
to appreci ate that dental i nf ecti ons and non-odontogeni c i nfecti ons commonl y occur i n
l eukaemi c pati ents and gi ve ri se to ei ther f aci al swel l i ng and/or cervi cal l ymphadenopathy.
Secondary neoplasia
Spread to l ymph nodes of the neck may occur at any stage i n the growth of a mal i gnant
neopl asm of the oral cavi ty, pharynx, antrum, or adjacent structures. The nodes are i ni ti al l y
pai nl ess, hard, and usual l y uni l ateral . The presence of pai nl ess enl arged l ymph nodes tends
to be more suggesti ve of a pathol ogi cal process other than i nfecti on. Troi si er' s si gn
i s the presence of a l ef t supracl avi cul ar node i n the presence of gastri c cancerso-cal l ed
because as a physi ci an i t was the fi rst si gn that caused hi m to suspect hi s own gastri c
carci noma.
Discussion of problem case
Case 7.1 Discussion
Persi stent hoarseness i s hi ghl y suggesti ve of mal i gnant di sease of the l arynx and must be
excl uded, as a matter of urgency. The hard, fi xed jugul o-omohyoi d l ymph node i s a
parti cul arl y worryi ng f eature and suggesti ve of metastati c spread. The pati ent shoul d be
speci f i cal l y asked about other symptoms, such as diff i cul ty swal l owi ng (dysphagi a) or
breathi ng (dyspnoea), dry cough, earache, haemoptysi s (coughi ng up bl ood), and wei ght
Fig. 7.1 Pri nci pal groups of l ymph nodes i n the head and neck.
Q1 What other questi ons woul d you ask thi s gentl eman?
l oss.
It i s i mportant to exami ne the mouth and oropharynx for obvi ous mucosal l esi ons. The
pati ent shoul d be i mmedi atel y ref erred for f urther i nvesti gati on by an ENT or head and neck
surgeon. A di rect ref erral can be faxed to the hospi tal uni t concerned or arranged by the
pati ent' s doctor, who shoul d al so be tel ephoned. It i s essenti al to check that your referral
has been recei ved as admi ni strati ve errors are al ways possi bl e.
Squamous cel l carci noma of the l arynx i s mul ti factori al but consumpti on of tobacco and
al cohol are recogni zed as ri sk factors. In the European Uni on, carci noma of the l arynx i s i n
the top 10

most common mal i gnanci es f or mal es. Other causes of persi stent hoarseness i ncl ude chroni c
l aryngi ti s due to i nfl ammatory condi ti ons or vocal fati gue (overuse), vocal cord paral ysi s,
and, l ess commonl y, syphi l i s, tubercul osi s, hypothyroi di sm, myaestheni a gravi s, and
functi onal dysphasi a. Beni gn tumours rarel y cause hoarseness.
Project
1. Magneti c resonance i magi ng (MRI) and ul tasonography are techni ques that can be used
to i nvesti gate sof t-ti ssue swel l i ngs i n the head and neck. What are the i ndi cati ons,
comparati ve advantages, and di sadvantages of these two techni ques?
P. 80
> Tabl e of Cont ent s > 8 - Sal ivary gl ands and sal i va
8
Salivary glands and saliva
Problem cases
Case 8.1
A 48-year-ol d mal e attends f or a 6-month revi ew appoi ntment at your practi ce. On updati ng
hi s medi cal hi story you l earn that he i s about to start a course of external beam radi otherapy
for tonsi l l ar carci noma.
Case 8.2
A 55-yearol d f emal e presents to your practi ce for routi ne revi ew. She reports xerostomi a. On
exami nati on you noti ce that she has a dry oral mucosa. New cari ous l esi ons, on the proxi mal
surfaces of previ ousl y sound l ower i nci sors, have devel oped si nce her l ast vi si t.
Case 8.3
A 45-year-ol d mal e pati ent presents to your practi ce and compl ai ns that over the past month
he has been experi enci ng pai n and swel l i ng under his tongue and l ower jawon the l eft
si de. The pai n and swel l i ng i s worse just before eati ng and i mproves consi derabl y after a
meal .
Saliva and the salivary glands
A number of condi ti ons aff ecti ng the sal i vary gl ands are managed surgi cal l y. These i ncl ude
cysts and neopl asms ari si ng i n the gl ands and al so condi ti ons ari si ng as a resul t of the
presence of cal cul i or other obstructi ons i n the glands or ducts. However, wi th the
el i mi nati on of these two groups of i mportant sal i vary gl and di seases there remai ns a number
of condi ti ons that shoul d be consi dered wi thi n the scope of oral medi ci ne. Thi s chapter wi l l
consi der si al adeni ti s, si al osi s, necroti zi ng si al ometapl asi a, and di sturbances i n sal i vary f l ow
Q1 What advi ce woul d you gi ve to thi s pati ent concerni ng hi s f uture oral heal th?
Q1 What questi ons woul d you ask thi s l ady?
Q2 What advi ce woul d you gi ve?
Q3 What i s the most frequent cause of xerostomi a?
Q1 What f urther questi ons woul d you ask thi s pati ent?
Q2 Cl i ni cal l y how woul d you exami ne thi s pati ent?
Q3 How woul d you manage hi m?
Q4 Is thi s pati ent l i kel y to compl ai n of a dry mouth?
rate. The cl i ni ci an shoul d be cogni zant wi th the functi ons of sal i va and the anatomy of the
sal i vary gl ands. Thi s i nformati on i s hel pf ul i n understandi ng the nature of the i nvesti gati ons
requi red to study sal i vary gl and di sease, thei r ensui ng sequel ae, and the therapi es avai l abl e.
Saliva
Sal i va i s a gl andul ar secreti on that i s essenti al for the mai ntenance of heal thy orodental
ti ssues. Sal i va i s a compl ex f l ui d and many of the f uncti ons of sal i va have a protecti ve rol e,
some of whi ch are shown i n Tabl e 8. 1. The physi cal properti es of sal i va vary accordi ng to the
di f ferent types of sal i vary gl ands, wi th paroti d secreti ons havi ng a serous (watery)
consi stency. The submandi bul ar and subl i ngual gl ands secrete a more vi scous sal i va due to
thei r hi gher gl ycoprotei n content. A severe reduction i n sal i vary fl ow rate can have
devastati ng consequences on oral heal th and, subsequentl y, on the psychol ogi cal and soci al
wel l -bei ng of the suf ferer.

Table 8.1 Functions of saliva
Function Description
Lubri cant Coats and protects the mucosa agai nst mechani cal , thermal ,
and chemi cal i rri tati on
Cl eanses the
teeth
Cl ears f ood f rom the oral cavi ty and oral mucosa
Ion reservoi r Faci l i tates remi neral i zati on of the teeth
Buf fer Neutral i zes pl aque pH af ter eati ng
Anti mi crobi al Secretory i mmunogl obul i ns, enzymes, and other sal i vary
protei ns hel p regul ate the oral fl ora
Pel l i cl e
formati on
A protecti ve l ayer of sal i vary protei n that f orms over enamel
acts as a di f fusi on barri er
Di gesti on Sal i vary amyl ase i ni ti ates the di gesti on of starch
Faci l i tates
taste
Sal i va i s a sol vent and theref ore al l ows the i nteracti on of
foodstuff wi th taste buds
Water bal ance Dehydrati on causes a reducti on i n sal i vary f l ow rate wi th an
associ ated oral dryness; thi s shoul d sti mul ate a need to
i ncrease fl ui d i ntake
P. 84
Sal i va i s i mportant for the mai ntenance of oral heal th.
A di mi nuti on of sal i vary f l ow can have a detri mental i mpact on the qual i ty of l i f e of a
pati ent.
The composi ti on of sal i va i s af fected by a number of factors, i ncl udi ng the type of sal i vary
gl and. The majori ty of amyl ase i s produced by the paroti d gl ands but the bl ood group
substances are secreted mai nl y by the mi nor mucous gl ands. The unsti mul ated f l ow rate i s
more i mportant than the sti mul ated fl ow rate f or oral comf ort. However, the sti mul ated fl ow
rate i s i mportant to faci l i tate chewi ng and swal l owi ng duri ng masti cati on. The submandi bul ar
gl and contri butes approxi matel y 65 per cent of the resti ng whol e sal i vary fl ow rate. Onl y
1520 per cent i s deri ved from the paroti d, wi th the subl i ngual and mi nor gl ands both
del i veri ng 78 per cent. In contrast, the paroti d provi des 4550 per cent of the
sti mul ated whol e sal i vary fl ow rate. Sal i vary fl ow rates exhi bi t di urnal vari ati on wi th an
average unsti mul ated f l ow rate throughout the day of approxi matel y 0.3 ml /mi nutei n
sl eep thi s may f al l to 0.1 ml /mi nute. Many textbooks menti on that approxi matel y 1500 ml of
sal i va i s produced each day. More recentl y, however, i t has been suggested that thi s fi gure i s
an overesti mati on of total dai l y sal i vary fl ow rate, and a dai l y fl ow rate of 500600 ml /day
may be a more real i sti c esti mate. The breakdown of sal i vary fl ow over a 24-hour peri od i s
shown i n Tabl e 8. 2.
The salivary glands
The paroti d gl ands are the l argest sal i vary gl ands. They are wedge-shaped and si tuated i n
front of the ear and behi nd the ramus of the mandi bl e. The apex of the wedge i s the deepest
part of the gl and. The peri pheral branches of the faci al nerve (CN VII) are i nti matel y
associ ated wi th the paroti d gl and. Thi s rel ati onshi p i s i nadvertentl y demonstrated when an
i nferi or dental nerve anaestheti c bl ock i s admi ni stered i ncorrectl y, and causes a temporary
droopi ng of the upper eye l i d.
Paroti d sal i va i s transferred al ong the paroti d duct i nto the oral cavi ty. The thi ck-wal l ed
paroti d duct (Stenson' s duct) emerges at the anteri or border of the paroti d gl and and runs
over the surface of the masseter bef ore hooki ng medi al l y over the anteri or muscl e border.
The ori fi ce of the duct i s covered by a smal l fl ap of mucosa cal l ed the paroti d papi l l a and thi s
i s si tuated opposi te the maxi l l ary second permanent mol ar.
The two submandi bul ar gl ands are approxi matel y hal f the si ze of the paroti ds. The superfi ci al
part of the submandi bul ar gl and i s wedged between the body of the mandi bl e and the
myl ohyoi d muscl e, wi th the smal l er deep part hooki ng around the posteri or border of the
muscl e to l i e on the fl oor of the mouthabove the myl ohyoi d. The submandi bul ar
(Wharton' s) duct runs forward, al ong the f l oor of the mouth to open i nto the subl i gual
papi l l a, just l ateral to the l i ngual frenum. The secreti ons are a mi xture of serous and mucous
fl ui ds.
The subl i ngual gl ands are the smal l est of the three pai rs of sal i vary gl ands and are l ocated
just bel ow the f l oor of the mouth beneath the subl ingual fol ds of mucous membrane. There
are numerous subl i ngual ducts that open i nto the mouth al ong the subl i ngual fol ds. The
secreti ons of these gl ands are predomi nantl y mucous.
Table 8.2 Salivary flow over a 24-hour period
Sl eep
40 ml sal i va wi l l be produced over 7 hours
The mi nor sal i vary gl ands consi st of numerous smal l mucosal gl ands si tuated on the tongue,
pal ate, buccal and l abi al mucosa. They produce pri mari l y a mucous secreti on.
Assessment of the salivary glands
Examination
The paroti d gl ands, l yi ng parti al l y conceal ed by the ascendi ng ramus of the mandi bl e, are not
parti cul arl y easy to pal pate. Tenderness and swel l ing are best detected by standi ng i n front
of the pati ent and by pl aci ng two or three fi ngers over the posteri or border of the ascendi ng
ramus of the mandi bl e. Backwards and i nwards movement of the fi ngers wi th l i ght pressure
i s al most al ways al l that i s needed to detect tenderness i n the superfi ci al part of the paroti d.
Thi s manoeuvre i s necessary to di fferenti ate parotid tenderness from that of the
temporomandi bul ar joi nt or the masseter wi th whi ch i t i s often conf used. It must al so be
remembered that the pai nf ul si gns resul ti ng from temporomandi bul ar joi nt/muscl e
dysfuncti on may extend to the upper pol e of the paroti d wi th whi ch the joi nt i s i n cl ose
anatomi cal rel ati onshi p. Swel l i ng of a paroti d gl and may al so be vi sual i zed by standi ng
behi nd the pati ent who i s seated i n a recl i ned dental chai r. When exami ni ng a paroti d gl and
the duct papi l l a must al so be exami ned i ntraoral l y for si gns of i nfl ammatory change. Paroti d
sal i va can be vi sual i zed by l i ghtl y compressi ng the ski n overl yi ng the duct wi th the fi ngers. If
the cheek i s hel d retracted, the sal i va expressed by thi s manoeuvre wi l l be seen coursi ng
downwards over the buccal mucosa from the duct papil l a. It i s hel pful for the cl i ni ci an to
know that paroti d sal i va can expressed, but thi s method i s not of val ue i n quanti tati vel y
assessi ng the paroti d f l ow rate of sal i va.
The submandi bul ar gl and may be fel t bel ow the angl e and body of the mandi bl e, thi s si mpl e
pal pati on bei ng rei nforced by bi manual pal pati on wi th a fi nger i n the fl oor of the mouth,
gentl e pressure bei ng exerted between the exami ni ng hand (bel ow the mandi bl e) and the
fi nger. As i n the case of the paroti d gl and, the submandi bul ar (Wharton' s) duct shoul d be
observed for si gns of i nfl ammati on and a subjecti ve assessment made of the qual i ty of the
sal i va. It i s si mi l arl y di ffi cul t to speci fy the normal pal pati on features of the
submandi bul ar gl and. In some enti rel y

normal i ndi vi dual s i t i s possi bl e to pal pate the gl and, whi l e i n others i t i s not.
Measurement of salivary flow
Sialometry
Sal i vary fl ow rate i s measured by si al ometry and ei ther resti ng (unsti mul ated/basal ) or
Awake
300 ml of unsti mul ated sal i va over 16 hours
200 ml of sti mul ated sal i va duri ng meal sover 54 mi nutes
P. 85
sti mul ated sal i va may be col l ected. Whol e sal i va, representi ng contri buti ons from al l the
sal i vary gl ands or secreti ons from i ndi vi dual major gl ands, can be measured. For exampl e, a
Carl sonCri ttenden col l ector (a smal l cup pl aced over the ori fi ce of the paroti d gl and duct)
can be used to col l ect sal i va f rom the paroti d gl and. The paroti d or submandi bul ar ducts can
al so be cannul ated to measure f l ow rates. These techni ques are rel ati vel y i nvasi ve and make
measurement of unsti mul ated fl ow rates unrel i abl e for i ndi vi dual gl ands. Sti mul ated whol e
sal i va can be col l ected and measured by a vari ety of vol umetri c and gravi metri c techni ques.
Si al ometry shoul d be measured under standardi zed condi ti ons. Consi derati on must be gi ven
to the ti me of day, the type of sti mul ant used, and the pre-col l ecti on i nstructi ons, for
exampl e, aski ng the pati ent to refrai n from smoki ng, dri nki ng, and eati ng f or a defi ned
peri od. Despi te the measurement of whol e sal i vary fl ow rates bei ng a rel ati vel y si mpl e and
non-i nvasi ve approach, i t i s not usual l y undertaken i n general dental practi ce. Si al ometry i s
probabl y most benef i ci al for the l ongi tudi nal assessment of fl ow rates f or i ndi vi dual pati ents
as there i s consi derabl e vari ati on wi thi n the populati on.
A wi de range of f l ow rates have been quoted for both basal and sti mul ated sal i va i n the
general popul ati on, but i t has been shown that changes i n sal i vary f l ow are probabl y more
i mportant i ndi cators of sal i vary functi on than a singl e fl ow rate measurement. Vol unteers
gi ven atropi ne reported that a dry mouth devel oped when thei r resti ng f l ow fel l to about 50
per cent of thei r normal fl ow rate. The normal rate of fl ow of unsti mul ated whol e sal i va i s
approxi matel y 0.3 ml /mi nute and for sti mul ated whol e sal i va 12 ml /mi nute. Sti mul ated
sal i va i s mai nl y secreted i n response to masti catory and gustatory sti mul i and the f l ow rate
wi l l ri se si gni fi cantl y (46 ml /mi nute) when chewi ng a powerful si al ogogue.
Salivary gland imaging
Plain radiography
Cal cul i can someti mes be seen on pl ai n f i l ms, but it shoul d be remembered that not al l
cal cul i are radi o-opaque. When i nvesti gati ng a suspected sal i vary cal cul us, two vi ews shoul d
be taken at 90. For the paroti d gl and, a panoramic or obl i que l ateral vi ew can be combi ned
wi th rotated anteri orposteri or or posteri oranteri or vi ew. For the submandi bul ar gl and,
panorami c and l ower occl usal vi ews (true and obl i que) are appropri ate. In the i nterest of
radi ati on dose reducti on, i t i s advi sabl e to take a secti onal panorami c fi l m where possi bl e for
these pati ents, l i mi ted just to the symptomati c si de.
Sialography
Si al ography i s an i magi ng techni que used to demonstrate the ductal system of the paroti d or
submandi bul ar gl and. A water-sol ubl e radi o-opaque contrast medi um i s i njected i nto the duct
ori fi ce and post contrast radi ographs are then taken i n two di fferent pl anes.
Fol l owi ng the removal of the cannul a, a f i nal drai nage fi l m i s usual l y taken to assess
the cl earance of the contrast medi um. Thi s gi ves some i dea of the gl and functi on and wi l l
of ten hi ghl i ght an obstructi on wi thi n the ductal system. Tradi ti onal l y, si al ography was carri ed
out usi ng an oi l -based contrast medi um, but thi s coul d cause probl ems i f extravasated
outsi de the ductal system and i t coul d al so damage the gl and i n pati ents wi th l i ttl e or no
sal i vary f l ow rate. Water-sol ubl e, preferabl y non-ioni c, contrast medi a are now used
routi nel y i n most centres.
Si al ography demonstrates a major sal i vary gl and i n three phases:
preoperati vel y
the fi l l i ng phase
the emptyi ng phase
Si al ography i s hel pful i n demonstrati ng structural abnormal i ti es of the duct system.
Stri ctures of the submandi bul ar gl and and duct di l atati ons are cl earl y seen. When there i s
atrophy of the sal i vary aci ni , an extravasati on of contrast medi um wi l l be evi dent i n the body
of the gl and (si al ectasi s). In Sjgren' s syndrome there i s someti mes a characteri sti c
snowstorm appearance (punctate si al ectasi s).
Si al ography shoul d not be undertaken i n the presence of acute i nf ecti on or when a cal cul us i s
known to be cl ose to the duct openi ng. There i s a ri sk that the contrast medi um may di spl ace
the cal cul us further i nto the duct.
Suspected mass l esi ons wi thi n the sal i vary gl ands shoul d not be i nvesti gated wi th
si al ography as such l esi ons cannot be seen di rectl y. Di spl acement of the ductal system,
gi vi ng the bal l i n hand appearance, may suggest the presence of such a l esi on, but
thi s pattern i s not al ways seen and may resul t i n lesi ons bei ng mi ssed. Pati ents wi th di screte
masses wi thi n the sal i vary gl ands shoul d be sent for ul trasound and possi bl y a magneti c
resonance i magi ng (MRI) scan.
Scintigraphy (radioisotope imaging)
Sal i vary gl ands, i n common wi th the thyroi d, have the abi l i ty to concentrate certai n
radi oi sotopes. In sal i vary sci nti scanni ng l abel l ed techneti um pertechnetate (
99
Tc
m
) i s
frequentl y used because i t has a shorter hal f-l i f e than i odi ne. An i ntravenous i njecti on of the
radi oi sotope i s fol l owed by scanni ng of the sal i vary gl ands at i nterval s of 30 seconds.
Sal i vary gl and functi on i s then assessed wi th computer-assi sted quanti tati ve programmes.
The rate of concentrati on of the i sotope i n the gl ands i s pl otted and, after 30 mi nutes,
sal i vary secretory acti vi ty i s sti mul ated by droppi ng ci tri c aci d sol uti on on the tongue. The
resul ti ng acti vi ty of the gl ands i s agai n fol l owed by scanni ng at i nterval s of

30 seconds. Ti meacti vi ty prof i l es of the gl ands are thereby produced and can bui l d up a
ful l pi cture of sal i vary secretory acti vi ty (Fi gs 8. 1 and 8. 2). Thi s procedure measures gl and
uptake of the radi oi sotope and gi ves an i ndi cati on of i ts cl earance. The cl i ni ci an can vi sual i ze
and compare the acti vi ty of al l the major sal i vary gl ands. Sci nti graphy i s theref ore benefi ci al
for compari ng the f uncti on of a di seased gl and (as i n a l ocal i zed chroni c si al adeni ti s) wi th the
remai ni ng heal thy gl ands or to detect a general i zed l oss of gl andul ar functi on (as seen i n
Sjgren' s syndrome). Sci nti graphy provi des no i nformati on about sal i vary gl and anatomy.
Sci nti graphy i s used to assess sal i vary gl and f uncti on i n sal i vary gl and di seases such as
Sjgren' s syndrome.
P. 86
Ultrasonography
Ul trasound may be used f or superf i ci al sof t-ti ssue swel l i ngs and the i ni ti al i nvesti gati on of
suspected mass l esi ons. Ul trasound i s parti cul arl y useful for di f ferenti ati ng between sol i d and
cysti c l esi ons but the sound waves are bl ocked by bone. Ul trasound may be used i n cases of
chroni c i nfecti on where si al ography i s contrai ndi cated and may i denti f y the presence of
si al ectasi s or of a cal cul us, though subtl e changes are di f fi cul t to see.
Magnetic resonance imaging
MRI i s used to i nvesti gate space-occupyi ng l esi ons such as sal i vary gl and tumours. However,
onl y l i mi ted i nformati on i s gi ven on adjacent hard ti ssues. Thi s techni que provi des good soft-
ti ssue detai l and l ocal i zati on of massesthe facial nerve i s usual l y i denti fi abl e. Imagi ng
techni ques are di scussed more f ul l y i n Chapter 2.
Fig. 8.1 Ti meacti vi ty curve of normal sal i vary gl and duri ng sci nti scanni ngsee text
for detai l s of the procedure. Part a of the curve represents the i ni ti al i sotope uptake
and part b the response to sti mul usthe wash-out eff ect. Part c shows conti nui ng sl ow
uptake whi ch reaches a maxi mum and then decl i nes.
Fig. 8.2 The computer screen duri ng a sci nti scan of a pati ent wi th poor uptake and
response to sti mul us of the l eft submandi bul ar gl and.
Table 8.3 Clinical conditions in which saliva may play a
diagnostic role
Monitoring Condition

Sialochemistry
The measurement of the bi ochemi cal consti tuents of sal i va has been undertaken for many
di seases of the sal i vary gl ands; however the resul ts are frequentl y mi sl eadi ng or di f fi cul t to
i nterpret. Si al ochemi stry i s not routi nel y used for di agnosti c purposes but i t remai ns a
potenti al l y useful research tool . It can al so be of val ue i n measuri ng drug, anti body, and
hormone l evel s. Tabl e 8. 3 i denti f i es some condi ti ons i n whi ch the use of sal i va can contri bute
to the di agnosi s.
Biopsy
Open bi opsy of a major sal i vary gl and can be hazardous and i s best avoi ded. Paroti d gl and
bi opsy carri es a ri sk of faci al scarri ng, damage to the faci al nerve, or the establ i shment of a
sal i vary f i stul a. It i s common practi ce to assess the hi stopathol ogy of the sal i vary gl ands by
an i ntraoral bi opsy of the mi nor gl ands, notabl y the l abi al gl ands. Fi ne needl e aspi rati on (see
Chapter 2) of the major sal i vary gl ands may be undertaken.
Labial gland biopsy
Thi s entai l s maki ng a smal l i nci si on i nsi de the l ower l i p, through normal mucosa, under l ocal
anaesthesi a and harvesti ng a number of smal l mucosal gl ands for hi stopathol ogi cal anal ysi s.
Thi s bi opsy i s parti cul arl y useful i n establ i shi ng a di agnosi s of Sj gren' s syndrome, and the
pathol ogi cal changes mi rror the focal l ymphocyti c si al adeni ti s that occurs i n the major gl ands
i n thi s condi ti on. The pati ent shoul d al ways be warned of the possi bi l i ty of l ocal i zed l i p
parasthesi a (or anaesthesi a) fol l owi ng bi opsy of the l abi al gl ands. Thi s i s a wel l recogni zed
postoperati ve compl i cati on.
Salivary gland disease
Sialadenitis
Si al adeni ti s i s the term used to descri be i nf l ammati on of sal i vary gl ands, most commonl y the
resul t of vi ral or bacteri al i nf ecti on, but occasi onal l y due to other causes (for exampl e,
al l ergi c reacti ons, i rradi ati on). Bacteri al si al adeni ti s i s usual l y a secondary consequence of
ei ther a l ocal i zed or systemi c cause of reduced sal i vary fl ow and i s rarel y the pri mary
pathol ogi cal process. Vi ral i nfecti ons, however, frequentl y aff ect previ ousl y normal sal i vary
gl ands. General l y speaki ng, si al adeni ti s i s a cl i nical probl em aff ecti ng the maj or sal i vary
gl ands. However, i t may al so occur i n mi nor sal i vary gl ands, ei ther as a pri mary phenomenon
(as i n Sj gren' s syndrome) or as a secondary feature of some other condi ti on such as pi pe-
Bl ood groups Usef ul i n forensi c medi ci ne
Drug l evel s Li thi um, methadone, di goxi n
Detecti on of drugs Al cohol , amphetami nes, benzodi azepi nes, opi oi ds
Hormones Corti sol , testosterone
Anti body detecti on HIV i nfecti on, measl es, mumps, rubel l a
P. 87
smoker' s pal ate, i n whi ch the pal atal sal i vary gl ands often become i nfl amed and enl arged
(see Chapter 9).
Of the vi ral i nfecti ons of the sal i vary gl ands by far the most common i s mumps, whi ch most
commonl y affects the paroti d gl ands but al so from ti me to ti me i nvol ves the other major
gl ands. The mumps vi rus (an RNA paramyxovi rus) i s transmi tted by di rect contact or by
dropl et i nfecti on, and a cl i ni cal i nfecti on i s heral ded by a feel i ng of mal ai se and of ten by
abdomi nal pai n. The sal i va of pati ents devel opi ng mumps i s i nf ecti ous for several days bef ore
paroti ti s devel ops and for approxi matel y 2 weeks after the presentati on of cl i ni cal symptoms.
Fol l owi ng thi s, there i s swel l i ng of one or both paroti d gl ands, someti mes associ ated wi th a
swel l i ng of the submandi bul ar gl ands. In many cases, one of the paroti d gl ands i s by f ar the
more severel y aff ected. Wi th the swel l i ng of the gl and there i s pai n and tenderness together
wi th a general mal ai se and a f ever. Pati ents often have tri smus. The swel l i ng of the sal i vary
gl and i s at i ts maxi mum wi thi n 1 or 2 days of the onset of symptoms and i s fol l owed by a
gradual resol uti on over 1 or 2 weeks. Mumps often occurs i n mi nor epi demi c form and i s
usual l y recogni zed on cl i ni cal symptoms al one. Rarel y, conf i rmati on may be obtai ned by
anti body measurements. Treatment of mumps i s enti rel y symptomati c, wi th the use of
anti pyreti cs, si mpl e anal gesi cs, ampl e fl ui d i ntake, and rest. Isol ati on i s i mportant si nce the
di sease i s very contagi ous. Mumps i s al so di scussed i n Chapter 4.
A wel l -establ i shed compl i cati on of mumps i s the onset of orchi ti s or oophori ti soccasi onal l y
steri l i ty may ensue. These condi ti ons are caused, not by di rect vi ral i nfecti on, but by the
producti on of autoanti bodi es di rected agai nst the ti ssues of the reproducti ve organs. After an
attack of mumps, i mmuni ty i s usual l y compl ete and, theref ore, a recurrent swel l i ng of the
paroti d regi on i s not l i kel y to be due to a recurrence of mumps. It shoul d be remembered,
however, that, rarel y, sal i vary gl and i nfecti ons may be caused by other vi ruses, i ncl udi ng
cytomegal ovi rus and Coxsacki evi ruses. The wi despread use of i mmuni zati on agai nst mumps
wi l l resul t i n a f al l i n i nci dence.
Si al adeni ti s can:
be chroni c or acute
aff ect the mi nor or major sal i vary gl ands
have a systemi c or l ocal aeti ol ogy
have an i nf ecti ve or non-i nfecti ve aeti ol ogy
A reducti on i n sal i vary gl and fl ow rate, ei ther f rom a l ocal i zed or systemi c cause, i s a
predi sposi ng f actor i n acute si al adeni ti s. As has been poi nted out, bacteri al i nf ecti on of the
sal i vary gl ands i s commonl y secondary to an obstructi ve condi ti on such as cal cul us f ormati on
or duct stri cture; i t i s therefore a l ocal i zed condi ti on. Thi s bei ng so, i t i s evi dent that a
si ngl e

gl and i s normal l y af fected. Most of ten thi s i s a paroti d gl and. The reduced sal i vary fl ow
predi sposes the gl and to an ascendi ng i nf ecti on from bacteri a wi thi n the oral cavi ty. In thi s
condi ti on the gl and i s pai nful , tender, and swol l en, and pai n i s radi ated to the ear and the
temporal area. Intraoral l y, the duct of the af fected gl and may be seen to be swol l en and
reddened, and the duct papi l l a enl arged. Purul ent sal i va may be mi l ked from the duct by
manual pressure. When pus can be expressed f rom the duct i t shoul d be sent f or cul ture and
sensi ti vi ty testi ng. In the absence of sensi ti vi ty tests, fl ucl oxaci l l i n i s the anti mi crobi al of
choi ce i n the pati ent who i s not sensi ti ve to peni ci l l i n. There wi l l often be an associ ated
l ymphadenopathy of the cervi cal nodes.
Sjgren' s syndrome i s a predi sposi ng factor for bacteri al i nfecti ons, both acute and chroni c.
In pati ents i n whom i l l heal th has l ed to l owered resi stance, ascendi ng i nf ecti on of any of the
P. 88
sal i vary gl ands may occur from the mouth vi a a sal i vary gl and duct. Thi s, once a common
sequel to gastroi ntesti nal surgery, especi al l y i n ol der pati ents, i s now not so often seen due
to i mproved peri operati ve care. In these cases the treatment i s evi dentl y nonsurgi cal , bei ng
dependent on anti bi oti c therapy, the mai ntenance of a correct fl ui d bal ance, and, i f possi bl e,
the resol uti on of the predi sposi ng condi ti on.
Treatment of an acute paroti ti s tends to be somewhat protracted. Bacteri al swabs shoul d be
taken and anti bi oti c sensi ti vi ti es determi ned whenever possi bl e. It i s cl ear, however, that the
use of anti bi oti cs shoul d be regarded onl y as f i rst-l i ne treatment. Any predi sposi ng factor
must be i denti fi ed. It i s possi bl e that surgi cal treatment may be necessary, such as removal
of a duct cal cul us or, occasi onal l y, the exci si on of a severel y damaged gl and.
Chroni c si al adeni ti s, ei ther of the paroti d or the submandi bul ar gl ands, may fol l ow the
resol uti on of an acute i nfecti on or may occur wi thout any evi dent pri mary acute phase. In
these ci rcumstances symptoms are rel ati vel y l ow-grade wi th tenderness and a mi nor degree
of swel l i ng of the affected gl and, and occasi onal l y wi th some degree of swel l i ng and redness
of the duct and the papi l l a. As i n acute si al adeni ti s, purul ent sal i va may be expressed f rom
the duct. Frequentl y i n these cases, mi nor di l atations of the ductal system of the gl ands may
be detected on si al ography and, presumabl y, these provi de foci of i nfecti on and stagnati on.
Such a recurrent chroni c si al adeni ti s may occur foll owi ng radi otherapy aff ecti ng the gl ands or
may f ol l ow the mi nor damage due to the presence of a cal cul us. The pri nci pl es of treatment
are exactl y the same as those of acute si al adeni ti s. In the pati ent wi th recurrent si al adeni ti s
of a submandi bul ar gl and, exci si on of the gl and mi ght be requi red. Tabl e 8. 4 summari zes the
mai n causes of si al adeni ti s.
Sialosis
Si al osi s (si al adenosi s) i mpl i es a pai nl ess, non-i nfl ammatory, non-neopl asti c swel l i ng of the
sal i vary gl ands. There are many preci pi tati ng factors f or thi s condi ti on, i ncl udi ng the effect
of anti rheumati c drugs, drugs contai ni ng i odi ne, and adrenergi c drugs. Si mi l ar experi mental
si al osi s may be sti mul ated i n ani mal model s by the admi ni strati on of i soprenal i ne. On the
whol e these drug-i nduced enl argements are reversi bl e, al though, i n some cases, acute
si al adeni ti s has been reported f ol l owi ng wi thdrawal of the drug. Si al osi s may al so occur i n
hormonal abnormal i ti es (di abetes mel l i tus, acromegal y) and i n nutri ti onal def i ci ency states,
i ncl udi ng anorexi a nervosa and chroni c al cohol i sm. The paroti d gl ands are most f requentl y
aff ected, and the swel l i ng i s commonl y bi l ateral . The mechani sm i nvol ved i s not understood,
but, hi stol ogi cal l y, there i s hypertrophy of serous aci ni . Investi gati on of pati ents wi th
possi bl e si al osi s shoul d i ncl ude the i denti fi cati on of predi sposi ng causes. Thus, l i ver functi on
tests and tests for bl ood gl ucose and growth hormone may be i ndi cated. A detai l ed drug
hi story shoul d be recorded and the possi bi l i ty of eati ng di sorders consi dered (Chapter 17).
Appropri ate i magi ng shoul d be undertaken. However, bi opsy i s rarel y i ndi cated.
Table 8.4 Causes of sialadenitis
Bacteri al
Ascendi ng si al adeni ti s
Recurrent paroti ti s of chi l dhood
Vi ral
Mumps
HIV
Cytomegal ovi rus
Sj gren' s syndrome
Necrotizing sialometaplasia
Thi s rel ati vel y uncommon, tumour-l i ke condi ti on occurs more frequentl y i n mal es,
especi al l y smokers, and i s of unknown aeti ol ogy. It appears to be the resul t of a vascul i ti c
phenomenon that occurs i n mi nor sal i vary gl ands, usual l y i n the pal ate. It i s possi bl e that
i schaemi a l eads to i nfarcti on of sal i vary ti ssue. The consequence i s rel ati vel y pai nl ess
ul cerati on of rapi d onset. The margi ns are often everted and may be i ndurated, resembl i ng a
carci noma. Anaesthesi a of the pal atal mucosa has been reported as an earl y i ndi cator of thi s
form of ul cerati on. Hi stol ogi cal l y, the squamous metapl asi a found i n the sal i vary ducts,
together wi th pseudoepi thel i omatous hyperpl asi a of the surroundi ng pal atal epi thel i um, may
gi ve an i ncorrect i mpressi on of mal i gnancy. Thi s i s a sel f-l i mi ti ng condi ti on that resol ves i n
about 8 weeks ti me wi thout anythi ng other than symptomati c treatment. Lesi ons of
necroti zi ng si al ometapl asi a are often exci sed for di agnosti c purposes. As yet, there i s no real
i nformati on about the i mmedi ate cause of thi s condi ti on or of i ts i mpl i cati ons i n rel ati on to
systemi c factors.
Sarcoidosis
Sarcoi dosi s i s a chroni c granul omatous di sorder that may rarel y present as pai nl ess,
persi stent enl argement of the major sal i vary

gl ands. There i s often an associ ated reducti on i n sal i vary fl ow and there may be an
accompanyi ng Sj gren' s-l i ke condi ti on. Sarcoi dosi s i s descri bed more ful l y i n
Chapter 12.
Irradi ati on
P. 89
Table 8.5 Causes of salivary gland swelling
Inf ecti ve Drug-associ ated
Bacteri al Al cohol
Ascendi ng si al adeni ti s Iodi ne compounds
HIV-associated salivary gland disease
Pati ents wi th HIV i nf ecti on can devel op sal i vary gland probl ems and xerostomi a. The sal i vary
gl and swel l i ng may be due to a Sj gren' s-l i ke condi ti on wi th l ymphocyti c i nfi ltrati on
and a dry mouth. However, there may be other pathology present i n the sal i vary gl and such
as Kaposi ' s sarcoma or a l ymphoma. It i s al so possibl e that sal i vary gl and swel l i ng may be a
consequence of other vi ral i nfecti ons such as cytomegal ovi rus or Epstei nBarr vi rus
(Chapter 4). Chroni c paroti ti s i n chi l dren i s hi ghl y suggesti ve of HIV i nfecti on.
Salivary gland tumours
Sal i vary gl and tumours compromi se about 3 per cent of al l tumours. The majori ty occur i n
the paroti d gl ands and onl y 10 per cent af fect the mi nor sal i vary gl ands. There i s a great
vari ety of sal i vary gl and tumours and i n 1991 the WHO proposed a cl assi fi cati on and
nomencl ature for these (see Tabl e 8. 6 for summary).
Recurrent paroti ti s of chi l dhood Thi ouraci l
Vi ral Sul fonami des
Mumps Phenothi azi nes
HIV paroti ti s Chl orhexi di ne
Inf l ammatory Endocri ne
Obstructi ve si al adeni ti s Acromegal y
Sj gren' s syndrome Di abetes
Sarcoi dosi s Metabol i c
Si al osi s Al cohol i c ci rrhosi s
Neopl asms Mal nutri ti on
Pl eomorphi c sal i vary adenoma Others
Adenol ymphoma Sarcoi dosi s
Mucoepi dermoi d carci noma
Aci ni c cel l carci noma
Adenoi d cysti c carci noma, etc.
Necroti zi ng si al ometapl asi a
The cl i ni cal presentati on, hi stopathol ogi cal f eatures, and management of these sal i vary gl and
tumours i s outsi de the scope of thi s book, and readers are advi sed to consul t other reference
sources (see the project at the end of chapter).
Tumours af fecti ng the mi nor sal i vary gl ands wi l l be bri efl y di scussed, as these present
i ntraoral l y. Si nce the greatest concentrati on of these gl ands i s i n the area of the juncti on of
the hard and sof t pal ates, thi s i s the regi on i n whi ch these neopl asms are most often seen.
About 20 per cent of mi nor sal i vary gl and tumours occur i n the upper l i p. The majori ty of
these l esi ons are pl eomorphi c adenomas but more aggressi ve l esi ons such as adenocysti c
carci nomas may occur.
Ten per cent of al l sal i vary gl and tumours af fect the mi nor sal i vary gl ands. The majori ty of
these are pl eomorphi c adenomas occurri ng i n the palate.
Clinical features
The growth of pl eomorphi c adenomas i s usual l y sl ow and pai nl ess and ul cerati on i s unusual
unl ess there has been some degree of trauma. The texture of such a tumour i s usual l y f i rm
and the overl yi ng mucosa may appear vi rtual l y normal (Fi g. 8. 3).
The outstandi ng characteri sti c of these growths i s thei r unpredi ctabi l i ty, both i n hi stol ogi cal
appearance and i n cl i ni cal behavi our. More aggressive growth may occur i n some tumours
wi th ul cerati on of the underl yi ng mucosa. These features must be consi dered as possi bl e
markers of a mal i gnant l esi on such as an adenocystic carci noma, whi ch may resul t i n death,
both by l ocal ti ssue i nvasi on and the producti on of di stant metastases. The onl y cl ue as to
the probabl e eventual behavi our of a sal i vary neopl asm i s f rom the hi stol ogi cal exami nati on
Table 8.6 Classification of salivary tumours*
Adenomas Carcinomas
Pl eomorphi c adenoma Mucoepi dermoi d carci noma
Warthi n' s tumour (adenol ymphoma) Aci ni c cel l carci noma
Basal cel l adenoma Adenoi d cysti c carci noma
Oncocytoma Carci noma ari si ng i n pl eomorphi c
adenoma
Canal i cul ar adenoma Pol ymorphous l ow-grade adenocarci noma
Ductal papi l l omas Other carci nomas
*Source: Soames, J. V. and Southam, J. C. (1988). Oral pathol ogy, 3rd edn. Oxf ord
Uni versi ty Press, Oxford.
of ti ssue, and thi s must form the basi s of the surgi cal treatment of the l esi on.

Sal i vary gl and tumours, because of thei r often sl ow and pai nl ess growth, are often very
decepti ve, and l ack of compl ai nt from the pati ent may produce a qui te unwarranted sense of
securi ty. Any suspected sal i vary tumour shoul d be ref erred at once for i nvesti gati on and
treatment wi thout any attempt at mi nor surgi cal i nterventi on, whi ch mi ght make l ater
def i ni ti ve treatment di ff i cul t. Most pl eomorphi c adenomas affecti ng the mi nor sal i vary gl ands
are exci sed wi th a margi n of surroundi ng normal ti ssue.
Any suspected sal i vary gl and tumour shoul d be referred f or i nvesti gati on and treatment
wi thout any attempt at mi nor surgi cal i nterventi on, whi ch mi ght make l ater defi ni ti ve
treatment di ffi cul t.
Disturbances of salivary flow
Xerostomia
Xerostomi a i s the subjecti ve feel i ng of oral dryness, whi ch may or may not be associ ated
wi th hypof uncti on of the sal i vary gl ands. A l ack of sal i va may be due to ei ther a l oss of
secretory ti ssue i n the sal i vary gl ands, or a di sturbance i n the secretory i nnervati on
mechani sm brought about by the acti on of xerogeni c drugs or, much l ess commonl y, by
neurol ogi cal di sease. A si gni fi cant proporti on of pati ents compl ai ni ng of xerostomi a are
found, after i nvesti gati ons, to have some systemi c factor responsi bl e for the reducti on i n
sal i vary f uncti on. These systemi c f actors may be associ ated wi th a wi de range of di sease
processes (such as renal and endocri ne di sturbances) but many of these pati ents suff er from
Sjgren' s syndrome; thi s wi l l be di scussed i n more detai l bel ow. The causes of xerostomi a
are summari zed i n Tabl e 8. 7.
Xerostomi a i s a symptom that shoul d be i nvesti gated because i t may be i ndi cati ve of
Therapeuti c radi ati on to the head and neck regi on, for the treatment of mal i gnancy, can al so
Fig. 8.3 A l arge pl eomorphi c adenoma of the pal ate.
P. 90
cause a marked di mi nuti on i n sal i vary fl ow and severe oral dryness. Pati ents treated by
whol e-body radi ati on (for exampl e, pri or to bone marrow transpl antati on for l eukaemi a) and
those gi ven radi oacti ve i odi ne (I
131
) for thyroi d cancer can al so suffer f rom xerostomi a. Earl y
di mi nuti on of sal i vary fl ow due to radi ati on may be due to damage to the bl ood suppl y of the
gl ands, but l ater eff ects are the resul t of destructi on of the gl and' s secretory apparatus.
Neurol ogi cal di sease, ei ther central or peri pheral , may be responsi bl e for a decrease i n the
secretomotor sti mul ati on of the sal i vary gl and and, hence, the dryness of the mouth.
However, the most common cause f or thi s i s the acti on of drugs wi th the si te of acti on bei ng
ei ther central or i n the autonomi c pathway. Groups of drugs i mpl i cated as havi ng thi s ki nd of
acti on i ncl ude anti hi stami nes, anti hypertensi ves, and sedati ves. However, the more common
ones to cause xerostomi a are the psychotropi c drugs and, i n parti cul ar, the anti depressants
and tranqui l l i zers. Over 400 drugs have been i denti fi ed as havi ng the potenti al to cause
varyi ng degrees of oral dryness, and the ef fects may be potenti ated i n pati ents taki ng
mul ti pl e xerogeni c drugs. Drug-rel ated oral dryness shoul d be a reversi bl e si de-eff ect, wi th
resol uti on occurri ng fol l owi ng cessati on of the drug.
Commonly used drugs that cause xerostomia
Anti depressants
Anti hi stami nes
Decongestants
Anti parki nsoni an agents
Table 8.7 Causes of xerostomia
Devel opmental
Apl asi a or atresi a
Sal i vary gl and di sease
Sj gren' s syndrome (pri mary, secondary)
Sarcoi dosi s
HIV i nfecti on
Iatrogeni c
Drug-i nduced
Therapeuti c i rradi ati on (e. g. external beam radi otherapy, total body i rradi ati on
Graf t-versus-host di sease
Psychogeni c
Oral dysaesthesi a
Burni ng mouth syndrome
Anxi ety/depressi on
Dehydrati on
Febri l e i l l ness
Di abetes mel l i tus
Di abetes i nsi pi dus
Renal fai l ure
Di arrhoea
Al cohol
May cause sal i vary gl and di sease, l i ver di sease, and dehydrati on
Local
Mouth-breathi ng
Tranqui l l i zers and hypnoti cs
Anti psychoti cs
Di ureti cs
Appeti te suppressants
Sal i vary hypofuncti on can al so be due to an underl yi ng cogni ti ve di sorder, such as depressi on
or chroni c anxi ety. The abi l i ty of acute anxi ety to cause a transi ent reducti on i n sal i vary fl ow
i s wel l known to students taki ng exami nati ons and those engaged i n publ i c speaki ng. Pati ents
wi th a sensory or cogni ti ve di sorder may al so have a percepti on of oral dryness, but objecti ve
measurements of sal i vary fl ow may be normal and the mouth apparentl y moi st. These
i ndi vi dual s frequentl y compl ai n of other symptoms, such as a bad taste and abnormal
sensati ons i n the

mouth. Xerostomi a i s frequentl y reported by pati ents wi th burni ng mouth syndrome.
There are confl i cti ng reports on the eff ect of age on sal i vary gl and functi on, but there i s
some evi dence that sti mul ated sal i vary fl ow rates are uni mpai red wi th age i n heal thy,
unmedi cated i ndi vi dual s. Unsti mul ated whol e sal i vary fl ow rates, however, have been shown
to decrease wi th age i n heal thy non-medi cated subjects. Age, i n addi ti on to drugs and
di sease, i s i mportant i n reduci ng the secreti on of resti ng whol e sal i va. Iatrogeni c causes and
systemi c di sease are ri sk factors for xerostomi a that are more l i kel y to be encountered i n the
mi ddl e-aged or el derl y popul ati on.
Investigation of xerostomia
Symptoms of a dry mouth i ncl ude thi rst, di f fi cul ty i n eati ng dry foods and swal l owi ng,
di f fi cul ty i n speaki ng and weari ng dentures, and the need to take f requent si ps of water whi l e
eati ng. Pati ents may al so compl ai n of a burni ng sensati on i n the mouth and abnormal taste or
hal i tosi sfrequentl y they report cracked l i ps or soreness at the corners of the mouth. The
questi ons l i sted i n Tabl e 8. 8 are f requentl y hel pf ul i n i denti f yi ng pati ents wi th oral
dysfuncti on associ ated wi th a reduced sal i vary fl ow. Orofaci al si gns and symptoms associ ated
wi th sal i vary gl and hypofuncti on are gi ven i n Tabl e 8. 9.
Cl i ni cal si gns associ ated wi th sal i vary gl and hypofuncti on i ncl ude dryness of the oral mucosa
(whi ch of ten appears l i ke parchment), fi ssuri ng and l obul ati on of the tongue (Fi g. 8. 4), and
evi dence of oral candi dosi s, parti cul arl y i n the form of angul ar chei l i ti s. The sal i va may
appear stri ngy and thi ck and tends to accumul ate as smal l beads on the mucosa. There may
be di ffi cul ty i n mi l ki ng sal i va from the major ducts and there i s often dental cari es at
si tes not usual l y suscepti bl e to decay. The sal i vary gl ands may al so be swol l en, ei ther due to
chroni c i nfecti on or i nvol vement i n an autoi mmune si al adeni ti s (Fi gs 8. 5 and 8. 6).
P. 91
Table 8.8 Questionnaire to identify patients with salivary
gland hypofunction
1. Does the amount of sal i va i n your mouth feel too l i ttl e, too much or do you
not noti ce i t?
2. Does your mouth feel dry when you eat a meal ?
3. Do you frequentl y si p l i qui ds when you eat a meal?
4. Do you have di ffi cul ti es swal l owi ng any foods?
Table 8.9 Signs and symptoms suggestive of salivary
gland hypofunction
Symptoms reported
Oral dryness
Burni ng, ti ngl i ng sensati on of tongue
The need for frequent dri nks to be taken whi l st eati ng or tal ki ng
Di ff i cul ty i n chewi ng and swal l owi ng dry foods
Al tered taste (dysguesi a) and smel l
Recurrent sal i vary gl and swel l i ngs/i nf ecti ons
Increase i n rate of dental decay
Dry, sore, cracked l i ps and angl es of mouth
Di ff i cul ty i n tal ki ng (dysphoni a)
General i zed mucosal soreness and ul cerati on of denture-beari ng areas
Oral exami nati on reveal s
Swol l en sal i vary gl ands
Absence of sal i vary fi l m over oral mucosa
Dry, paper-thi n parchment appearance of oral mucosa or appearance of
smal l amounts of frothy sal i va i n an otherwi se dry mouth
Fi ssuri ng and l obul ati on of the tongue
Dry, cracked l i ps, angul ar chei l i ti s
Evi dence of chroni c oral candi dosi s
Devel opment of new cari ous l esi ons, especi al l y on i nci sal or cuspal surf aces
Sal i vary gl and hypof uncti on i ncreases a pati ent' s suscepti bi l i ty to:
cari es
candi dosi s, i ncl udi ng angul ar chei l i ti s
oral mucosi ti s
Management of xerostomia and salivary hypofunction
Management depends on the underl yi ng cause of the xerostomi a and the degree of sal i vary
gl and i mpai rment. Pati ents who have detectabl e sal ivary functi on can be encouraged to use
some f orm of l ocal sti mul ati on, f or exampl e, chewi ng sugar-free gum or sugar -free pasti l l es.
The anti bacteri al properti es possessed by xyl i tol , whi ch i s often used as a sweetener i n
sugar-free gums, may have a si gni f i cant addi ti onal anti -cari es ef fect. Dentate pati ents must
be di scouraged f rom usi ng sugar-contai ni ng or aci di c sweets as these can exacerbate the
tendency to demi neral i zati on of the teeth and cause dental cari es. Systemi c medi cati on to
sti mul ate sal i vary fl ow has been assessed i n a number of

cl i ni cal tri al s and, to date, pi l ocarpi ne seems to have been the most eff ecti ve drug tested.
However, such sti mul ati ng therapy i s of l i ttl e use i f the xerostomi a i s due to near total l oss
of secretory uni ts i n the sal i vary gl ands. Pi l ocarpi ne al so has di sadvantages: i t has a number
of i nteracti ons wi th other drugs and may have adverse eff ects on the cardi ovascul ar system.
Mi nor si de-eff ects such as sweati ng and the urge to uri nate have been reported by pati ents
taki ng systemi c pi l ocarpi ne and are due to i ts choli nergi c agoni st acti vi ty.
Fig. 8.4 A dry and somewhat l obul ated tongue associ ated wi th xerostomi a.
P. 92
Fig. 8.5 Paroti d swel l i ng i n a pati ent wi th autoi mmune si al adeni ti s.
Symptomatic relief of dry mouth
Intri nsi c (i ncrease gl and acti vi ty)
sugar-free gum,
pi l ocarpi ne
Extri nsi c
sal i va substi tutes
The use of sal i va substi tutes may be of some hel p to pati ents compl ai ni ng of a dry mouth
and can be of fered to provi de symptomati c rel i ef for pati ents who have i nsuffi ci ent sal i vary
functi on to benefi t from sti mul ati on. A l arge number of di fferent arti fi ci al sal i vas are
now commerci al l y produced, and most are based on carboxymethyl cel l ul ose or muci ns. If
porci ne muci n preparati ons are preferred by the cl ini ci an, care must be taken not to
prescri be them to vegetari ans and vari ous rel i gi ous groups. A number of preparati ons contai n
fl uori de and thi s coul d be benefi ci al as a protective measure agai nst cari es. Cl i ni cal tri al s
have i ndi cated that arti fi ci al sal i vas can be useful for the management of xerostomi a but
pati ents f requentl y di sconti nue thei r use. Ei ther they do not l i ke the taste or consi stency or
fi nd that frequent appl i cati on i s requi red, maki ng use i nconveni ent and expensi ve. Many
pati ents pref er to take frequent si ps of water from a smal l contai ner carri ed around wi th
them. The use of sugar-free pasti l l es may someti mes be more hel pful than sprays i n a soci al
setti ng. Cracki ng and dryi ng of the l i ps can be control l ed by a petrol eum-based oi ntment
such as Vasel i ne, and angul ar chei l i ti s i s treated accordi ng to the mi cro-organi sms cul tured.
Oral candi dosi s can usual l y be control l ed wi th topical anti fungal agents. Dentate pati ents
requi re regul ar dental i nspecti ons, whi ch may need to be at i nterval s of 3 months or l ess.
Advi ce shoul d be gi ven about mai ntenance of oral hygi ene, avoi dance of sugary f oods, and
use of f l uori de suppl ements. The use of astri ngent mouthwashes, especi al l y those that
contai n al cohol , are best avoi ded. Fl uori de-contai ni ng gel s may be tol erated more
successful l y by pati ents wi th sal i vary gl and hypofuncti on. They often l ast l onger and thi s may
Fig. 8.6 Pus expressed from the paroti d ductthe resul t of i nfecti on secondary to
autoi mmune si al adeni ti s as part of Sjgren' s syndrome.
render them more cost-eff ecti ve. It i s of ten di ff i cul t f or pati ents to al ter thei r di et whi ch
tends to be severel y restri cted to bl and, sof t foodstuff s. Many f oods are too astri ngent and
hard foods can traumati ze the fragi l e oral mucosa. Neverthel ess, i ndi vi dual di etary anal ysi s
i s advi sabl e for pati ents who have a hi gh cari es i nci dence.

Chewi ng sugar-free gum:
i ncreases the f l ow of sti mul ated sal i va to l evel s about 3 to 10 ti mes resti ng val ues
can hel p prevent cari es: sti mul ated sal i va has enhanced buff eri ng capaci ty and a
greater remi neral i zi ng potenti al than resti ng sal i va
wi l l i ncrease the resti ng sal i vary f l ow rate for up to 30 mi nutes beyond the peri od of
chewi ng
has an anti mi crobi al acti vi ty i f i t contai ns xyl i tol
may be probl emati c i n pati ents wi th hi atus herni a or gastri c and i ntesti nal ul cerati ons
Pati ents who compl ai n of xerostomi a, but have no cli ni cal or objecti ve evi dence of a dry
mouth, often have other oral symptoms suggesti ve of oral dysaesthesi a and many compl ai n
of a burni ng sensati on i n the mouth. These pati ents can best be managed by counsel l i ng,
i ncl udi ng psychotherapy i f appropri ate. Psychotropic medi cati on, parti cul arl y the use of
tri cycl i c medi cati on, i s best avoi ded as i t may exacerbate xerostomi a. Thi s subject i s further
di scussed i n Chapter 17.
Sjgren's syndrome
Sjgren' s syndrome (SS) i s an autoi mmune di sease of the exocri ne gl ands that parti cul arl y
i nvol ves the sal i vary and l acri mal gl ands. Tradi ti onal l y, the symptoms of Sjgren' s
syndrome were thought to resul t f rom the destructi on of sal i vary and l acri mal gl and ti ssue.
More recentl y, i t has become cl ear that thi s i s not the case and that many Sj gren' s
syndrome suf ferers have substanti al reserves of hi stol ogi cal l y normal aci nar ti ssue that
si mpl y does not functi on properl y. Re-eval uati on of Sjgren' s syndrome i n thi s l i ght gi ves a
much more opti mi sti c outl ook f or treatment devel opment. Whereas there can be no
possi bi l i ty of recovery i f the aci nar cel l s have been destroyed, reversal of aci nar cel l
hypofuncti on may be attai nabl e i f the cause of the hypof uncti on can be determi ned.
Pri mary Sjgren' s syndrome consi sts of dry eyes (xerophthal mi a) and dry mouth and
i s not associ ated wi th a connecti ve ti ssue di sease
Secondary Sjgren' s syndrome consi sts of dry eyes and dry mouth and i s associ ated
wi th a connecti ve ti ssue di sease, most commonl y rheumatoi d arthri ti s
Systemi c l upus erythematosus, systemi c scl erosi s, pri mary bi l i ary ci rrhosi s, and mi xed
connecti ve ti ssue di sease may al so be associ ated wi th secondary Sj gren' s syndrome
Thi s process may occur as an i sol ated phenomenon, in whi ch case i t i s termed pri mary
Sjgren' s syndrome (1SS), or i n conjuncti on wi th a connecti ve ti ssue or col l agen di sease,
i n whi ch case i t i s ref erred to as secondary Sjgren' s syndrome (2SS). The preval ence of
thi s syndrome i s unknown and i t i s thought that many cases remai n undi agnosed. Recent
data has suggested that the esti mated preval ence of SS i s between 13 per cent of the UK
popul ati on. Mi ddl e-aged and el derl y women are mai nl y aff ected and present wi th a compl ai nt
of dry mouth (xerostomi a) and dry eyes (xerophthal mi a), wi th or wi thout evi dence of a
connecti ve ti ssue di sease. In pati ents wi th secondary SS, rheumatoi d arthri ti s i s the most
P. 93
commonl y associ ated condi ti on. Other associ ated di seases i ncl ude systemi c l upus
erythematosus, pri mary bi l i ary ci rrhosi s, and systemi c scl erosi s. In common wi th many
autoi mmune di seases SS shows a sexual di morphi sm with a femal e to mal e rati o of
approxi matel y 9 to 1. Xerostomi a i s often severe i n pri mary SS, but some studi es suggest
that i t i s l ess marked i n secondary SS. A hi story of sal i vary gl and swel l i ng i s common and i s
ei ther due to i nfi l trati on of the gl and by l ymphoepi thel i al ti ssue or to recurrent i nfecti ons
(Fi gs 8. 5 and 8. 6). Swel l i ngs due to the repl acement of sal i vary gl and ti ssue do not tend to
be pai nf ul . Sal i vary gl and swel l i ngs of an i nfecti ve nature are pai nful , and there i s an
associ ated l ocal or systemi c ri se i n temperature and pus may be expressed from the duct
ori fi ce (Fi g. 8. 6). Xerophthal mi a (dry eyes) i s the most common compl ai nt but many
suff erers al so compl ai n of i tchi ng or gri tti ness i n the eyes or gi ve a hi story
of recurrent eye i nfecti ons. Thi s can gi ve ri se to keratoconjunti vi ti s si cca. Untreated, thi s can
eventual l y l ead to corneal damage and l oss of si ght. Pati ents wi th SS may al so compl ai n of a
dry ski n or vagi nal dryness.
The earl y detecti on of Sj gren' s syndrome can prevent seri ous ocul ar di sease.
The assessment and management of pati ents wi th sal ivary gl and enl argement conti nues to be
a probl em i n the Sjgren' s pati ents. Persi stent gl andul ar swel l i ng i n these i ndi vi dual s i s a
worryi ng feature as they have an i ncreased ri sk of devel opi ng non-Hodgki n' s l ymphomas.
These are often l ow-grade B-cel l l ymphomas and appear to be si mi l ar to l ymphomas that
devel op i n other mucosa-associ ated l ymphoi d ti ssue (MALT). Low-grade B-cel l l ymphomas of
other MALT si tes (MALTomas) i ncl ude those of stomach. thyroi d, and l ung. Sal i vary gl and
i magi ng techni ques, i ncl udi ng CT and MR, can provi de i mportant di agnosti c i nformati on but
bi opsy i s i ndi cated i n some cases. Hi stopathol ogy of enl arged sal i vary gl ands (parti cul arl y
paroti ds) i n SS usual l y reveal s a beni gn l ymphoepi thel i al l esi on, but thi s i s not al ways the
case. Major gl and bi opsy i s, however, not i ndi cated for routi ne di agnosti c eval uati on of SS.
Cl i ni cal si gns, such as a rapi d and progressi ve uni l ateral or asymmetri cal gl and enl argement
or/and a change i n the consi stency of the gl and f rom a sof t swel l i ng to a hard or nodul ar
one, shoul d prompt major gl and bi opsy. Open procedures gi ve more usef ul i nf ormati on and
better ti ssue sampl i ng than a needl e bi opsy. There are f ew l aboratory markers that wi l l
i denti fy those pati ents at ri sk from mal i gnant l ymphoma, al though a f al l i n serum
i mmunogl obul i ns shoul d prompt further i nvesti gati ons.

SDiagnosis of Sjgren's syndrome
Pati ents shoul d be questi oned about the oral symptoms as outl i ned i n the secti on above, but
shoul d al so be asked about other symptoms (Tabl e 8.10) and dryness el sewhere i n the body.
there i s a general i zed exocri ne hypof uncti on. Measurement of sal i vary gl and fl ow shoul d be
determi ned by si al ometry, and gl andul ar functi on i s further assessed by sci nti scanni ng wi th
l abel l ed pertechnetate. Si al ography, usi ng a water-based dye, may be i ndi cated where there
i s a hi story or cl i ni cal si gns i ndi cati ng possi bl e structural damage of the sal i vary gl ands. Thi s
may occur as a resul t of secondary chroni c i nf ecti on (Fi g. 8. 6). Persi stent swel l i ng of the
gl ands can be i nvesti gated by si al ography, but modern techni ques, parti cul arl y MRI, gi ve a
much better pi cture of soft-ti ssue l esi ons and are essenti al i f a tumour i s suspected. Great
emphasi s has been pl aced on the di agnosti c i mportance of a l abi al gl and bi opsy for pati ents
wi th suspected Sjgren' s syndrome. The number of l ymphocyti c f oci wi thi n the gl ands i s
then measured and graded accordi ng to the scheme proposed by Chi shol m and Mason. There
i s i ncreasi ng evi dence, however, that a number of pati ents may have a negati ve l abi al gl and
bi opsy resul t whi l e, on other cri teri a, bei ng di agnosed as suf feri ng from SS.
A probl em i n the defi ni ti ve di agnosi s of SS has been the vari abl e cri teri a used i n di f ferent
centres and the fact that some of these have been undul y narrow, i n the sense that they
have hi gh speci fi ci ty but l ow sensi ti vi ty. In addi ti on, many rel i ed on l i p bi opsy for a defi ni te
di agnosi s of SS and, as a consequence, the condi ti on may have been underdi agnosed i n the
P. 94
past. Some pati ents are not prepared to undergo l abi al gl and bi opsy, whi ch they percei ve as
a rel ati vel y unpl easant procedure, al bei t mi nor surgery. A bi opsy posi ti ve conf i rmati on of SS
rarel y al ters the management of the pati ent and, as a consequence, many are prepared to
accept a provi si onal di agnosi s of SS. Currentl y, however, l abi al gl and bi opsy i s the procedure
wi th the greatest speci fi ci ty and di agnosti c val ue for the sal i vary component of SS. An
Ameri canEuropean SS consensus group has now revi sed the cri teri a f or the cl assi f i cati on
of thi s syndrome, i ncl udi ng the concept of bi opsy-negati ve SS (provi di ng that certai n
autoanti bodi es are present; see Fi gure 8. 7). The cri teri a for the cl assi f i cati on and di agnosi s
of SS may, however, undergo f urther modi fi cati ons in future. Autoanti bodi es are usual l y
present i n SS, and a routi ne i mmune profi l e for rheumatoi d and anti nucl ear factors, as wel l
as anti -SS-A and anti -SS-B anti bodi es shoul d be arranged, together wi th measurement of
serum i mmunogl obul i ns. Sal i vary duct autoanti bodi es may al so be present i n SS, but thei r
si gni f i cance remai ns uncl ear; they are not i ncl uded i n the di agnosti c cri teri a of SS. A ful l
haematol ogi cal and bi ochemi cal screen shoul d al so be undertaken to el i mi nate systemi c
di seases, such as di abetes. Pati ents wi th eye symptoms must be exami ned by an
ophthal mol ogi st. A prel i mi nary esti mate of xerophthal mi a can be made by i nserti ng a smal l
stri p of absorbent paper i nsi de the l ower eyel i d (Schi rmer test) to assess the vol ume of tears
present (Fi g. 8.8). Indi vi dual s who have cl i ni cal evi dence or symptoms suggesti ve of a
connecti ve ti ssue di sorder shoul d be assessed by a rheumatol ogi st. Oral symptoms are
managed as for other cases of xerostomi a, but many pati ents wi th Sj gren' s syndrome have
l i ttl e or no sal i vary functi on and can onl y be gi ven symptomati c rel i ef i n the f orm of sal i vary
substi tutes. Recurrent i nfecti ons, parti cul arl y of the paroti d gl ands, can be parti cul arl y
troubl esome and may resul t ul ti matel y i n surgi cal removal , wi th the attendant ri sks to the
faci al nerve. If there i s any cl i ni cal suspi ci on of a tumour devel opi ng i n any gl and, then
i magi ng and bi opsy are mandatory. Cal cul us formati on i s often troubl esome as a l ong-term
resul t of chroni c i nfecti on and stasi s i n the major sal i vary gl ands. A summary of the
management of a pati ent wi th Sjgren' s syndrome i s gi ven i n Tabl e 8. 12.
Table 8.10 Extra-orofacial signs and symptoms
associated with Sjgren's syndrome
Location of
symptom
Description
Ocul ar Persi stent, troubl esome dry eyes, recurrent sensati on of
sand or gravel i n the eyes, need to use tear substitutes dai l y
Respi ratory tract Dryness of upper and l ower respi ratory tract, dysphoni a,
di sturbances to sense of smel l
Vagi nal Vagi nal dryness, burni ng, hi story of recurrent fungal
i nfecti ons, pai nful i ntercourse
Ski n Dry ski n, butterfl y rash, vascul i ti s
Gastroi ntesti nal
tract
Dysphagi a, consti pati on
There i s, to date, no ef fecti ve cure for Sjgren' s syndrome, but symptoms can usual l y be
control l ed to some extent by the measures outl i ned above. Pati ents are often rel i eved at
obtai ni ng a defi ni ti ve di agnosi s and may benefi t from membershi p of a Sj gren' s syndrome
support group.
General Fati gue, weakness, depressi on
Sl eep di sturbance, l oss of sexual desi re, depressi on
Fig. 8.7 The Schi rmer test to assess the acti vi ty of the l acri mal gl ands.
Table 8.11 Revised international classification criteria
for Sjgren's syndrome*
I. Ocul ar symptoms: a posi ti ve response to at l east one of the fol l owi ng
questi ons:
Have you had dai l y, persi stent, troubl esome dry eyes for more than 3
months?
Do you have a recurrent sensati on of sand or gravel i n the eyes?
Do you use tear substi tutes more than 3 ti mes a day?
II. Oral symptoms: a posi ti ve response to at l east one of the f ol l owi ng questi ons:
Have you had a dai l y feel i ng of dry mouth for more than 3 months?
Have you had recurrentl y or persi stentl y swol l en sal i vary gl ands as an adul t?
Do you frequentl y dri nk l i qui ds to ai d i n swal l owi ng dry f ood?
III. Ocul ar si gns, i . e. objecti ve evi dence of ocul ar i nvol vement defi ned as a
posi ti ve resul t f or at l east one of the fol l owi ng two tests:
Schi rmer' s I test, performed wi thout anaesthesi a (< 5 mm i n 5 mi nutes)
Rose bengal score or other ocul ar dye score (> 4 accordi ng to van
Bi jstervel d' s scori ng system)
IV. Hi stopathol ogy: i n mi nor sal i vary gl ands (obtai ned through normal -appeari ng
mucosa)
Focal l ymphocyti c si al adeni ti s, eval uated by an expert hi stopathol ogi st, wi th a
focus score > 1, defi ned as a number of l ymphocyti c f oci (whi ch are adjacent
to normal -appeari ng mucous aci ni and contai n more than 50 l ymphocytes) per
4 mm
2
of gl andul ar ti ssue

V. Sal i vary gl and i nvol vement: objecti ve evi dence of sal i vary gl and i nvol vement
def i ned by a posi ti ve resul t for at l east one of the fol l owi ng di agnosti c tests:
Unsti mul ated whol e sal i vary f l ow (< 1. 5 ml i n 15 mi nutes)
Paroti d si al ography showi ng the presence of di f fuse si al ectasi as (punctuate,
cavi tary, or destructi ve pattern) wi thout evi dence of obstructi on i n the major
ducts
Sal i vary sci nti graphy showi ng del ayed uptake, reduced concentrati on, and/or
del ayed excreti on of tracer
VI. Autoanti bodi es: presence i n the serum of the fol l owi ng autoanti bodi es:
Anti bodi es to Ro(SSA) or La(SSB) anti gens, or both

For the di agnosi s of pri mary SS:
In pati ents wi thout any potenti al l y associ ated di sease, pri mary SS may be defi ned
as fol l ows:
The presence of any 4 of the 6 i tems i s i ndi cati ve of pri mary SS, as l ong as
ei ther i tem IV (hi stopathol ogy) or VI (serol ogy) i s posi ti ve
The presence of any 3 of the 4 objecti ve cri teri a i tems (that, i s i tems III, IV,
V, VI)
The cl assi fi cati on tree procedure represents a val id al ternati ve method of
cl assi fi cati on, al though i t shoul d be more properl y used i n
cl i ni cal epi demi ol ogi cal survey

For the di agnosi s of secondary SS:
In pati ents wi th a potenti al l y associ ated di sease (for i nstance, another wel l
def i ned connecti ve ti ssue di sease), the presence of i tem I or i tem II pl us any
2 from among i tems III, IV, and V may be consi dered as i ndi cati ve of
secondary SS

Excl usi on cri teri a:

Past head and neck radi ati on treatment
Hepati ti s C i nfecti on
Acqui red i mmunodefi ci ency di sease (AIDS)
Pre-exi sti ng l ymphoma
Sarcoi dosi s
Graf t versus host di sease
Use of anti chol i nergi c drugs (si nce a ti me shorter than 3-fol d the hal f-l i f e of the
drug).
*Source: Vi tal i , C. , Bombardi eri , S. , Jonsson, R. , et al . Ann Rheum Di s
(2002):61:554558. Cl assi fi cati on cri teri a for Sjgren' s syndrome: a revi sed
versi on of the European cri teri a proposed by the Ameri canEuropean Consensus
Group.
Table 8.12 Sjgren's syndrome: assessment and clinical
investigations
Hi story
Dry mouth
Di ff i cul ty eati ng
Di ff i cul ty swal l owi ng
Swel l i ng of sal i vary gl ands
Dry ski n
Vagi nal dryness
Connecti ve ti ssue di sease
Exami nati on
Mucosal dryness
No pool i ng of sal i va on f l oor of the mouth
Dental status
Bl ood tests
Ful l bl ood count
ESR
Immunol ogy
Inf l ammatory markers
Bi ochemi cal prof i l e
Sal i vary gl and i magi ng
Sci nti scanni ng
Si al ography
Bi opsy
Labi al gl and bi opsy.
Referral to
Ophthal mol ogi st
Physi ci an and/or rheumatol ogi st
P. 95
P. 96
Typical results of investigations in Sjgren's syndrome
Si al ometryl ow sal i vary fl ow rate
Labi al gl and bi opsyl ymphocyti c i nfi l trati on
Autoanti body screenposi ti ve autoanti bodi es, i n parti cul ar rheumatoi d f actor,
anti nucl ear, SS-A, SS-B
Sal i vary gl and i magi ngsci nti scan wi l l demonstrate reduced tracer uptake and
secreti on; si al ography wi l l show si al ectasi s
Schi rmer testreduced l acri mal fl ow rate
Excessive saliva
An i ncreased sal i vary fl ow rate i s al so known as si al orrhoea or ptyal i sm and, i n contrast to
xerostomi a, i t i s an uncommon compl ai nt. Hypersal i vati on may be transi ent or a chroni c
probl em. There are several reasons why pati ents may compl ai n of an i ncrease i n the
producti on of sal i va, but they are due to two mai n causes: hypersecreti on and neuromuscul ar
dysfuncti on. Excessi ve sal i va i s a frequent compl ai nt of pati ents who are weari ng an i ntraoral
prosthesi s for the f i rst ti me. In fact, one of the commonl y used methods of sti mul ati ng
sal i vary f l ow for experi mental purposes i s to use an i nert forei gn body wi thi n the mouth. Most
pati ents eventual l y become used to thei r new dentures or appl i ance and duri ng thi s process
the excess sal i vary fl ow usual l y di sappears. In a few pati ents, however, thi s may prove an
i ntractabl e probl em. Inf ected or ul cerati ve l esi ons i n the mouth may temporari l y cause an
i ncrease i n sal i vary fl ow, whi ch adds to the di scomf ort of the i ni ti al condi ti on. Thi s can be a
feature of pri mary herpeti c gi ngi vostomati ti s. A si mi l ar eff ect i s of ten seen i n carci noma of
the mouth, i n whi ch the i ncreased sal i vary fl ow may be accompani ed by a reduced swal l owi ng
refl ex and a constant dri bbl i ng of sal i va. It can be di ff i cul t to di sti ngui sh between
hypersal i vati on and drool i ngthe terms are not synonymous. In pati ents wi th
hypersal i vati on sal i va i s normal l y cl eared from the mouth by swal l owi ng. Drool i ng occurs due
to a fai l ure to swal l ow sal i va and i s common i n i nfants and al so i n those wi th poor
neuromuscul ar coordi nati on. Drool i ng i s not necessari l y caused by an overproducti on of
sal i va, but i t can occur because of i t.
Very few drugs i nduce excessi ve sal i vati on, a stark contrast to the number of drugs that
reduce sal i vary fl ow rate. Anti chol i nesterases, which enhance neuromuscul ar transmi ssi on
and are used i n the treatment of myastheni a gravi s, can cause hypersal i vati on. Interesti ngl y,
the anti psychoti c drug cl ozapi ne has been i mpl i cated i n causi ng a dry mouth and
hypersal i vati on.
Excessi ve sal i vati on may be due to:
hypersecreti on (for exampl e, the provi si on of a new i ntraoral prosthesi s, drugs)
neuromuscul ar dysf uncti on (for exampl e, cerebral pal sy)
oral dysaesthesi a (pati ents wi th oral dysaesthesi a are more l i kel y to report xerostomi a)
Systemi c condi ti ons, most notabl y neurol ogi cal di sturbances such as parki nsoni sm, cerebral
pal sy, and epi l epsy, can cause pati ents to compl ai n of excessi ve sal i vati on. In these
si tuati ons there may be no i ncrease i n the production of sal i va but swal l owi ng i s
uncoordi nated and i neffi ci ent. Mercury poi soni ng and rabi es are extremel y rare di seases that
have hypersal i vati on as a symptom.
Treatment for excessi ve sal i vati on depends l argel y on the el i mi nati on of (or habi tuati on to)
the causati ve factor, whether i t be a f orei gn body or an i nfecti ve l esi on. The use of drugs to
suppress sal i vary f l ow i s rarel y i ndi cated si nce virtual l y al l drugs wi th a marked sal i vary
suppressi ve eff ect al so exert other, and of ten more si gni fi cant, eff ects. Anti chol i nergi c drug
therapy i s someti mes used i n pati ents wi th cerebral pal sy who drool excessi vel y. One
frequent oral si de-eff ect of such medi cati on i s an i ncrease i n cari es rateof ten i n a
previ ousl y cari es-free denti ti on. Al ternati vel y, the major sal i vary gl and ducts can be
redi rected to the oropharynx to treat drool i ng. In a few pati ents compl ai ni ng of excessi ve
sal i vati on no i ncrease i n f l ow rate can be detected. In thi s si tuati on there may be an
underl yi ng cogni ti ve or psychi atri c di sturbance, and, i ndeed, some pati ents may

di spl ay obsessi onal trai ts. The cl i ni ci an can reassure the pati ent that there i s no seri ous
morbi di ty associ ated wi th thi s condi ti on and si al ometry may be hel pful i n demonstrati ng
sal i vary f l ow rates wi thi n the normal range. In a mi nori ty of pati ents, behavi oural therapy
may be benefi ci al .
Case 8.1 Discussion
Therapeuti c radi ati on can i nduce changes to mucosal , muscul ar, vascul ar, and osseous
ti ssues. Al terati ons to sal i vary gl and functi on are common as i s a shi f t i n oral mi crobi al fl ora,
resul ti ng i n a hi gher concentrati on of cari ogeni c bacteri a. The i rradi ated pati ent has an
i ncreased ri sk of devel opi ng cari es. Cl assi cal l y these occur at the cusp ti ps, i nci sal edges,
and cervi cal thi rds of the crown. The oncol ogi st, radi otherapi st, and denti st compri se a team
of heal th-care prof essi onal s whose coordi nated ef forts are requi red f or effecti ve management
of the radi otherapy pati ent. A pre-therapy dental eval uati on i s essenti al for the pati ent
undergoi ng radi ati on therapy that may aff ect the oral cavi ty and/or sal i vary gl ands. The
objecti ve shoul d be to establ i sh good oral heal th for the pati ent pri or to radi ati on
treatmentsfai l ure to do so coul d resul t i n compl i cati ons.
Teeth that requi re extracti on shoul d be removed as soon as possi bl e. It i s advi sabl e to l i ai se
wi th the oncol ogy uni t to di scuss the ti mi ng of extracti ons. Radi otherapy to the jaws can
make a pati ent suscepti bl e to osteoradi onecrosi s f ol l owi ng oral surgery (i ncl udi ng exodonti a,
especi al l y when mandi bul ar teeth requi re extracti on). Teeth wi th a l ong-term questi onabl e
prognosi s shoul d be removed preferabl y before radi otherapy commences.
The pati ent shoul d be warned that sal i vary fl ow may reduce dramati cal l y fol l owi ng
radi otherapy and the consequences of thi s on orodental heal th shoul d be di scussed wi th the
pati ent. The pati ent shoul d embark upon an i ntensi ve preventi ve programmeoral heal th
educati on i s of paramount i mportance. Nutri ti onal gui dance shoul d be offered and the use of
fl uori de therapy consi dered. The cl i ni ci an shoul d real i ze that compl i ance wi th such a regi men
may be a probl em, especi al l y i f the pati ent di d not mai ntai n an oral sel f -care programme
pri or to radi otherapy. Therefore treatment pl ans shoul d be real i sti c. Root canal treatment i s
not contrai ndi cated i n pati ents who are to undergo radi otherapy. Si al ogogues can be
benefi ci al i ncl udi ng the use of systemi c pi l ocarpi ne. The dose regi men and si de-eff ects for
thi s drug can be obtai ned from the Bri ti sh Nati onal Formul ary or the manufacturer' s data
sheet.
Long-term pati ent fol l ow-up i s necessary on the part of both the denti st and hygi eni st. The
pati ent may al so experi ence non-speci f i c oral mucosi ti s fol l owi ng radi ati on and wi l l be
suscepti bl e to oral candi dosi s. Post-i rradi ati on si al adeni ti s i s al so a possi bl e si de-eff ect.
SCase 8.2 Discussion
P. 97
Q1 What advi ce woul d you gi ve to thi s pati ent concerni ng hi s f uture oral heal th?
Q1 What questi ons woul d you ask thi s l ady?
Questi ons shoul d be asked to assess the severi ty of the dry mouth. the questi ons l i sted i n
Tabl e 8. 8 can hel p i denti f y i f a pati ent has oral dysfuncti on associ ated wi th sal i vary gl and
hypofuncti on. A ful l dental and medi cal hi story shoul d be taken. It i s al so hel pful to do a
di etary anal ysi sthe i nci dence of cari es coul d i ncrease as a resul t of more frequent i ntake
of carbohydrate. Pati ents who devel op a dry mouth may erroneousl y use mi nts or ci trus-
fl avoured sweets to obtai n symptomati c rel i ef. A ful l medi cal hi story shoul d be taken and
speci f i c questi ons that el i ci t the symptoms of Sjgren' s syndrome shoul d be used, f or
exampl e, do you have dry eyes? do you have rheumatoi d arthri ti s? A thorough drug hi story
shoul d be obtai ned. If you are i n doubt as to the si de-eff ects of any medi cati on, a ref erence
book such as the Bri ti sh Nati onal Formul ary shoul d be consul ted. The l ocal and regi onal UK
Medi ci nes Informati on servi ce wi l l al so be hel pful .
The treatment of thi s pati ent wi l l depend upon the aeti ol ogy. If sal i vary gl and hypof uncti on
i s suspected, then the pati ent shoul d embark upon an i ntensi ve preventi ve regi me (oral
hygi ene i nstructi on, di etary advi ce, f l uori de suppl ements, si al ogogues, and possi bl y
pi l ocarpi ne). The pati ent may meri t referral to an oral medi ci ne department i f you suspect
The most frequent cause of xerostomi a i s the i ntake of drugs wi th anti chol i nergi c acti vi ty.
Case 8.3 Discussion
The preci pi tati ng and rel i evi ng factors i n the pai n hi story are suggesti ve of an obstructi on i n
a submandi bul ar duct. The accumul ati on of sal i va proxi mal to the bl ockage i n the duct causes
pai n and swel l i ng. The symptoms are worse j ust pri or to a meal , when there i s a ri se i n the
producti on of sal i va. The obstructi on i s most l i kely to be due to a sal i vary stone (cal cul us,
si al ol i th). However, other causes coul d be a mucous pl ug, ductal stenosi s, or a neopl asm.
You woul d need to expand upon the pai n hi story and establ i sh i f the pai n i s preci pi tated by
any other gustatory or ol factory sti mul i . It i s unli kel y that thi s pai n has a spontaneous onset.
Is the pai n bi l ateral ? Si al ol i ths are usual l y uni l ateral aff ecti ng onl y one sal i vary gl and.
You shoul d pal pate the submandi bul ar gl and and ductboth extra- and i ntraoral l y
(bi manual pal pati on). The si ze of the sal i vary gl and shoul d be compared wi th that of the
contral ateral gl and and the duct shoul d be pal pated for a stone or any i rregul ari ti es of
contour or consi stency. A pl ai n occl usal radi ograph may be useful

i n i denti f yi ng cal cul i . However, they are not al l radi o-opaque. Si al ography may be requi red to
i denti fy the l ocati on of the obstructi on. A sci nti scan i s a functi onal assessment of sal i vary
gl and acti vi ty and i s of l i mi ted di agnosti c val ue. Ul trasonography can be useful i n i denti fyi ng
the si te of any obstructi on and the presence of cal cul i . It may al so i ndi cate the presence of
an extra- or i ntragl andul ar tumour.
The management woul d depend upon the cause of the obstructi on. The pati ent requi res
referral for sal i vary gl and i magi ng and removal of the obstructi on. A sal i vary duct cal cul us
can someti me be successf ul l y mi l ked al ong the duct and mani pul ated out of the
ori fi ce. A cal cul us l yi ng i n the submandi bul ar duct that runs superfi ci al l y al ong the fl oor of
the mouth can be removed by i nci si ng the overl yi ng mucosataki ng care not to damage the
Q2 What advi ce woul d you gi ve?
Q3 What i s the most frequent cause of xerostomi a?
Q1 What f urther questi ons woul d you ask thi s pati ent?
Q2 How woul d you exami ne thi s pati ent?
P. 98
Q3 How woul d you manage hi m?
l i ngual nerve. The i nci si on i s often l eft unsutured to mi ni mi ze possi bl e duct obstructi on due
to scarri ng. Li thotri psy has been used f or l arge sal i vary cal cul i . Submandi bul ar sal i vary
gl ands requi re surgi cal exci si on i f they are severel y damaged and associ ated wi th persi stent
symptoms.
In a heal thy pati ent, xerostomi a i s unl i kel y to accompany a condi ti on that affects onl y one
major sal i vary gl and. It i s usual l y a symptom of mul ti gl andul ar di sease.
Project
1. Fi nd out about the cl i ni cal presentati on, hi stopathol ogi cal f eatures, and management of
tumours that may af fect the sal i vary gl ands.
Q4 Is thi s pati ent l i kel y to compl ai n of a dry mouth?
> Tabl e of Cont ent s > 9 - Inf l ammat ory over gr owt hs, devel opment al and beni gn l esi ons, and
pi gment at ion of the oral mucosa
9
Inflammatory overgrowths, developmental and
benign lesions, and pigmentation of the oral
mucosa
Problem cases
Case 9.1
A 30-year-ol d Caucasi an man attends your dental practi ce for the fi rst ti me. He i s worri ed
about a pi gmented patch that he has noti ced on the roof of hi s mouththi s has caused no
symptoms. The pati ent i s f i t and wel l and not taki ng any medi cati on. Hi s father, however,
di ed of mal i gnant mel anoma on the ski n when the pati ent was a boy. Exami nati on reveal s an
i sol ated, smal l (34 mm i n di ameter), brown pi gmented macul e, on the pal atal mucosa.
The rest of the oral mucosa appears compl etel y normal and there are no pal pabl e l ymph
nodes.
Case 9.2
A 75-year-ol d edentul ous l ady attends f or provi si on of repl acement f ul l dentures. Her
exi sti ng dentures are 20 years ol d and i l l -fi tti ng. In the past, she had suffered some
di scomf ort where the top dentures had di gged i n, but thi s had eventual l y settl ed
down. At the ti me of your exami nati on she i s asymptomati c. Her exi sti ng dentures are
di scol oured and the teeth badl y worn. Exami nati on of the oral mucosa reveal s a soft-ti ssue
l esi on, extendi ng al ong the buccal sul cus, adjacent to the l eft maxi l l ary tuberosi ty regi on.
Thi s consi sts of mul ti pl e fol ds of hyperpl asti c-l ooki ng mucosal ti ssue.
Inflammatory overgrowths
Al l new growths of the oral ti ssues shoul d be treated wi th suspi ci on and must be ful l y
i nvesti gated. There are some wel l -def i ned condi ti ons (for i nstance, denture granul omas) of
whi ch the cl i ni cal di agnosi s can be accurate i n practi cal l y al l cases, but appearances can be
mi sl eadi ng and i t cannot be too strongl y stressed that absol ute certai nty as to the nature of
any l esi on can onl y come after hi stol ogi cal exami nati on.
Epulides
Q1 What i s the di f ferenti al di agnosi s of thi s l esi on and how woul d you manage thi s case?
Q1 What i s the most l i kel y cl i ni cal di agnosi s of this l ady' s l esi on?
Q2 How i s thi s l esi on managed?
Q2 A more si ni ster oral l esi on can occasi onal l y present i n a si mi l ar way. What i s thi s and
how can i t be di fferenti ated f rom the more common, beni gn oral l esi on?
An epul i s (pl ural , epul i des) i s def i ned as a sof t-ti ssue swel l i ng of the gi ngi val margi n. The
term i s more speci f i cal l y used to descri be a range of hyperpl asti c i nfl ammatory l esi ons
ari si ng f rom the peri odontal ti ssues. They represent an exaggerated i nfl ammatory response
of the peri odonti um, al though the source of i rri tati on i s not al ways obvi ous. The
i nfl ammatory process ari ses i ni ti al l y i n the i nterdental ti ssues, and there i s often an
associ ated l oss of al veol ar crest-bone that may become severe i n advanced cases. Three
types, the fi brous epul i s, the pyogeni c granul oma (pregnancy epul i s), and the gi ant-cel l
epul i s, are commonl y seen, ref l ecti ng di fferi ng stages of the i nfl ammatory and bone-
resorbi ng process. Most epul i des are more common i n f emal es than mal es and usual l y occur
anteri or to the mol ar teeth.
Fibrous epulis
The f i brous epul i s presents as a peduncul ated or sessi l e swel l i ng on the gi ngi vae and
essenti al l y consi sts of heavi l y f i brosed granul ati on ti ssue. Its content of col l agen fi bres gi ves
i t a fi rm, rubbery texture and i ts col our i s usual ly pal e pi nk. The degree of fi brosi s depends,
however, on the stage of maturi ty of the epul i s, and a l esi on i n i ts earl y stages may be soft
i n texture and wi th a hi stol ogi cal appearance that shows very many cel l s. In a l ongstandi ng
l esi on, f ocal mi neral i zati on and/or bone formati on may occur. Acute i nf l ammatory changes
may f ol l ow trauma or i nf ecti on and, i n such cases, the epul i s may become red and sore (Fi g.
9. 1).
Pyogenic granuloma and pregnancy epulis
These are both vascul ar epul i des and present cl i ni cal l y as reddi sh-purpl e swel l i ngs on the
gi ngi vae. A pregnancy epul i s i s hi stol ogi cal l y i denti cal to the pyogeni c granul oma but occurs
i n pregnancy (see Chapter 13). In the pyogeni c granul oma and pregnancy epul i s the
granul ati on ti ssue remai ns vascul ar and i mmature. It i s much redder i n col our than the
fi brous epul i s proper and has a tendency to bl eed easi l y because of i ts hi gh vascul ar content.
The giant-cell epulis (peripheral giant-cell granuloma)
The gi ant-cel l epul i s i s a l esi on i n whi ch the granul ati on ti ssue i s osteogeni c i n nature. Its
predomi nant hi stol ogi cal feature i s the

presence of mul ti nucl eated gi ant cel l s di spersed i n a vascul ar stroma. Wi th maturi ty the
l esi on may become l ess vascul ar and more fi brosed and may i ncl ude some areas of bone
formati on. In i ts i mmature form, thi s epul i s i s characteri sti cal l y red-purpl e i n col our.
P. 102
Epulides
Fi brous epul i s
Vascul ar epul i des
Pyogeni c granul oma
Pregnancy epul i s
Gi ant cel l epul i s (peri pheral gi ant-cel l granul oma)
Management
Treatment of al l of these forms of epul i s i s by l ocal exci si on. The ori gi n of the l esi on i s of ten
i nterdental and, i n more advanced cases, the peri odontal membrane may be qui te deepl y
i nvol ved. If exci si on i s not compl ete, there may be recurrence of the l esi on and so, al though
radi cal surgi cal techni ques are not cal l ed for, the i ni ti al removal shoul d i ncl ude al l affected
ti ssue. Wi th repeated recurrence, i t i s someti mes necessary to remove the adjacent teeth i n
order to secure the el i mi nati on of the ti ssue of ori gi n. In thei r tendency to recur, epul i des
may appear to be neopl asms, but the recurrence i s due onl y to persi stence of the condi ti ons
that caused the i ni ti al abnormal response. The ti mi ng for the exci si on of a pregnancy epul i s
i s further di scussed i n Chapter 13.
Al though the cl i ni cal di agnosi s may be a confi dent one, i t shoul d al ways be confi rmed by
hi stol ogi cal exami nati on. Occasi onal l y, a neopl asm may present i n a f orm resembl i ng a
si mpl e epul i s and i n a l i kel y si te for one. It shoul d al so be remembered that a central gi ant-
cel l granul oma may perforate al veol ar bone and appear as an epul i s. Appropri ate
i nvesti gati ons (i ncl udi ng bl ood tests f or pl asma cal ci um, phosphorus, and al kal i ne
phosphatase) shoul d be carri ed out on al l pati ents presenti ng wi th a hi stol ogi cal l y confi rmed
gi ant-cel l epul i s to excl ude hyperparathyroi di sm. Any symptoms or hi story that mi ght i mpl y
undi agnosed hyperparathyroi di sm (such as renal cal cul i ) shoul d be taken i nto consi derati on
(Chapter 18).
Fibroepithelial polyp
Thi s l esi on, si mi l ar i n structure to the mature f i brous epul i s, i s essenti al l y scar ti ssue
produced as a response to trauma, such as repeated i rri tati on of the buccal or l abi al mucosa,
al ong the occl usal pl ane of the teeth, often caused by a bi te. It i s usual l y seen i n adul ts and
there i s no sex di fferenti ati on. The l esi on appears as ei ther a sessi l e or a peduncul ated
swel l i ng (Fi g. 9. 2) and i s qui te f ree of symptoms unl ess secondari l y traumati zed. The usual
si ze of such a l esi on when the pati ent presents for treatment i s of the order of 1 cm i n
di ameter, but occasi onal l ongstandi ng l esi ons are seen that are very much l arger. The col our
of the l esi on i s pi nk and the texture vari es from soft to rubbery, dependi ng on the maturi ty
of the consti tuent f i brous ti ssue. Si nce thi s l esi on i s si mpl y an exaggerated and chroni cal l y
i rri tated mass of scar ti ssue, treatment need onl y be conservati ve, exci si on to the l i mi t of
the swel l i ng or to the base of the pedi cl e bei ng all that i s requi red. Recurrence wi l l occur
onl y i f trauma i s repeated and i s, i n fact, uncommon. As wi th al l other ti ssue overgrowths,
absol ute certai nty of di agnosi s can come onl y after hi stol ogi cal exami nati on, al though a
cl i ni cal di agnosi s can often be made wi th a fai r degree of confi dence. An i nteresti ng cl i ni cal
vari ant i s one i n whi ch a f i broepi thel i al l esi on (the so-cal l ed l eaf fi broma) devel ops
under the pal ate of an upper denture. In such cases the normal shape of the l esi on i s
di storted and fl attened i nto a thi n di sc that fi ts i nto a shal l ow depressi on i n the pal ate. It
Fig. 9.1 A rel ati vel y earl y and i mmature fi brous epul i s.
retai ns a pedi cl e and may be di spl aced downwards on i t l i ke a hi nged fl ap.

Denture granuloma
Thi s i s a l esi on essenti al l y si mi l ar to the fi broepi thel i al pol yp, the i rri tati ng factor i n thi s case
bei ng the f l ange of an overextended or i l l -fi tti ng denture. As i n the case of the pol yp,
prol i ferati ve scar ti ssue i s formed f ol l owi ng chroni c trauma. The typi cal fi ssured shape of the
denture granul oma resul ts f rom the i ndentati on caused by the f l ange of the denture (Fi g.
9. 3). These l esi ons are rarel y pai nful and, i ndeed, often cause astoni shi ngl y l i ttl e troubl e to
the pati ent. Thi s bei ng so, occasi onal l esi ons are seen that are very l arge i ndeed, wi th
mul ti pl e f ol ds of prol i f erati ve ti ssue. The denture granul oma i s a beni gn l esi on and i s treated
by si mpl e exci si on after removi ng the offendi ng denture or drasti cal l y tri mmi ng i t away from
the affected area. The removal of the source of chroni c i rri tati on i s i n many cases suff i ci ent
to reduce consi derabl y the si ze of the l esi on wi thin a rel ati vel y short ti me and even to make
exci si on unnecessary.
Focal epithelial hyperplasia (Heck's disease)
Thi s condi ti on (otherwi se known as Heck' s di sease) has been ful l y descri bed and i nvesti gated
onl y i n recent years. The pati ents are predomi nantl y chi l dren from Bl ack Afri can, Eski mo, and
Ameri can Indi an groups, al though a very f ew cases have been reported i n Whi te Europeans.
In thi s condi ti on, mul ti pl e rai sed sessi l e l esi ons appear on the buccal and l abi al mucosa. The
mucosa retai ns a rel ati vel y normal pi nk appearance and the texture of the l esi ons i s soft.
There i s no ul cerati on (except i n the case of secondary trauma) and the l esi ons are qui te
pai n-free. Hi stol ogi cal l y, the epi thel i um, overl yi ng a rel ati vel y normal cori um, appears
hyperpl asti c, wi th marked cel l ul ar i rregul ari ti es. Thi s condi ti on i s associ ated wi th speci f i c
types of the human papi l l omavi rus, al though there is thought al so to be a geneti c basi s
responsi bl e for i ts hi gh i nci dence i n the groups menti oned above. No treatment i s necessary.
It i s a sel f-l i mi ti ng condi ti on and regresses completel y af ter a vari abl e peri od.
Fig. 9.2 A l arge fi broepi thel i al pol yp of the buccal mucosa.
P. 103
Developmental lesions
Hamartomas
A hamartoma i s a l ocal i zed non-progressi ve ti ssue abnormal i ty resul ti ng from a defect i n
devel opment. It i s nei ther i nf l ammatory nor neopl asti c i n nature but, si nce i t may be
confused wi th condi ti ons of ei ther type, i t shoul d be consi dered i n rel ati on to them. When
present on ski n or mucous membrane the term naevus i s often used even when the
naevus cel l s (the mel anocytes) are i n no way i nvol ved. In such a l esi on, a si ngl e el ement of
the mucosa, epi thel i al , vascul ar, or l ymphati c, i s predomi nantl y i nvol ved.
Angiomatous naevae
These are the resul t of devel opmental abnormal i ti es i n ei ther the l ymphati c or vascul ar
components of the mucosa. Vascul ar naevae (haemangiomas) are rel ati vel y common l esi ons
of the oral mucosa resembl i ng the strawberry mark of ski n. Dependi ng on the degree
of di l atati on of the abnormal bl ood vessel s, the l esi on may appear as a f i ne network of
capi l l ari es or as a more pronounced nodul ar structure, usual l y fi l l ed wi th sl ow-movi ng venous
bl ood and, therefore, dark-bl ue i n col our. The cavernous f orm may be mi staken cl i ni cal l y for
a mel anoma, but can be qui ckl y di fferenti ated by i ts tendency to bl anch on pressure. Thi s
can be seen by pressi ng a gl ass sl i de down on to the surf ace. It shoul d be emphasi zed that
the vascul ar naevus i s a stati c devel opmental abnormal i ty that i s asymptomati c and i s best
l ef t undi sturbed. A si mi l ar naevus i nvol vi ng the l ymph vessel s i s the l ymphangi oma. It
consi sts of a col l ecti on of di l ated l ymphati c vessel s and spaces i n a connecti ve ti ssue stroma
and i s very si mi l ar to the vascul ar naevus i n structure. Al though i t can appear on any part of
the oral mucosa, i t i s seen much more commonl y on the tongue than el sewhere. If the l esi on
i s superfi ci al i t appears as a transl ucent whi te structure on the mucosa. If i t i s deepl y
si tuated the overl yi ng surface of the tongue appears greyi sh and nodul ar. Thi s i s al so an
enti rel y i nnocuous l esi on.
There may be probl ems of bl eedi ng as a resul t of trauma to these l esi ons and acti on may be
cal l ed for, al though the bl eedi ng rarel y reaches dangerous proporti ons i n these
ci rcumstances. Cryosurgery has been used to deal with thi s si tuati on i n the past, al though
Fig. 9.3 A denture granul oma i n the l ower buccal sul cus.
not wi thout some di ffi cul ti es. There can be probl ems wi th postcryotherapy oedema of the
tongue. The devel opment of l asers has, however, provi ded an answer to thi s probl em. If
there i s any doubt as to whether or not a l esi on i s a haemangi oma, bi opsy exami nati on may
be necessary, but angi omatous l esi ons of ten bl eed copi ousl y. No suspect vascul ar l esi ons
shoul d ever be subjected to any form of surgery except i n hospi tal .
Developmental white lesions
A number of geneti cal l y determi ned whi te l esi ons of the oral mucosa exi st, several of whi ch
are associ ated wi th l esi ons of other mucous membranes or of the ski n. These are
characteri zed by di sturbances of kerati ni zati on and are often cl assi fi ed as
genokeratoses or genodermatoses i n the dermatol ogi cal l i terature.

Oral epithelial naevus (white sponge naevus)
The best-known l esi on af fecti ng the oral mucosa al one i s that of the oral epi thel i al naevus
(whi te sponge naevus). The desi gnati on whi te f ol ded gi ngi vostomati ti s has al so been
used wi th ref erence to thi s condi ti on, but the fi rst use of thi s term was to descri be the oral
l esi on i n a mul ti pl e abnormal i ty i nvol vi ng other mucous membranes. These terms are,
however, of ten used i nterchangeabl y, and i t may well be that condi ti ons affecti ng the oral
mucosa al one represent an i ncompl ete expressi on of a more general i zed mucosal
abnormal i ty.
Whi te sponge naevus i s a l ocal i zed devel opmental tissue abnormal i ty that i s nei ther
i nfl ammatory nor neopl asti c. Whi te sponge naevus appears as a f ol ded whi te l esi on of the
oral mucosa that may be so extensi ve as to aff ect the whol e of the oral cavi ty. It may be
present from bi rth, but i n certai n cases the condi ti on may become evi dent onl y i n l ater l i fe.
There has been, i n the past, some doubt as to the exact geneti c mechani sm i nvol ved i n the
producti on of thi s l esi on, but i t has now been demonstrated that the condi ti on di spl ays
cl assi cal domi nant transmi ssi on characteri sti cs i n some, but not al l pati ents. In other cases
there i s no f ami l y hi story and the condi ti on appears to ari se i n i sol ati on. There are no
reported i nstances of mal i gnant transf ormati on i n these l esi ons, and they are general l y
regarded as bei ng enti rel y beni gn. Hi stol ogi cal exami nati on of the af fected oral mucosa
shows the consi stent presence of acanthosi s wi th parakeratosi s and i ntracel l ul ar oedema.
Characteri sti c of thi s l esi on and al so of many si mil ar l esi ons i n the more compl ex mucosal
syndromes i s the presence of many l arge cl ear cel l s extendi ng throughout the stratum
spi nosum and to the surf ace of the epi thel i um.
Pachyonchia congenita, benign intraepithelial dyskeratosis,
and dyskeratosis congenita
In pachyonchi a congeni ta, an autosomal domi nant i nheri ted condi ti on, whi te l esi ons of the
oral mucosa and rectum are associ ated wi th defecti ve formati on of the nai l s of the hands and
feet. In heredi tary beni gn i ntraepi thel i al dyskeratosi s, the abnormal i ty i s restri cted to the
oral mucous membrane and conjuncti va, but the hi stol ogy of the oral l esi ons reveal s a
degree of dyskeratosi s that woul d be consi dered al armi ng i n other l esi ons of the oral
mucosa. In spi te of thi s, however, the condi ti on, as i ts name i mpl i es, remai ns essenti al l y
beni gn. Dyskeratosi s congeni ta i s characteri zed by hyperkeratosi s of the mucous membranes
(i ncl udi ng the oral cavi ty), dystrophi c nai l s, and abnormal pi gmentati on of the ski n.
Peri odontal mani f estati ons of thi s condi ti on have been reported.
Tylosis (palmoplantar keratoderma)
In the 1950s an associ ati on was found between whi te l esi ons of the oral mucosa,
oesophageal carci noma, and tyl osi s (hyperkeratosi s of the pal ms and sol es). In thi s exampl e
of a compl ex dermal mucosal syndrome, the characteri sti c oral l esi ons cl i ni cal l y and
P. 104
hi stol ogi cal l y resembl e those of a l eukokeratosi s. Thi s l atter syndrome, l i ke most other
dermal mucosal syndromes of thi s type, i s transmi tted as a domi nant condi ti on. Li nkage
anal ysi s has recentl y been undertaken i n a l arge fami l y of tyl oti c pati ents and the l ocati on of
the oesophageal cancer gene i s l i kel y to be i denti fi ed i n the near future. It woul d seem l i kel y
that a wi de spectrum of such condi ti ons exi sts and, i ndeed, i sol ated exampl es of varyi ng
degrees of combi ned dermal and mucosal abnormal i ti es have frequentl y been reported. The
cl assi fi cati on of pal mopl antar keratoderma (PPK), of whi ch tyl osi s i s one type, has recentl y
been redefi ned. The term pal mopl antar ectodermal dyspl asi a has now been proposed
to emphasi ze the mul ti pl e ectodermal structures i nvol ved i n some patterns of PPK.
Darier's disease
Dari er' s di sease, al so known as fol l i cul ar keratosi s, i s a geneti cal l y determi ned
di sease. It i s usual l y fi rst seen i n young adul ts and general l y mani f ests as greasy brown
papul es on the chest and shoul ders. Pi tti ng of the ski n of the pal ms of the hands and nai l can
occur. The oral mucosa i s aff ected i n a si gni f i cant proporti on of cases and the di sease usual l y
presents as coal escent whi te l esi ons on the buccal mucosae or pal ate.
Benign neoplasms
Neopl asms of many ki nds, both pri mary and secondary, may occur i n the mouth. It i s
possi bl e to descri be typi cal cl i ni cal features f or many of these l esi ons, but i t must be
remembered that mi sl eadi ng and atypi cal forms may al so present and may l ead to di agnosti c
error. The f i nal di agnosi s of any ti ssue overgrowth depends enti rel y on hi stol ogi cal
i nvesti gati on and thi s must i nvari abl y be undertaken i f any doubt at al l exi sts as to the true
nature of a l esi on. The most common neopl asms af fecti ng oral mucosa are the squamous cel l
papi l l oma, whi ch i s a beni gn tumour, and squamous cel l carci noma, whi ch i s a mal i gnant
tumour (see Chapter 10). The rol e of the human papi l l omavi rus (HPV) i n the devel opment of
both of these neopl asms remai ns specul ati ve. A number of connecti ve-ti ssue neopl asms (for
exampl e, fi bromas, l i poma) can occur i n the mouth but these are l ess common.
Squamous cell papilloma
Thi s beni gn epi thel i al neopl asm i s rel ati vel y common. It may appear anywhere on the oral
mucosa, but i s most commonl y found at the juncti on of the hard and sof t pal ates. Its typi cal
appearance i s caul i fl ower -l i ke and peduncul ated wi th a pal e col our varyi ng from that
of normal mucosa to whi te. It i s a pai nl ess l esi on that rarel y gi ves troubl e. Mal i gnant
transformati on has not been descri bed i n these l esi ons and, i n thi s respect, the oral l esi ons
behave qui te di f ferentl y from those i n the l ower parts of the gastroi ntesti nal tract, whi ch
have a defi ni te tendency to undergo mal i gnant change.
Treatment of a papi l l oma i s by l ocal exci si on. Thi s, however, must be suff i ci entl y wi de and
deep to i ncl ude any abnormal cel l s that may extend beyond the area of the pedi cl e. It i s not
suff i ci ent

to si mpl y sever the pedi cl ethi s wi l l l ead to recurrence. Removal wi th a l aser i s an
al ternati ve.
Mul ti pl e vi ral warts, cl i ni cal l y si mi l ar to the sol i tary papi l l oma, occasi onal l y occur i n the
mouth or, more commonl y, on and around the l i ps. In chi l dren these are often the resul t of
autoi nocul ati on by chewi ng warts on the hands. Sexual transmi ssi on between geni tal and oral
si tes has al so been descri bed. Venereal warts (condyl oma acumi natum), whi ch
characteri sti cal l y occur i n the anogeni tal regi on and may be a mani festati on of HIV i nf ecti on
(see Chapter 4), can be seen on the oral mucosa. A l arge number of papi l l omavi ruses has
been descri bed and i t i s possi bl e to cl assi fy oral warts/papi l l omas i n vari ous ways. For
practi cal purposes, however, the di vi si on i s cl ear between sol i tary l esi ons wi th no evi dent
i nfecti ve aeti ol ogy and those that are the resul t of i nocul ati on. Management i s the same,
P. 105
al though, cl earl y, the wart-chewi ng habi t of af fected chi l dren shoul d be di scouraged.
Vi ral warts general l y spontaneousl y regress after a peri od of a year or so.
Miscellaneous benign conditions
Traumatic keratoses
Traumati c keratoses on the oral mucosa are caused by a l ocal reacti on to earl i er physi cal ,
mechani cal , el ectri cal , or thermal causes and are reversi bl e. Chemi cal trauma caused by an
i rri tant substance, such as topi cal use of an aspi ri n, can cause ei ther oral ul cerati on (see
Chapter 5) or hyperkeratosi s, whi ch mani f ests as a whi te l esi on on the oral mucosa. Acute
chemi cal i nsul ts, such as i ngesti on of a causti c or aci d substance, tend to cause oral
ul cerati on. Lesi ons seen on the oral mucosa of i ndi vi dual s who smoke ci garettes, ci gars, or
pi pes are due to a combi nati on of chemi cal and thermal i nsul ts. The rol e of tobacco i n the
devel opment of premal i gnant l esi ons i s f ul l y di scussed i n Chapter 10. Ni ctoni ni c stomati ti s
due to pi pe-smoki ng i s di scussed bel ow.
Frictional keratosis
An i mportant factor i n the producti on of whi te patches of the oral mucosa i s the ef fect of
mechani cal trauma. It i s wel l known that such trauma may have a wi de vari ety of effects on
the mucosa, dependi ng on the exact nature of the trauma, i ts rate of appl i cati on, and an
i ndi vi dual ' s response. When the trauma i s acute and l ocal i zed, the epi thel i um i s destroyed
and an ul cer i s produced. If , however, the trauma is l ess acute and l ess l ocal i zed, then there
wi l l be a range of responses dependi ng on the precise cl i ni cal condi ti ons. If the i rri tati on i s
rel ati vel y chroni c i n character and of l ow i ntensi ty, the major change i nduced i n the oral
mucosa may wel l be an abnormal i ty i n the kerati ni zati on pattern, whi ch must be associ ated
wi th some degree of change i n the underl yi ng cel l s. There i s no doubt that such traumati c
keratoses exi st and that the l arge majori ty are reversi bl e, compl etel y di sappeari ng i f the
trauma i s removed. It i s of i nterest to recal l that the mucosal response to trauma most of ten
seen i n the oral cavi ty i s that of the denture granul oma. The pri nci pal change i n thi s
parti cul ar l esi on i s i n the connecti ve ti ssue component of the mucosa rather than i n the
epi thel i um.
Nicotinic stomatitis (pipe-smokers' palate)
Ni coti ni c stomati ti s i s usual l y found, as the name i mpl i es, i n pi pe-smokers, but al so,
occasi onal l y, i n ci garette- and ci gar-smokers. The characteri sti c hi stol ogi cal feature of thi s
condi ti on i s the combi nati on of epi thel i al acanthosi s and hyperkeratosi s wi th i nf l ammatory
changes i n the mucous gl ands of the pal ate. As a resul t of these changes, the pal ate
becomes whi te and a number of nodul es project f rom the surface, each representi ng the si te
of a mucous gl and and i ndi vi dual l y beari ng a smal l red spot at the centre that marks the
openi ng of the duct of the gl and (Fi g. 9. 4). In advanced cases the mucous gl ands may break
down to form qui te l arge chroni c ul cers. The condi ti on usual l y appears most marked on the
hard pal ate, al though the soft pal ate may al so be invol ved. It has been suggested that the
si te of i nvol vement depends upon the projecti on of pi pe smoke di rectl y on to the pal ate, but
thi s i s not easy to prove. In spi te of the constant and chroni c exposure to the i rri tant factor,
mal i gnant transformati on i s not a feature of thi s lesi on, whi ch i s not consi dered to be
premal i gnant. Management i s di rected at persuadi ng the pati ent to stop smoki ng. If thi s i s
done, resol uti on of the l esi on (of ten compl ete) can be reasonabl y expected. An added benefi t
i s that the oral mucosa i s no l onger exposed to chemi cal carci nogens that may predi spose to
the devel opment of premal i gnant l esi ons f or carci noma el sewhere i n the mouth or
aerodi gesti ve tract.
It shoul d be poi nted out that the above descri pti on of the condi ti on appl i es onl y to that
characteri sti cal l y seen i n European and North Ameri can pi pe-smokers. Other tobacco-i nduced
pal atal l esi ons have been descri bed that have a very di fferent prognosi s (see Chapter 10 for
pal atal l esi ons associ ated wi th reverse smoki ng).
Leukoedema
Leukoedema i s the name gi ven to a fi l my grey coati ng of the buccal mucosa that i s found i n a
hi gh proporti on of pati ents. There have been wi del y di vergent esti mates of both the
i nci dence

and the si gni f i cance of thi s condi ti on, one of the di ffi cul ti es i n thi s bei ng that of defi ni ti on.
However, there can be no doubt that such a fi l my coati ng appears on the buccal mucosa of a
l arge number of compl etel y asymptomati c pati ents when vi ewed i n adequate l i ghti ng
condi ti ons.
Hi stol ogi cal l y, thi s l esi on has been descri bed as bei ng parakeratoti c, wi th l arge swol l en cel l s
i n the superf i ci al l ayers of the epi thel i um. Thi s hi stol ogi cal appearance i s qui te compati bl e
wi th the observati ons that the grey surf ace fi l m (consi sti ng of the superfi ci al oedematous
cel l s) may easi l y be scraped away f rom the mucosa, l eavi ng an apparentl y i ntact surface that
agai n rapi dl y acqui res the superfi ci al l y grey appearance. Leukoedema i s not associ ated wi th
epi thel i al atypi a and shoul d not be regarded as a premal i gnant l esi on.
Pigmentation of the oral mucosa
Pi gmentati on of the oral mucosa can occur as a resul t of envi ronmental or occupati onal
exposure to heavy metal sal ts, such as bi smuth, l ead, and mercury, some of whi ch were used
i n the past as therapeuti c agents for a number of di seases such as syphi l i s. Fol l owi ng
absorpti on of the metal s, they are deposi ted as metal l i c sul f i des as a grey (or bl ue/bl ack)
l i ne al ong the margi nal gi ngi vae.
Amalgam tattoos
Amal gam tattoos are a resul t of fragments of the materi al bei ng embedded i n the oral
mucosa. These appear as i sol ated pi gmented l esi ons (l i ght-brown to dark-bl ue/bl ack i n
col our). Amal gam tattoos may be radi o-opaque, but thi s i s not al ways the case (Fi g. 9. 5).
They usual l y occur on the f l oor of the mouth or al veol ar mucosa, near to exi sti ng or previ ous
P. 106
Fig. 9.4 Smoker' s keratosi s of the pal ate.
amal gam restorati ons. Marked amal gam tattoos may be seen on the attached gi ngi vae,
overl yi ng teeth that have been api cetomi zed and api cal l y seal ed wi th amal gam.
Other forei gn substances such as road gri t can be impl anted i n the oral mucosa and
occasi onal pati ents present wi th messages (often rude!) tattooed on thei r l abi al mucosae.
Melanotic pigmentation of oral mucosa
The si gni fi cance of oral pi gmentati on i n endocri ne di sturbances, and parti cul arl y i n Addi son' s
di sease, i s di scussed i n Chapter 13. An associ ati on between oral mel anosi s (parti cul arl y of
the soft pal ate), smoki ng, and bronchogeni c carci noma has al so been reported. Oral
mel anosi s may occur i n HIV i nf ecti on (Chapter 4). A number of drugs may al so sti mul ate
i ncreased pi gmentati oni ncl udi ng oral contracepti ves, anti mal ari al s, and major
tranqui l l i zers. There have been recent reports of mi nocycl i ne causi ng pi gmenti on of the ski n
and oral mucosa. Increased mel ani n producti on may al so occasi onal l y be seen i n associ ati on
wi th the oral l esi ons of l i chen pl anus and wi th l eukopl aki asthi s i s reacti ve and of no
cl i ni cal si gni f i cance. However, i n many pati ents, parti cul arl y those wi th heavi l y pi gmented
ski ns, oral pi gmentati on i s qui te normal . Thi s may be patchy or di ffuse, but the gi ngi vae are
al most al ways i nvol ved, even when the ski n pi gmentati on i s mi ni mal .
The PeutzJegher syndrome i s an autosomal domi nant syndrome, compri si ng mel anoti c
macul es peri oral l y and oral l y together wi th i ntesti nal pol yposi s. Cl i ni cal l y, thi s syndrome
mani fests as mul ti pl e freckl es occurri ng around the nose and eyes and on the l i ps and the
oral mucosa. The i ntesti nal pol yps can be present throughout the gut and may gi ve ri se to
symptoms, such as abdomi nal pai n or cause i ntesti nal obstructi on. These pol yps do not
usual l y become mal i gnant.
The Laugi erHunzi ker syndrome (i di opathi c l enti cul ar mucocutaneous pi gmentati on) i s a
rare condi ti on i n whi ch there i s wi despread mel ani n pi gmentati on affecti ng the oral and
geni tal mucosa and l ongi tudi nal pi gmentati on of the nai l s.
The recogni ti on of a l ocal i zed and symptoml ess mel anoti c-l i ke l esi on on the oral
mucosa may cause consi derabl e probl ems. Such a l esion may be enti rel y beni gn and stati c,
but the i ni ti al stages of mal i gnant mel anoma may have a si mi l ar appearance to that of
beni gn l esi ons. The fi rst step must be to el i mi nate the possi bi l i ty of an amal gam tattoo (see
above).
Idi opathi c mel anoti c macul es are rel ati vel y common, beni gn l esi ons of the mel anocytes,
whi ch appear as smal l brown or bl ack patches on the oral mucosa or the l i ps. Oral
mel anocyti c naevi are much l ess common and subdi vi ded i nto juncti onal ,
compound, i ntramucosal , and bl ue vari ants. Oral mel anocyti c naevi
and mel anoti c macul es shoul d be exci sed to confi rm the di agnosi s

and, i n parti cul ar, to excl ude the possi bi l i ty of a mal i gnant mel anoma. Tabl e 9. 1 summari zes
the causes of mel anoti c pi gmentati on of the oral mucosa.
Oral melanoma
Intraoral mel anomas are hi ghl y mal i gnant oral tumours that usual l y occur as i sol ated dark
brown or bl ack patches on the oral mucosa. Amel anoti c mel anomas can mani f est as red
l esi ons. Oral mel anomas are rare mal i gnanci es and tend to occur on the pal ate. In the earl y
stages they may be symptomati c. The appearance i s usual l y of a nodul ar or macul ar l esi on
Fig. 9.5 (a) Amal gam tattoo. (b) Radi ograph showi ng amal gam debri s.
P. 107
Table 9.1 Melanotic pigmentation of the oral mucosa:
principal causes
Raci al
Addi son' s di sease
HIV di sease
Reacti ve
Li chen pl anus and l eukopl aki a
Drug-rel ated
PeutzJegher syndrome
Isol ated mel anoti c l esi ons
Idi opathi c mel anoti c macul e
Mel anoti c naevus
Mal i gnant mel anoma
that i s fi rm to pal pate. Thi s ul ti matel y ul cerates and causes di scomfort or bl eeds. Earl y
di agnosi s of any l esi on suspected of bei ng a mal i gnant mel anoma i s essenti al as metastasi s
to the l ymph nodes and other organs (l ungs, l i ver, and bone) occurs earl y. The prognosi s for
oral mel anoma i s poor. Superfi ci al spreadi ng mel anomas are rarel y seen i n the mouth and
thei r appearance can be qui te spectacul ar (Fi g. 9. 6).
Earl y di agnosi s and treatment of oral mal i gnant mel anomas i s essenti al , as they metastasi ze
earl y to l ymph nodes and other organs.
Case 9.1 Discussion
An amal gam tattoo woul d be unusual at thi s si te, although the authors have seen a few such
cases. Mal i gnant mel anoma must be i ncl uded i n the di ff erenti al di agnosi s or possi bl y an earl y
Kaposi ' s sarcoma. The most l i kel y di agnosi s of thi s i sol ated l esi on i s an i di opathi c mel anoti c
macul e but exci si onal bi opsy i s i ndi cated. The bi opsy shoul d be arranged as soon as possi bl e,
parti cul arl y i n vi ew of the pati ent' s fami l y hi story.
Case 9.2 Discussion
Thi s i s l i kel y to be a denture granul oma, caused by chroni c trauma from the buccal f l ange of
thi s l ady' s i l l -fi tti ng upper denture.
The f l ange of the denture shoul d be tri mmed back so that i t i s no l onger traumati zi ng the
mucosa and the pati ent shoul d be di scouraged from weari ng her dentures at ni ght. Surgi cal
exci si on of the remai ni ng hyperpl asti c ti ssue may be necessary but shoul d not be carri ed out
unti l the response to modi f yi ng the denture has been assessed. New dentures shoul d be
Fig. 9.6 Di f fuse advanced oral mel anoma.
Q1 What i s the di fferenti al di agnosi s of thi s l esi on and how woul d you manage thi s case?
Q1 What i s the most l i kel y, cl i ni cal di agnosi s of thi s l ady' s l esi on?
Q2 How i s thi s l esi on managed?
provi ded i n the l ong term.
Carci noma of the maxi l l ary antrum may i nfi l trate the maxi l l ary bone and occasi onal l y
presents as a prol i f erati ve l esi on i n the buccal sul cus. The appearance of a ti ssue mass i n
thi s si te, wi thout any evi dence of trauma from the denture, woul d i mmedi atel y al ert the
cl i ni ci an. Pati ents wi th antral carci noma may al so present wi th symptoms such as bl ocked
nose, nasal di scharge, pai n, or paraesthesi a over the cheek. Antral carci noma can al so
present as a swel l i ng i n the pal ate, whi ch tends to ul cerate. Radi ography reveal s opaci ty of
the antrum, often wi th erosi on of the antral wal l s. If there i s any doubt whatsoever,
i mmedi ate bi opsy of the l esi on must be carri ed out. Antral carci noma has been descri bed i n
parti cul ar ri sk groups, for exampl e, workers i n the wood or shoe i ndustri es and i ndi vi dual s
who use snuf f.
Q3 A more si ni ster oral l esi on can occasi onal l y present i n a si mi l ar way. What i s thi s and
how can i t be di fferenti ated f rom the more common beni gn oral l esi on?
> Tabl e of Cont ent s > 10 - Pr ecancer ous lesi ons and condi t i ons. Oral car ci noma and car ci nogenesi s
10
Precancerous lesions and conditions. Oral
carcinoma and carcinogenesis
Problem cases
Case 10.1
A 60-year-ol d edentul ous gentl eman presents to your dental surgery for the f i rst ti me
requesti ng a new set of dentures. Hi s medi cal hi story i s cl ear but he i s a l i fe-l ong smoker,
averagi ng 2030 ci garettes per day. Hi s upper denture i s badl y stai ned wi th several teeth
ei ther fractured or mi ssi ng.
Duri ng your oral exami nati on you note an i sol ated ul cer on the l ef t l ateral border of the
tongue, whi ch i s approxi matel y 1 cm i n di ameter. Pal pati on of hi s neck reveal s no obvi ous
abnormal i ty.
Introduction
There are a number of oral l esi ons that have a potenti al for mal i gnant change. However,
rel ati vel y f ew carci nomas devel op wi thi n a recogni zabl e precancerous l esi on or i n a pati ent
wi th a precancerous condi ti on.
A precancerous l esi on i s a morphol ogi cal l y al tered ti ssue i n whi ch cancer i s more l i kel y to
occur than i n i ts apparentl y normal counterpart, that i s, the l esi on i tsel f undergoes mal i gnant
transformati on. Exampl es of precancerous oral l esi ons i ncl ude l eukopl aki a and erthyropl aki a.
A precancerous l esi on i s a morphol ogi cal l y al tered ti ssue i n whi ch cancer i s more l i kel y to
occur than i n i ts apparentl y normal counterpart.
An oral precancerous condi ti on i s a general i zed state associ ated wi th a si gni fi cantl y i ncreased
ri sk of cancer devel opi ng somewhere i n the mouth, that i s, not necessari l y i n a pre-exi sti ng
l esi on. In precancerous condi ti ons the l i nki ng factor appears to be epi thel i al atrophy such as
that whi ch occurs i n oral submucous fi brosi s and si deropeni c dysphagi a. Some authori ti es
prefer the term potenti al l y mal i gnant to precancerous because not al l precancerous
l esi ons undergo mal i gnant transformati on and a proporti on regress or stay the same.
A precancerous condi ti on i s a general i zed state associ ated wi th a si gni fi cantl y i ncreased ri sk
of cancer devel opi ng.
Q1 What questi ons woul d you ask thi s pati ent?
Q2 What f eatures of the ul cer are i mportant to note?
Q3 What i s the most l i kel y di f ferenti al di agnosi s i n thi s parti cul ar case?
Precancerous lesions
Leukoplakia
Definition of leukoplakia
Leukopl aki a i s currentl y defi ned as a predomi nantl y whi te l esi on of the oral mucosa that
cannot be characteri zed as any other defi nabl e l esioni t i s theref ore a di agnosi s of
excl usi on. Vari ous attempts have been made by the Worl d Heal th Organi sati on to defi ne oral
l eukopl aki a for use i n epi demi ol ogi cal surveys and there have been a number of revi sed
def i ni ti ons. No doubt the current defi ni ti on of l eukopl aki a wi l l change as our understandi ng of
i ts aeti ol ogy and progressi on i ncreases.
Leukopl aki a i s currentl y defi ned as a predomi nantl y whi te l esi on of the oral mucosa that
cannot be characteri zed as any other defi nabl e l esi on.
It i s i mportant to note that the term oral l eukopl aki a i s a cl i ni cal term wi th no hi stol ogi cal
connotati ons. From a cl i ni cal poi nt of vi ew, i t i s usef ul to di vi de these l esi ons i nto
homogeneous and non-homogeneous l esi ons. Non-homogeneous l eukopl aki as, i ncl udi ng those
wi th a speckl ed appearance, have a worse prognosi s wi th regard to mal i gnant
transformati on than homogeneous l esi ons. Leukopl akias i n parti cul ar si tes, such as the fl oor
of the mouth and the ventral surface of the tongue, are consi dered to be more at ri sk of
mal i gnant transformati on than those el sewhere on the oral mucosa.
Incidence of leukoplakia
The i nci dence of oral l eukopl aki a i s di ffi cul t to ascertai n but reported preval ences throughout
the worl d range from 0. 2 to 4 per cent. However, there are marked vari ati ons i n preval ence
between di f ferent geographi cal areas (Western Europe and Ameri ca) that probabl y refl ect
di f ferent ethni c and cul tural groups and aeti ol ogi cal f actors, such as smoki ng and chewi ng

tobacco. Oral l eukopl aki a used to occur predomi nantl y i n mal es and af fect ol der age groups
but gender and age rati os are now changi ng.
Aetiological factors associated with oral leukoplakia
Two groups of l eukopl aki as may be recogni zed: i di opathi c l eukopl aki as, i n whi ch no
aeti ol ogi cal factors have been recogni zed, and l eukopl aki as i n whi ch an evi dent predi sposi ng
factor may be present. It i s probabl e that the i di opathi c group may i ncl ude a certai n number
of l esi ons i n whi ch the aeti ol ogi cal f actor remai ns unrecogni zed, but, even wi th thi s
reservati on, i t woul d seem reasonabl e to accept the possi bi l i ty of the exi stence of i di opathi c
l esi ons.
It i s cl earl y establ i shed that there are a number of cl i ni cal l y si gni fi cant aeti ol ogi cal f actors
that may contri bute to the devel opment of oral l eukopl aki a. The most i mportant of these i s
the use of tobacco, ei ther when smoked or chewed i n one or other of the l arge number of
tobacco-usi ng habi ts that have been reported. The preci se nature of the acti on of tobacco or
of i ts smoke on the oral mucosa i s not yet known, but there can be no doubt that a prof ound
eff ect i s of ten exerted. Apart from the tobacco i tsel f, other substances i nvol ved i n tobacco-
chewi ng habi ts (such as betel nut and l i me) may al so be i mpl i cated i n the producti on of
l eukopl aki a. There have been many surveys concerned wi th the effect of tobacco habi ts on
the oral mucosa and, i n vi rtual l y al l cases, i t woul d appear that those i ndi vi dual s who use
tobacco i n any of i ts forms are much more l i kel y to devel op oral l eukopl aki a. It i s al so wel l
establ i shed that the preci se nature of the tobacco habi t i s of great si gni fi cance i n the
determi nati on of the exact f orm of the l esi on produced and al so of i ts eventual prognosi s. In
an i nvesti gati on of the rel ati onshi p between the i nci dence of l eukopl aki a and the sex, age,
P. 112
and tobacco habi ts of a Bombay (renamed Mumbai ) popul ati on, i t was shown qui te
cl earl y that the most i mportant extri nsi c f actor by f ar was thei r smoki ng habi t. Furthermore,
the i ncreased i nci dence of l esi ons i n mal es was di rectl y attri butabl e to thei r i ncreased use of
tobacco compared to that of femal es. It i s not di ffi cul t to extrapol ate these fi ndi ngs to other
seri es i n whi ch there has been a constant marked predomi nance of l eukopl aki a i n mal e
subjects. There i s no doubt that those l eukopl aki as that are di rectl y attri butabl e to tobacco
habi ts are, to a l arge degree, reversi bl e, and i t has been cl earl y demonstrated on many
occasi ons that regressi on of a l esi on may be i nduced by the cessati on of the habi t, al though
the regressi on may not be permanent.
Indi vi dual s who use tobacco i n any of i ts forms are much more l i kel y to devel op oral
l eukopl aki a.
A second i mportant aeti ol ogi cal f actor i n the producti on of oral l eukopl aki a i s that of
i nfecti on and, of the organi sms i nvol ved. It appears that Candi da al bi cans i s by f ar the most
i mportant. It i s wel l establ i shed (Chapter 4) that there may be a heavy i nfi l trati on of the
pseudohyphal f orm of Candi da i nto the epi thel i um of oral l eukopl aki as, and i t has been
shown that such l esi ons (candi dal l eukopl aki as) are associ ated wi th an i ncreased i nci dence of
mal i gnant transformati on. The preci se rel ati onshi p between the candi dal i nf ecti on and the
producti on of the l eukopl aki a i s not known and, i n parti cul ar, i t has not yet been determi ned
whether the i nfecti on i s a pri mary or a secondary event. Hi stol ogi cal studi es woul d seem to
provi de evi dence for ei ther vi ewpoi nt, and i t wi l l probabl y be as the resul t of i mmunol ogi cal
studi es that the fi nal deci si on as to the exact aeti ol ogi cal factor i n these cases i s made.
However, recent work has shown that, on an experi mental basi s, candi dal i nfi l trati on i nto
epi thel i um may of i tsel f produce changes resembl i ng those of l eukopl aki a, and there woul d
al so seem to be evi dence that compl ex i mmune defi ci enci es may occur i n some pati ents wi th
l eukopl aki a, i ncl udi ng a def i ci ent i mmune response to Candi da al bi cans.
There i s al so some evi dence of an addi ti ve effect between the acti ons of tobacco and Candi da
i n the formati on of l eukopl aki as. It i s currentl y suggested that heavy smokers are at
i ncreased ri sk, f i rstl y, of devel opi ng candi dal l eukopl aki a and, subsequentl y, of devel opi ng
carci noma wi thi n i t. Thi s i s not a proven associ ati on but there i s a consi derabl e bul k of
anecdotal i nformati on to support the concept. The rol e of vi ruses, such as the human
papi l l omavi rus (HPV), i n the aeti ol ogy of oral l eukopl aki as remai ns uncertai n. However, hai ry
l eukopl aki a, whi ch occurs i n HIV and other i mmunosuppressed pati ents, i s associ ated wi th
the Epstei nBarr vi rus (EBV).
Clinical features of leukoplakia
Leukopl aki a appears as an i ntri nsi c whi te area of the oral mucosa, someti mes homogeneous
(Fi g. 10. 1), someti mes wri nkl ed, and someti mes wi th a verrucous (Fi g. 10. 2) or f i ssured
surface. The whi te patch may vary from bei ng transparent and f i l my i n appearance to bei ng
dense and thi ck. In some cases, the l eukopl aki a i s a si ngl e, di screte, and wel l -ci rcumscri bed
pl aque area, whereas i n other pati ents there may be wi despread abnormal i ty of the mucosa
wi th a number of l esi ons di stri buted i n varyi ng si tes. In a Dani sh popul ati on the hi ghest
i nci dence of l eukopl aki a has been found to be on the buccal mucosa and commi ssures, and
then, i n descendi ng order of f requency, on the al veol ar ri dges, tongue, buccal sul ci , f l oor of
mouth, l abi al mucosa, and pal ate. However, i n common wi th al l other stati sti cs regardi ng
l eukopl aki a and associ ated l esi ons, i t must be remembered that the f i gures refer to a speci fi c
popul ati on and that other popul ati ons i n di fferent areas and wi th di ff erent i nfl uenci ng factors
(such as tobacco habi ts) may show di verse cl i ni cal features.
Histological features of oral leukoplakia
There i s a wi de range of hi stol ogi cal changes that can occur i n oral l eukopl aki as, and these
range f rom hyperkeratosi s wi thout epi thel i al dyspl asi a, to squamous epi thel i al dyspl asi a and
squamous cel l carci noma i n si tu. Kerati nocytes show a number of cytol ogi cal changes
(cel l ul ar atypi a) i n epi thel i al dyspl asi a. The various hi stol ogi cal changes are di scussed i n
detai l i n standard textbooks of oral pathol ogy. Not al l the i ndi vi dual cel l ul ar atypi a occurs i n
one l esi on. It i s conventi onal for oral pathol ogi sts to

subjecti vel y grade the degree of dyspl asi a as mi l d, moderate, or severe dependi ng on the
proporti on of kerati nocyte l ayers showi ng cel l ul ar atypi a.
Erythroplakia
P. 113
Fig. 10.1 Homogeneous l eukopl aki a of the fl oor of the mouth and l abi al mucosa.
Fig. 10.2 Verrucous l eukopl aki a affecti ng f l oor of mouth, alveol ar ri dge, and ventral
surface of tongue. (Hi stopathol ogy showed moderate dyspl asi a).
As i mpl i ed by i ts name, erythropl aki a i s essenti al ly a red l esi on of the mucosa. An al ternati ve
termerythropl asi ai s taken f rom the name gi ven to a somewhat si mi l ar l esi on of the
peni s (erythropl asi a of Queyrat), whi ch has f or some ti me been recogni zed as a premal i gnant
l esi on. The oral l esi on i s rel ati vel y uncommon and appears as a bri ght red patch that i s wel l
def i ned f rom the surroundi ng mucosa and has a vel vet-l i ke surface texture. There may be
occasi onal whi te areas adjoi ni ng the l esi on and, i n some cases, patches of erythropl aki a may
be i nterspersed wi th l eukopl aki c l esi ons. It i s most of ten seen on the buccal mucosa,
al though i t may occur on other si tes of the oral mucosa, such as the soft pal ate, ventral
surface of the tongue, and fl oor of the mouth.
The most i mportant feature of thi s l esi on i s the high i nci dence of cel l ul ar atypi a shown on
hi stol ogi cal exami nati on, and often thi s i s associ ated wi th atrophy of the epi thel i um. A
si gni f i cant number of these l esi ons di spl ay epi theli al atypi a throughout al l cel l ul ar
l ayersknown as carci noma i n si tu, a di agnosi s that warrants i mmedi ate surgi cal
i nterventi on. Paradoxi cal l y, i t has been poi nted out that some of these l esi ons di spl ay
vi rtual l y no atypi a and, i ndeed, may be of a si mpl e i nfl ammatory nature. It i s qui te evi dent
that bi opsy exami nati on i s essenti al i n al l these cases. As i n the case of l eukopl aki a, i t i s by
no means cl ear as to whether the Candi da i s a pri mary or a secondary feature of such
l esi ons. However, i t i s general l y fel t (perhaps wi thout any cl ear evi dence) that the presence
of Candi da i n the epi thel i um i s l i kel y to coi nci de wi th an i ncreased tendency to mal i gnant
change. Whatever the aeti ol ogi cal f actors i nvol ved, there seems l i ttl e doubt that a hi gh
proporti on of l esi ons cl i ni cal l y di agnosed as erythropl aki a present suff i ci ent epi thel i al
abnormal i ti es to be cl assi f i ed on hi stol ogi cal exami nati on as i nci pi entl y mal i gnant. It woul d
seem that erythropl aki a warrants the descri pti on that has been appl i ed to i t of bei ng the
most seri ous of the oral precancerous l esi ons.
Speckled leukoplakia
Thi s l esi on can be consi dered as a vari ant of ei ther l eukopl aki a or erythropl aki a. It appears
as a seri es of whi te nodul ar patches on an erythematous background and so, i n appearance,
i s mi dway between these two condi ti ons. Any area of the oral mucosa may be i nvol ved, but
many l esi ons appear on the buccal mucosa near to the commi ssures (Fi g. 10. 3).
Hi stol ogi cal l y, these l esi ons show a hi gh i nci dence of atypi a and, i n many cases, the
presence of candi dal hyphae i n the epi thel i um. The same doubts have been expressed about
the exact rol e of Candi da i n these l esi ons as i n the case of homogeneous l eukopl aki as, but
there i s no doubt that there are f requentl y two coinci dental factors i n al l these l esi onsthe
presence of Candi da and the presence of epi thel i al atypi a. Whether there i s a causal effect
remai ns to be determi ned. The l esi ons shoul d al ways be consi dered as potenti al l y mal i gnant.
Al though accurate i nf ormati on i s not as yet avai l abl e, al l avai l abl e surveys suggest that
speckl ed l eukopl aki as have a si gni f i cantl y hi gher rate of mal i gnant transf ormati on than
homogeneous l esi ons.

Candidal leukoplakia
Candi dal l eukopl aki a i s al so known as chroni c hyperpl asti c candi dosi s. The i mportant rol e of
Candi da as an aeti ol ogi cal factor i n the producti on of oral l eukopl aki a has al ready been
di scussed. It i s not easy to ascri be a characteri sti c appearance to candi dal l eukopl aki as and,
i ndeed, a wi de vari ety of l esi ons may be shown to incl ude the organi sms. However, i t i s
general l y accepted that candi dal l eukopl aki as are frequentl y speckl ed and of ten have a
somewhat i rregul ar and nodul ar appearance. A hi gh proporti on of commi ssural l esi ons
extendi ng to the external angl e of the l i ps are candi dal l eukopl aki as (Fi g. 10. 3). Di agnosi s of
a candi dal l eukopl aki a cannot be made by taki ng superfi ci al swabsthe hyphae are wi thi n
the l esi on and very few may be on the surf ace. For a rel i abl e di agnosi s i t i s necessary to
stai n secti ons from a bi opsy speci men wi th peri odi c aci d Schi f f (PAS) reagents. In thi s way
the hyphae are readi l y seen wi thi n the superf i ci al l ayers and stratum corneum of the
epi thel i um. In vi ew of the i ncreased rate of mal i gnant change i n candi dal l eukopl aki as, i t i s
of evi dent i mportance that these l esi ons shoul d be recogni zed and regarded wi th hei ghtened
suspi ci on.
Malignant transformation of precancerous lesions
Oral l eukopl aki a i s a marker of an i ncreased ri sk of cancer anywhere i n the oral cavi ty, but to
date there are no rel i abl e cl i ni cal or hi stol ogi cal features that can be used to predi ct whether
the l esi on wi l l regress spontaneousl y, remai n the same, or progress to cancer. It has been
reported that mal i gnant transformati on occurs i n between 4 and 8 per cent of oral
l eukopl aki as and about 15 per cent wi l l spontaneousl y regress. The si te of i nvol vement i s
i mportant and i n subl i ngual l eukopl aki as the rate of transformati on i s as hi gh as 40 per cent.
Erythropl aki a has the greatest tendency to devel op i nto a mal i gnant l esi on, wi th a
Fig. 10.3 Candi dal speckl ed l eukopl aki a at the commi ssurea characteri sti c si te.
P. 114
transformati on rate as hi gh as 80 per cent and above.
The management of precancerous lesions
It i s not wi thi n the remi t of thi s book to deal i n any detai l wi th the surgi cal treatment of
l eukopl aki as. It shoul d be poi nted out, however, that one of the probl ems i n therapy l i es i n
accuratel y defi ni ng the area at ri sk. It i s evi dent that the oral mucosa as a whol e must, to
some extent, be exposed to the parti cul ar aeti ol ogical agent, whether the l atter be i ntri nsi c
or extri nsi c. Thus, i t i s often di f fi cul t to come to an arbi trary deci si on about the l i mi ts of
surgi cal or l aser exci si on necessary for opti mum treatment. It i s by no means uncommon f or
an apparentl y generous exci si on to prove i nadequate or for enti rel y new f i el ds of abnormal i ty
to appear. Indeed, i t i s cl ear that l eukopl aki a often represents more than a l ocal i zed
abnormal i ty and that the whol e of the mucosa may be i nvol ved, even though l ocal i zed l esi ons
may be the expressi on of thi s. Si mi l arl y, the recurrence of l esi ons repai red by mucosal grafts
or al l owed to epi thel i al i ze can be easi l y expl ai ned by the real i zati on that such a manoeuvre i s
eff ecti ve onl y i n removi ng speci fi c epi thel i al cel ls that have become i nvol ved i n a compl ex
abnormal i ty. If the pre-exi sti ng aeti ol ogi cal factors remai n, then recurrent abnormal i ty may
be expected even though the aeti ol ogy may remai n unrecogni zed. It may wel l be, i n
recurrent cases of thi s nature, that abnormal mesodermal epi thel i al reacti ons are
responsi bl e for the i ni ti al l esi on and are mai ntai ned by the presence of the i nfl uenci ng cori um
after surgery. There i s i ncreasi ng doubt regardi ng the recurrence of cryosurgi cal l y treated
l eukopl aki as as carci noma, even though i t seems that some degree of undertreatment mi ght
be i nvol ved i n such cases. Currentl y, exci si on of the l esi ons usi ng a CO
2
l aser i s fel t to be a
safer al ternati ve. Certai nl y, thi s techni que greatly reduces the necessi ty for grafti ng of
exci sed areas, there i s l i ttl e ti ssue di storti on fol l owi ng re-epi thel i al i zati on, and, i n terms of
postoperati ve di scomf ort, i t i s greatl y pref erabl e to cryotherapy.
Apart from surgi cal treatment i t i s possi bl e to obtai n a marked regressi on of a si gni f i cant
number of tobacco-i nduced l eukopl aki as by di sconti nui ng the habi t. Bi opsy studi es of such
regressi on l esi ons have shown marked reducti ons i n the i nci dence of atypi a as wel l as i n the
extent of the l esi ons. Thi s manoeuvre shoul d be the fi rst empl oyed i n the management of
l esi ons i n whi ch a tobacco-medi ated aeti ol ogy i s suspected. Si mi l arl y, i n those l esi ons wi th
associ ated candi dal i nfecti ons, i t i s often possi bl e to ef fect a marked i mprovement by the use
of systemi c anti fungal s. It i s unl i kel y that a compl ete regressi on wi l l be obtai ned i n thi s way,
but the reducti on i n the si ze of the l esi on may make the eventual surgi cal management much
si mpl er. The devel opment of saf er systemi c anti f ungal agents, such as fl uconazol e, has
faci l i tated thei r use i n the routi ne management of candi dal l eukopl aki a.
Leukopl aki as wi th l i ttl e or no dyspl asi a are often l ef t untreated, parti cul arl y i f pati ents are
abl e to reduce or stop smoki ng or chewi ng tobacco. Lesi ons are moni tored on a l ong-term
basi s, both cl i ni cal l y and hi stol ogi cal l y, as appropri ate. Thi s pol i cy of watchf ul
wai ti ng i s cl earl y unsati sf actory and rel i es on the experi ence and subjecti ve assessment
of the cl i ni ci an. The key to managi ng oral l eukopl aki a i s l i kel y to be the establ i shment of
mol ecul ar assays that can redefi ne the assessment of the ri sk of mal i gnant transformati on
and hopeful l y l ead to successf ul methods of chemopreventi on, such as the use of reti noi ds.
Mol ecul ar markers are di scussed i n the next secti on of thi s chapter.
Precancerous conditions
Oral submucous fibrosis (OSF)
Thi s i s a di sease that was fi rst recogni zed i n the 1950s, despi te presumpti ve evi dence that i t
must have been present l ong before that ti me. The vast majori ty of cases have been f ound i n
the Indi an subconti nent, al though si mi l ar exampl es have been reported i n other Asi ati c
countri es as wel l as an i ncreasi ng number of pati ents i n the UK.
Oral submucous fi brosi s (OSF) i s a condi ti on i n which f i brous ti ssue i s l ai d down i n the
cori um of the oral mucosa.

Si mul taneous changes occur i n the oral epi thel i um. In the earl y phase vesi cl es and smal l
ul cers may be f ormed, but thi s stage i s soon superseded by one of general i zed epi thel i al
atrophy. The ef fect of the f i brosi s i s a sti ff eni ng of the oral mucosa l eadi ng to di f fi cul ty i n
openi ng the mouth and to a bi ndi ng down of the tongue. The appearance of the mucosa i s of
a bl anched, marbl ed nature, whi ch seems to be qui te characteri sti c of the condi ti on.
It i s possi bl e to pal pate bands of f i brous ti ssue wi thi n the mucosa and i t i s reported that
eventual l y the scar formati on wi thi n the soft pal ate i s suff i ci ent to cause the near
di sappearance of the uvul a.
The cause of OSF i s not known wi th certai nty, but recent work suggests that both a geneti c
suscepti bi l i ty and a f i brobl asti c response to areca (betel ) nut chewi ng may be i nvol ved.
Epi demi ol ogi cal studi es i ndi cate that thi s condi ti on i s i nduced by chewi ng areca nut and i t i s
theref ore parti cul arl y common i n Asi an communi ti es. Studi es i ndi cate there i s probabl y a
geneti c predi sposi ti on for OSF and the rol e of autoi mmuni ty i s al so bei ng i nvesti gated. The
i nfl uence of nutri ti onal factors, i f any, remai ns uncl ear. Pati ents wi th OSF have an i ncreased
ri sk of devel opi ng oral carci noma, whi ch has been esti mated to be as hi gh as 10 per cent
over 10 to 15 years. So far, there does not seem to be any sati sfactory treatment for OSF
al though i ntral esi onal steroi ds have been used. Primary preventi on by reduci ng the use of
areca nut products woul d appear to be the best way forward to reduce the i nci dence of OSF.
A condi ti on, know as betel -chewers mucosa has been descri bed i n whi ch there i s
browni sh-red di scol orati on of the oral mucosa (parti cul arl y the buccal mucosa) together wi th
desquamati on or peel i ng of the mucosa. The l atter may be partl y due to trauma f rom
chewi ng. In addi ti on, the mucosa often has a wri nkled appearance. Thi s i s not per se
consi dered to be a precancerous l esi on but may progress to oral submucous f i brosi s and/or
oral l eukopl aki a. Pati ents are of ten rel uctant to stop chewi ng qui d, al though thi s i s the key
to successf ul management.
Sideropenic dysphagia
Si deropeni c dysphagi a i s al so known as the PatersonKel l y (or Pl ummerVi nson)
syndrome and predomi nantl y aff ects mi ddl e-aged femal es who have i ron defi ci ency (see al so
Chapter 13). The oral and pharyngeal mucosa may appear atrophi c and shi ny red. Oral
l eukopl aki as and mul ti pl e squamous cel l carci nomas can devel op i n thi s condi ti on but i t i s
parti cul arl y associ ated wi th postcri coi d carci noma.
Lichen planus
The premal i gnant potenti al of oral l i chen pl anus i s di scussed i n Chapter 11 and the mal i gnant
transformati on rate i s ci ted as 0.43. 3 per cent. Whether or not pati ents wi th atrophi c or
erosi ve f orms of oral l i chen pl anus are more suscepti bl e to mal i gnant change has yet to be
proved by l ong-term prospecti ve studi es.
Other precancerous conditions
Di scoi d l upus erythematosus has been cl assi fi ed as a premal i gnant condi ti on but onl y a few
cases of mal i gnant transformati on of l i p l esi ons have been reported. Terti ary syphi l i s i s
rarel y reported these days because of earl y recogni ti on and treatment. In the past, oral
l eukopl aki as and squamous cel l carci nomas devel oped i n associ ati on wi th the atrophi c
gl ossi ti s of terti ary syphi l i s but the use of potenti al l y carci nogeni c agents such as arseni c to
treat thi s condi ti on may have predi sposed to thi s transf ormati on. Other rare condi ti ons that
have oral mani f estati ons have been reported as precancerous and i ncl ude xeroderma
pi gmentosum and epi dermol ysi s bul l osa. Overal l , condi ti ons i n whi ch there i s epi thel i al
atrophy appear to be associ ated wi th an i ncreased ri sk of mal i gnant transformati on.
P. 115
Oral carcinoma and carcinogenesis
Mal i gnant tumours of the head and neck i ncl ude squamous cel l carci noma (SCC) of the oral
cavi ty, l arynx, and pharynx, sal i vary/gl andul ar cancers, mal i gnant mel anomas, l ymphomas,
and sarcomas. SCC i s the most common neopl asm of the head and neck and accounts for
more than 90 per cent of al l oral mal i gnanci es. Worl dwi de, the annual i nci dence of SCC
exceeds 300 000 wi th approxi matel y 2000 new cases bei ng regi stered per year i n the UK.
Each year nearl y hal f that number di e from oral SCC (OSCC). The hi gh morbi di ty rate i s due
to a number of factors i ncl udi ng l ate presentati on, fai l ure to respond to treatment regi mens
currentl y avai l abl e, and a l ack of sui tabl e markers for earl y detecti on. The dental professi on
has a cruci al rol e i n the earl y detecti on of SCC, whi ch, i f treated earl y, has the best
prognosi s. Current approaches for control l i ng thi s cancer i ncl ude i mproved preventi on (ri sk
factors such as tobacco are wel l recogni zed) and earl y detecti on of pati ents wi th suspi ci ous
oral l esi ons.
Aetiological factors for oral squamous cell carcinoma
(OSCC)
The aeti ol ogi cal factors i mpl i cated i n OSCC are tobacco use, al cohol consumpti on, sunl i ght
(cancer of the l i p), di et and nutri ti onal status, chroni c candi dal i nfecti ons, vi ral i nf ecti ons,
and i mmune defi ci ency. Of these, tobacco use and alcohol are consi dered to be the most
i mportant. Consi derati on of al l these factors i s cl earl y outsi de the scope of thi s book but the
rol e of tobacco, al cohol consumpti on, and di et wi l l be bri efl y consi dered. The potenti al rol e of
vi rus i n oral carci nogenesi s i s di scussed i n a l ater secti on of thi s chapter.
The specul ati ve rel ati onshi p between Candi da speci es and mal i gnant transformati on of oral
l eukopl aki a has al ready been di scussed i n Chapter 4.
Tobacco use
Tobacco use, i n any of i ts forms (ci garettes, ci gars, pi pe-smoki ng, tobacco-chewi ng, reverse
smoki ng), i s one of the most i mportant aeti ol ogi cal f actors i n the devel opment of OSCC. The
rel ati ve ri sk

of devel opi ng oral carci noma from ci garette-smoki ng depends on a number of factors,
i ncl udi ng l evel of consumpti on and whether the ci garettes are hi gh or l ow tar. However, a
reasonabl e esti mate i s that an i ndi vi dual who smokes more than 20 ci garettes a day has a
ri sk of devel opi ng oral carci noma 10 ti mes hi gher than that of a nonsmoker. Pi pe- and ci gar-
smoki ng have been l i nked wi th cancer of the l i p. In one regi on of Indi a, where reverse
cheroot smoki ng i s practi sed amongst women, a greatl y i ncreased i nci dence of carci noma of
the pal ate i n femal e pati ents has been reported. Reverse smoki ng i s al so common i n other
parts of the worl d. The i ncreased i nci dence of oral cancer i n Indi a i s l i kel y to be due to the
common practi ce of smoki ng bi di sa cheap type of ci garette (made from l ocal tobacco
rol l ed i n a l eaf)and reverse smoki ng.
The chewi ng of betel qui d i s endemi c throughout the Indi an subconti nent, South-east Asi a,
and l arge parts of the western Paci fi c. Onl y three substances, ni coti ne, ethanol , and caffei ne,
are consumed more wi del y than betel . Qui d consumption i s hi gher i n femal es and there i s
consi derabl e regi onal vari ati on i n i ts consti tutents. Qui d has been defi ned as a substance
or mi xture of substances pl aced i n the mouth or chewed and remai ni ng i n contact wi th the
mucosa, usual l y contai ni ng one or both of the two basi c i ngredi ents, tobacco or areca nut, i n
raw or any manufactured or processed f orm. Ingredi ents commonl y used i n the
preparati on of betel qui d are l i sted i n Tabl e 10. 1. Pan masal a has al l the i ngredi ents of the
betel qui d, except the betel l eaf, and i s conveni entl y packaged i n smal l sachets and ti ns (Fi g
10. 4).
A number of case control studi es have reported an increased rel ati ve ri sk of devel opi ng
P. 116
cancers of the oral cavi ty due to the use of betel qui d, wi th or wi thout tobacco. Oral
submucous f i brosi s and oral l eukopl aki a, both consi dered to be premal i gnant, are associ ated
wi th betel -chewi ng (see Precancerous l esi ons and Precancerous condi ti ons).
It i s reported that thi s habi t i mproves di gesti on and sal i vati on, di mi ni shes hunger pangs, and
produces a f eel i ng of euphori a, and some cl ai m i t has aphrodi si ac powers. Chi l dren as young
as 3 years of age parti ci pate i n thi s practi ce. It i s an essenti al el ement i n soci al , cul tural ,
and economi c l i f e i n many parts of the worl d.
Chewi ng of betel qui d i s associ ated wi th oral l eukopl aki a, oral submucous fi brosi s, and
squamous cel l carci noma.
Tobacco-chewi ng i s tradi ti onal l y practi sed by mi ners, as smoki ng i s obvi ousl y dangerous
underground. Mi ners who adopt thi s practi ce are, theref ore, suscepti bl e to devel opi ng oral
l eukopl aki a, whi ch may undergo mal i gnant transformati on.
It i s i mportant to appreci ate that tobacco usage i s not onl y an i mportant ri sk f actor f or oral
carci noma but a common aeti ol ogi cal f actor of oral l eukopl aki a (see above).
Alcohol consumption
Al cohol consumpti on as a ri sk factor for oral carcinoma i s di ff i cul t to quanti fy, parti cul arl y as
many pati ents who dri nk heavi l y al so have a hi gh consumpti on of ci garettes. The general
consensus, however, i s that al cohol i s an i ndependent ri sk factor but the effect of al cohol
and tobacco together i s mul ti pl i cati ve, that i s, greater than the added ri sk of al cohol or
tobacco.
Table 10.1 Ingredients commonly used in the
preparation of betel quid
Betel l eaf (al so known as pan)
Areca nut (supari )seed of areca catechu tree
Li me (cal ci um hydroxi de)
Catechu (resi nous extract f rom acaci a tree)
Tobacco
Diet and nutritional status
The rol e of di et and nutri ti onal status i n the preventi on of oral cancer has been the subject
of a number of studi es and i t i s general l y consi dered that def i ci enci es of vi tami ns A, C, and E
may predi spose to the devel opment of oral carci noma. Lack of di etary i ron may l ead to
aerodi gesti ve (i ncl udi ng oral ) carci nomas as i s i n the Pl ummerVi nson syndrome. Hi gh
consumpti on of f rui t and fresh vegetabl es may l ower the ri sk, and recent studi es suggest

that pati ents wi th head and neck cancers have a hi gh f at and red meat i ntake.
Clinical features and diagnosis of oral squamous cell
carcinoma
Fig. 10.4 Pan masal a, on sal e to the publ i c.
P. 117
Not al l oral carci nomas are preceded by a recogni zabl e premal i gnant l esi on, al though i n
many cases evi dent abnormal i ty may have been present f or some ti me (Tabl e 10. 2). The
si gni f i cance of l eukopl aki a as a premal i gnant l esi on has been di scussed above but i t must be
repeated that the cl i ni cal cri teri a by whi ch a whi te patch may be judged as premal i gnant are
far from cl ear, and that i t i s onl y after a study of the hi stol ogi cal appearance of the l esi on
that any attempt at prognosi s can be made.
The cl assi cal si gns of a mal i gnant condi ti on, whi ch shoul d arouse i mmedi ate suspi ci on, are:
1. persi stent ul cerati on: any unexpl ai ned ul cer that l asts for l onger than 10 days shoul d
be treated wi th suspi ci on;
2. i ndurati on: thi ckeni ng and hardeni ng of the ti ssues;
3. prol i ferati ve growth of ti ssue above i ts normal level , often wi th changes i n the surface
texture and col our changes;
4. fi xati on of the af fected ti ssue to the underl yi ng structures.
Pai n i s not al ways present i n the earl y stages of a carci noma and the pati ent may be qui te
unaware of any abnormal i ty unti l the l esi on has become l arge and secondari l y i nfected. If the
aff ected area i ncl udes the teeth, these may become mobi l e due to the repl acement of the
peri odontal membrane by tumour. Thus, unexpl ai ned rapi d l ooseni ng of the teeth shoul d be
caref ul l y i nvesti gated.
Lymph node i nvol vement may occur earl y i n oral carci noma. However, enl arged regi onal
l ymph nodes may i n fact show onl y non-speci f i c i nf lammatory changes i n some earl y cases.
Unfortunatel y, thi s cannot be rel i ed on and, i n some cases, mal i gnant deposi ts i n the l ymph
nodes are found whi l e the pri mary l esi on i s sti l l smal l . It i s certai nl y true that prognosi s i s
more favourabl e i f treatment i s i nsti tuted before the l ymph nodes are i nvol ved.
Lesi ons showi ng the si gns outl i ned above are i mmediatel y suspect but such a l esi on i s at a
rel ati vel y l ate stage (Fi g. 10. 5). In i ts earl y stages, a carci noma may show none of these
si gns and may be detectabl e onl y by a change i n col our or surf ace texture of the mucosa. It
may be i mpossi bl e to di fferenti ate cl i ni cal l y between l eukopl aki a or erythropl aki a and earl y
carci noma and, thus, bi opsy exami nati on i s mandatory i n every case i n whi ch the possi bi l i ty
of carci noma may ari se. Any prol onged ul cer, erythema, or unexpl ai ned whi te patch shoul d
be i nvesti gated i n thi s way. A di agnosi s of carci noma shoul d be fol l owed as rapi dl y as
possi bl e by treatment and thus i t i s essenti al that the surgeon who carri es out bi opsy
procedures shoul d be i n a posi ti on to arrange for immedi ate treatment i f requi red. For thi s
reason, i t i s of ten better for such i nvesti gati on to be carri ed out i n speci al i st centres rather
than i n the dental practi ti oner' s surgery. Prognosis i n l ate oral carci noma i s poor and much
depends on the earl y detecti on of the l esi on bef ore the cervi cal nodes become i nvol ved, the
overal l 5-year survi val rate bei ng effecti vel y doubl ed i f the l esi on i s di scovered earl y. It i s
al so true that the prognosi s becomes worse the further back i n the oral cavi ty the l esi on l i es.
Thus, i t i s qui te cl ear that the pati ent' s survi val may depend on a thorough systemati c
exami nati on of the oral mucosa. The use of tol ui di ne bl ue dye has been advocated as an
adjunct to the cl i ni cal di agnosi s of suspi ci ous oral l esi ons. Products such as OraTest are
now approved i n the UK for oral screeni ng, detecti on of second pri mary l esi ons, and defi ni ng
margi ns of l esi ons for bi opsy and surgery and are the subject of ongoi ng cl i ni cal tri al s.
Suspi ci ous oral l esi ons shoul d al ways be bi opsi ed regardl ess of the resul ts of tol ui di ne bl ue
stai ni ng.
Table 10.2 Oral carcinoma: suspicious clinical features
Carci noma of the l i p i s a rather di fferent l esi on i n that i t i s cl earl y vi si bl e and so i s noti ced
earl y. It has a much better prognosi s than i ntraoral carci noma and earl y di agnosi s often
i mpl i es successful and rel ati vel y smal l -scal e surgery. The l ower l i p i s practi cal l y al ways
aff ected, the pati ents bei ng predomi nantl y ol der mal es (Fi g. 10. 6). The carci noma i s often
mi staken i n i ts earl y stages for a herpeti c l esi on, but i ts persi stent nature shoul d arouse
suspi ci on and the same cri teri a f or i nvesti gati on shoul d be adopted as for i ntraoral l esi ons.
Pati ents wi th acti ni c chei l i ti s are at i ncreased ri sk of devel opi ng carci noma of the l i p (see
Chapter 6).

Staging systems for oral carcinoma
In order to ai d uni formi ty i n studi es of the epi demi ol ogy of oral carci noma and the prognosi s
of those suf feri ng from i t, there have been a number of assessment methods i ntroduced.
These are general l y known as stagi ng systems. The most wi del y used i s the TNM system
(tumour, nodes, metastasi s). In thi s system scores are gi ven to each pati ent dependent on
the si ze of the pri mary tumour, the extent of regi onal l ymph node i nvol vement, and the
Persi stent, unexpl ai ned ul cerati on
Indurati on
Prol i ferati ve growth
Changes i n texture and col our
Fi xati on to underl yi ng ti ssues
Sudden l ooseni ng of teeth
Lymph node i nvol vement
Pai nof ten a l ate feature
Fig. 10.5 Carci noma of the pal atal mucosa.
P. 118
presence of di stant metastases. Stagi ng systems have been found of consi derabl e val ue i n
sel ecti ng the most appropri ate management and assessi ng the overal l prognosi s of pati ents
i n each group.
The management of oral carcinoma
The management of oral carci noma shoul d be coordi nated by a mul ti di sci pl i nary team,
i ncl udi ng maxi l l ofaci al surgeons, oncol ogi sts, radi otherapi sts, speech therapi sts, dedi cated
oncol ogy nurses, and other personnel i nvol ved i n rehabi l i tati on. In some centres, a cl i ni cal
psychol ogi st may al so be i nvol ved i n the assessment and rehabi l i tati on of pati ents
undergoi ng treatment for oral cancer. Speci al i sts in pal l i ati ve care may al so be i nvol ved i n
the management of these pati ents who have advanced di sease or for whom treatment has
proved unsuccessful . The treatment of choi ce depends on a number of f actors i ncl udi ng
pati ent preference, bi ol ogi cal age, general heal th, and si te and stagi ng of the tumour. Over
the l ast 2 decades, there have been great advances i n the reconstructi on of pati ents who
have undergone surgi cal treatment for oral carci noma and these have l ed to a greatl y
i mproved qual i ty of l i f e.
The management of oral cancer shoul d be coordi nated by a mul ti di sci pl i nary team.
The treatment of oral carci noma i s cl earl y a compl ex matter, whi ch i n the majori ty of cases
i nvol ves surgery (wi th reconstructi on), radi otherapy, or a combi nati on of both.
Chemotherapy i s not routi nel y used for the treatment of oral carci noma at the present ti me.
Management regi mens for carci noma must focus on l ongevi ty and qual i ty of l i fe.
Management regi mens for cancer must focus on l ongevi ty and qual i ty of l i fe.
Radiation mucositis
Duri ng the course of radi otherapy (whi ch i s l i kel y to l ast for several weeks) the pati ent
devel ops a progressi ve and general i zed erythematous and ul cerati ve response of the oral
mucosa (radi ati on mucosi ti s) (see Tabl e 10. 3). Radi ati on mucosi ti s general l y starts about the
Fig. 10.6 Carci noma of the l ower l i p.
second week and may be accompani ed by a compl ai nt about an al tered taste. It i s extremel y
pai nful , the probl em bei ng compl i cated by the fact that the sal i vary gl ands are al so vi rtual l y
al ways aff ected, wi th consequent l esseni ng of sal i vary secreti on and xerostomi a. Secondary
candi dosi s al most i nvari abl y occurs, and Gram-negati ve organi sms may pl ay a si gni fi cant
rol e. Fol l owi ng the course of radi ati on the mucosi ti s gradual l y subsi des l eavi ng an atrophi c
oral epi thel i um on a rel ati vel y avascul ar submucosa. The sal i vary fl ow may be permanentl y
reduced and the pati ent wi l l devel op si gns and symptoms rel ated to sal i vary gl and
dysfuncti on (see Chapter 8). In the l ong term, radiati on therapy may resul t i n tri smus due to
ti ssue fi brosi s.
In the acute phase, si mpl e non-astri ngent mouthwashes (for exampl e, sodi um bi carbonate or
tabl e sal t) may have a soothi ng ef fect on the oral mucosa. Benzydami ne hydrochl ori de
mouthwash has anti -i nfl ammatory, and anti mi crobi al properti es and may reduce the severi ty
of the mucosi ti s. Mi conazol e gel may be used to combat candi dosi s. Pati ents may onl y be
abl e to tol erate a soft, bl and di et. In the l ong term, the combi nati on of dry mouth and an
atrophi c mucosa may cause conti nui ng di scomf ort for whi ch arti fi ci al sal i vas (and sal i va
repl acement gel s) are the onl y avai l abl e treatment, together wi th benzydami ne hydrochl ori de
ri nse. Those contai ni ng added fl uori de mi ght wel l be consi dered. These pati ents become
unusual l y suscepti bl e to cari es agai nst whi ch topi cal fl uori des may be effecti ve. Pati ents
shoul d be encouraged to mai ntai n thei r oral hygi ene as much as possi bl e. Radi ati on cari es
shoul d be managed by a combi nati on of oral hygi ene procedures, restorati on of earl y l esi ons,
and the appl i cati on of topi cal fl uori des. The management of sal i vary gl and hypofuncti on has
been di scussed i n Chapter 8.
Recent studi es have shown that sel ecti ve decontami nati on of oral fl ora wi th PTA (pol ymyxi n
E, tobramyci n, and amphoteri ci n) l ozenges reduces the durati on and degree of mucosi ti s i n
pati ents i rradi ated for oral carci nomathi s should be started before and duri ng
radi otherapy (Tabl e 10. 4).
Osteoradionecrosis
Radi otherapy f or oral mal i gnancy al so af fects the vascul ari ty of the bone, whi ch becomes
nonvi tal . It i s, therefore, hi ghl y suscepti bl e to i nfecti on and the effects of trauma.
Osteomyel i ti s occurs as a consequence of i nfecti on and resul ts i n a pai nful necrosi s
(osteoradi onecrosi s) wi th sl oughi ng of the overl yi ng sof t ti ssues. Modern methods of
radi otherapy have reduced thi s compl i cati on. However, extracti ons i n previ ousl y i rradi ated
bone must be carri ed out wi th great care and consi derati on shoul d be gi ven to

anti bi oti c cover. Hyperbari c oxygen therapy i s now bei ng i ncreasi ngl y used to manage these
pati ents. It appears that osteoradi onecrosi s after extracti ons i s more l i kel y when a l onger
ti me has el apsed si nce radi otherapy (Tabl e 10. 5).
P. 119
Table 10.3 Oral complications of radiotherapy
Early onset Later onset
Mucosi ti s Radi ati on cari es
Al tered taste Osteoradi onecrosi s
Xerostomi a Tri smus (fi brosi s of muscl es and other soft ti ssues)
Secondary i nf ecti ons
Demi neral i zati on of teeth
Hypersensi ti vi ty of teeth

Table 10.4 Prevention and management of radiation
mucositis
Mai ntenance of oral hygi ene
Sel ecti ve decontami nati on (e. g. l ozenges contai ni ng pol ymyxi n E, tobramyci n, and
amphoteri ci n)
Di etary advi ce (bl and, non-i rri tant f oods and avoi d al cohol )
Bl and mouthwashes (e. g. sal t/bi carbonate of soda)
Benzydami ne hydrochl ori de (topi cal use)
Mi conazol e gel (oral candi dosi s)
Topi cal steroi ds*
Management of sal i vary gl and hypofuncti on (Chapter 8)
* May predi spose to oral candi dosi s.
The role of the dental team in the management of patients
diagnosed with oral carcinoma
Cl i ni cal gui del i nes (see project 1 at the end of chapter) recommend that oncol ogy protocol s
for pati ents di agnosed wi th oral carci noma shoul d incl ude an earl y pretreatment oral
assessment and the organi zati on of oral care, i ncl udi ng any dental treatment requi red.
Ideal l y, a hospi tal denti st shoul d be an i ntegral part of the oncol ogy team and can l i ai se wi th
the pati ent' s own denti st to organi ze dental treatment pri or to, duri ng, and af ter therapy for
oral carci noma. Any prophyl acti c requi rements need to be i nsti tuted pri or to radi ati on
therapy. Essenti al dental procedures al so need to be carri ed out as soon as possi bl e, so not
to del ay cancer therapy. Pati ents who have undergone radi otherapy may be at l i f el ong ri sk
for oral di sease, parti cul arl y dental cari es and peri odontal di sease.
Input from a speci al i st prosthodonti st i s requi red for those pati ents who have undergone
abl ati ve procedures i nvol vi ng reconstructi on. Endosseous i mpl ants may be used to retai n
bri dges or dentures. The dental hygi eni st has an i mportant rol e i n assi sti ng the pati ent wi th
di etary advi ce, the mai ntenance of oral hygi ene, and appl i cati on of topi cal fl uori des. Pati ents
usual l y need to be moni tored by the dental team at 2- or 3-monthl y i nterval s after
radi otherapy.
Prevention of oral carcinoma
Thi s can be di vi ded i nto pri mary, secondary, and terti ary preventi on. The dental surgeon has
a pi votal rol e i n preventi ve aspects of oral carci noma and i s i n an i deal posi ti on to gi ve
advi ce concerni ng l i festyl e (for exampl e, tobacco cessati on and di etary advi ce) and to
i denti fy hi gh-ri sk oral l esi ons (see Tabl e 10. 6).
Oral carcinoma as a genetic disease
Oral squamous cel l carci nomas and those at other si tes i n the head and neck ari se as a
consequence of mul ti pl e mol ecul ar events. These are i nduced by the ef fects of vari ous
carci nogens from habi ts such as tobacco and i nf l uenced by envi ronmental factors, possi bl y
vi ruses i n some i nstances, agai nst a background of heri tabl e resi stance or suscepti bi l i ty.
Human cancers

devel op by accumul ati ng a range of somati c geneti c changes throughout thei r progressi on;
Table 10.5 Prevention and management of
osteoradionecrosis
Arrange extracti ons before radi otherapy commences
Avoi d trauma to oral mucosa
Conserve teeth postradi otherapyi f possi bl e
Extracti ons shoul d be atraumati c and under anti bi oti c cover
Ri nse wi th 0. 2% chl orhexi di ne, pri or to extracti on
Consi der the use of hyperbari c oxygen
P. 120
however, the mol ecul ar basi s f or these changes remai ns uncl ear i n the majori ty of cancers. It
has been postul ated that geneti c damage af fecti ng the majori ty of autosomal chromosome
arms, i ncl udi ng acti vati on of oncogenes and/or i nacti vati on of tumour suppressor genes
(TSGs) possi bl y worki ng i n conjuncti on wi th an i mpai red capaci ty of DNA repai r mechani sms,
may be a factor i n the devel opment of squamous cel l carci noma of the head and neck. It i s
general l y accepted that there are three mai n cl asses of genes i nvol ved i n
tumourgenesi sTSGs, oncogenes, and DNA repai r genesal though di sti ncti ons between
the parti cul ar genes and thei r properti es, and thei r cl assi fi cati on wi thi n a certai n group, are
subject to controversy. Oncogenes i ncl udes growth factors, growth factor receptors, si gnal
transducers, and nucl ear protei ns. However, mutati ons, ampl i f i cati on, or rearrangement of
proto-oncogenes may resul t i n the acti vati on of oncogenes, whi ch l eads to mal i gnant
transformati on. The range of oncogenes that have been studi ed i n oral carci noma (f or
exampl e, myc and ras gene fami l i es and erB-1) and acti vated oncogenes produce ei ther
abnormal (mutated) protei ns or aberrant expressi on of protei ns at an i nappropri ate stage i n
the cel l cycl e. A second group of genes i s i nvol ved i n mal i gnant transformati on, TSGs. One of
the most studi ed TSGs i s p53, whi ch i s l ocated on chromosome 17p. The p53 gene codes for
a 53 kDa nucl ear phosphoprotei n that hol ds a key posi ti on i n the compl ex network control l i ng
genome stabi l i ty, cel l cycl e, and apoptosi s. p53 acts as a TSG by arresti ng cel l s carryi ng DNA
damage i n the G
0
/G
1
phase, provi di ng adequate ti me for the cel l ' s DNA repai r mechani sm to
functi on and, i f unsuccessf ul , l eads cel l s to apoptoti c death. p53 mutati ons have been
previ ousl y reported i n many cancer studi es i ncl udi ng oral cancer and are consi dered to be
the most f requent geneti c event contri buti ng to the mol ecul ar pathogenesi s of oral cancer. Of
cruci al i mportance i s the fact that abnormal i ti es of p53 have been i denti fi ed i n oral cancer,
associ ated wi th smoki ng and dri nki ng, and i n some dyspl asi c l eukopl aki as. The rol es of
oncogenes, TSGs, and DNA repai r mechani sms are currentl y very acti ve areas of research i n
oral cancer, as they are consi dered to be the basi c mechani sms i n the aeti ol ogy of the
di sease as wel l as havi ng enormous potenti al as earl y detecti on markers and for use as
targets for therapy.
Table 10.6 Summary of methods for the prevention of
oral carcinoma*
Pri mary preventi on
Advi ce on stoppi ng (or reduci ng) smoki ng and chewi ng areca (betel ) nut
Advi se moderati on of al cohol i ntake
Di etary advi ce (especi al l y encouragi ng i ntake of f resh frui t and vegetabl es)
Secondary preventi on (potenti al l y mal i gnant l esi ons)
Al ways screen enti re oral mucosa
Recogni ze abnormal i ti es (e.g. change of col our)
If candi dal i nfecti on present, i denti fy cause and treat
Refer any suspi ci ous l esi on to speci al i st, especi all y i f there i s no i mprovement
wi thi n 2 weeks of removi ng possi bl e causati ve factor
Terti ary preventi on (preventi on of recurrence)
Importance of routi ne extraoral and i ntraoral examinati on
Regul ar revi ew
Low threshol d for re-referral
Di etary advi ce (f resh frui t, vegetabl es)
Chemopreventi on may pl ay a rol e i n the future
*Reproduced (wi th sl i ght modi f i cati ons) from Tabl e 1 i n Ogden, G.R. and
Oral carci noma i s a geneti c di sease.
One emergi ng concept, however, i s that a number of acti vati ng and i nacti vati ng geneti c
events must occur f or the i ni ti ati on and progressi on of oral carci noma and that these geneti c
changes most probabl y occur as a mul ti step process (Fi g. 10. 7).
Geneti c changes i nvol ved i n the i ni ti ati on and progressi on of cancer occur i n a mul ti step
process.
The potential role of viruses in oral carcinogenesis
There i s consi derabl e i nterest i n the possi bl e i nteracti ons i n carci nogenesi s between HPVs,
si mi an vi rus (SV40), and l arge T anti gen. The reason f or thi s renewed i nterest i s based on
the experi mental evi dence surroundi ng the p53 TSG' s i nvol vement i n cancer and evi dence
that i ts aberrant expressi on may be al tered by p53 gene mutati ons as a consequence of
carci nogens, radi ati on, or other mutagens, or by other mechani sms, such as i nacti vati on of
the p53 protei n by vi ral or other protei ns. Si gni fi cant i n thi s respect may be vi ral protei ns
such as SV40, l arge T anti gen, HPV type 16 E6 protei n, as wel l as the cel l ul ar protei ns such
as MDM-2 (muri ne doubl e mi nute-2). The presence of hi gh-ri sk HPV types i n squamous cel l
carci noma of the head and neck may resul t i n i nacti vati on of p53 TSG functi on by nongeneti c
mechani sms. HPV has been f ound i n p53 mutated oral carci nomas.
Macl uskey, M. (2000). An overvi ew of the preventi on of oral cancer and di agnosti c
markers of mal i gnant change. Dental Update 27, 959 by permi ssi on of George
Warman Publ i cati ons (UK) Ltd.
Molecular techniques to identify genetic changes/alterations
in the oral mucosa
Geneti c al terati ons can be studi ed i n cel l ul ar DNA, RNA, and protei n from tumour ti ssue
speci mens; bi opsi es; ti ssue scrapes; and al so i n pl asma or serum from pati ents wi th
premal i gnant and mal i gnant di sease.
Mol ecul ar techni ques currentl y avai l abl e for genetic anal ysi s i ncl ude: at the DNA l evel , al l el i c
i mbal ance (l oss of heterozygosi ty anal ysi s), DNA sequenci ng, si ngl e-stranded conformati onal
anal ysi s, methyl ati on prof i l i ng, and mol ecul arcytogeneti c approaches; at the RNA l evel ,
Northern bl otti ng and reverse transcri ptase pol ymerase chai n reacti on (RT-PCR); and, at the
protei n l evel , Western bl otti ng, i mmunohi stochemi cal anal ysi s, i n si tu hybri di zati on, and
pepti de sequenci ng. The publ i cati on of the Human Genome Sequence now makes l arge-scal e
approaches to cancer geneti cs i n pati ent groups appl i cabl e, by usi ng expressi on chi p anal ysi s
whi ch contai ns most of al l of the known human genes, as wel l as approaches

such as CGH chi p and methyl ati on chi p anal ysi s. Furthermore, the i denti fi cati on of mi l l i ons of
si ngl e nucl eoti de pol ymorphi sms (SNPs) i n the human genome has made genotypi ng anal ysi s
a real i ty. Detai l s of these techni ques are cl earl y outsi de the scope of thi s book but i t i s
i mportant to gai n an understandi ng of the sci enti f ic basi s of these tests, thei r l i mi tati ons,
Fig. 10.7 Progressi on from l eukopl aki a to causes of the oral cavi ty. Reproduced from
Li ppman (2001). Mol ecul ar markers of the ri sk of oral cancer. New Engl and Journal of
Medi ci ne, 344, p. 1323. Copyri ght 2001 Massachusetts Medi cal Soci ety. Al l ri ghts
reserved.
P. 121
and appl i cati ons.
Tumour markers
In future, mol ecul ar geneti c markers of premal i gancy and mal i gnancy wi l l be used al ongsi de
conventi onal hi stopathol ogi cal techni ques to ai d i n the di agnosi s of oral cancer as wel l as
bei ng used i n the management of these pati ents to opti mi ze treatment. Many of these
markers are onl y used as research tool s at the present ti me but are currentl y bei ng
devel oped to become a part of routi ne di agnosti c testi ng.
Molecular markers for malignant transformation of oral
leukoplakia
There are currentl y no rel i abl e cl i ni cal or hi stol ogi cal features of oral l eukopl aki a that can be
used to predi ct whether a l esi on wi l l regress or progress to carci noma. The devel opment of
mol ecul ar markers that wi l l determi ne the ri sk of mal i gnant transformati on i s therefore of
fundamental i mportance. Furthermore, mol ecul ar markers may make i t possi bl e to moni tor
cancer recurrence as wel l as provi di ng the key for the devel opment of ef fecti ve
chemopreventi ve and chemotherapeuti c agents.
There have been recent i mportant advances i n the mol ecul ar assessment of the ri sk of
mal i gnant transformati on i n pati ents wi th oral l eukopl aki a. These studi es have i nvol ved
assessment of potenti al l y predi cti ve markers such as the establ i shment of pl oi dy status,
detecti on of al l el ei c i mbal ance (or l oss of heterozygosi ty), mutati onal profi l i ng of TSGs such
as p53, or determi ni ng aberrant expressi on of a range of genes i nvol ved i n carci nogenesi s
However, i t i s now cl ear that a si ngl e geneti c marker or cl ass of markers cannot be used to
predi ct the outcome of every case of oral l eukopl aki a. Compl ex model s to predi ct the ri sk of
cancer i n pati ents wi th oral l eukopl aki a are therefore bei ng devel oped.
Mol ecul ar assays may, i n f uture, defi ne pati ents who are at a l ow ri sk of oral cancer
devel opment and for whom non-i nvasi ve revi ew of thei r oral l eukopl aki as and modi f i cati on of
l i f estyl e (f or exampl e, tobacco cessati on) may be reasonabl e and based on sci enti fi c
evi dence. For those pati ents i denti fi ed as hi gh ri sk, resecti on of thei r l esi ons wi th
veri fi abl e cl ear resecti on margi ns may be the most appropri ate management wi th or wi thout
chemopreventi on. A wi de range of new chemopreventi on agents are currentl y bei ng assessed
and mol ecul ar bi omarkers wi l l be used as i ntermedi ate end-poi nts to assess thei r val i di ty. We
are enteri ng a new era of mol ecul ar di agnosi s and targeted treatment modal i ti es that wi l l be
based on the f i ndi ngs from the post-genomi c revol uti on.
Earl y detecti on and target drug therapy i n oral carci noma wi l l be based on future geneti c
profi l i ng.
Case 10.1 Discussion
The pati ent shoul d be asked i f he has noti ced thi s ul cer and, i f so, for how l ong and whether
i t has been sore. It i s al so i mportant to f i nd out i f there has been any soreness and i f he has
a habi t of bi ti ng hi s tongue or catchi ng i t wi th the denture. He shoul d be questi oned as to
whether he has suff ered from mouth ul cers i n the past. You shoul d recheck hi s medi cal
hi story, wi th parti cul ar emphasi s on ski n, eye, geni tal , or gut probl ems and ascertai n i f he
has general l y fel t wel l or suffered any wei ght l oss. The pati ent' s drug hi story shoul d be
revi ewed to check for any prescri bed or over the counter drugs. He shoul d al so be asked
about hi s i ntake of al cohol .
Q1 What questi ons woul d you ask thi s pati ent?
The f ol l owi ng si gni fi cant f eatures of the ul cer shoul d be noted by vi sual i nspecti on and
pal pati on: i ndurati on; prol i ferati ve growth; changes i n surface texture and col our; fi xati on to
the af fected ti ssue or underl yi ng structures.
Thi s pati ent appears to have presented wi th an asymptomati c ul cer but may, when
speci f i cal l y asked, report soreness or di scomf ort on eati ng. The broken denture coul d
i ndi cate the ul cer i s traumati cal l y i nduced, parti cul arl y i f i t has no cl i ni cal features
suggesti ve of mal i gnancy. An i sol ated aphthous ul cer i s rarel y symptom-free and the pati ent
i s l i kel y to have gi ven a hi story of RAS i n the past. Drugs, such as ni corandi l or NSAIDS can
predi spose to oral ul cerati on but l esi ons are usuall y pai nf ul and the pati ent woul d report a
posi ti ve drug hi story. In the case of thi s pati ent who has one (or possi bl y more) si gni fi cant
ri sk f actors (that i s, he i s a heavy smoker) and presents wi th an i sol ated, asymptomati c
ul cer on the l ateral border of the tongue (ri sk si te), the l i kel i hood of a squamous cel l
carci noma i s hi gh.
If you suspect that the l esi on i s a traumati c ul cer and there i s an obvi ous source of trauma,
thi s shoul d be el i mi nated by adjusti ng the denture. The pati ent must, however, be revi ewed
i n 1014 days, to check i f there are any si gns of heal i ng.
Thi s gentl eman shoul d be tol d at thi s stage why i t i s i mportant for hi m to be f ol l owed up. If,
however, the ul cer has any suspi ci ous features, he shoul d be referred to the nearest
speci al i st centre (that i s, oral medi ci ne or oral and maxi l l ofaci al surgery) for f urther
i nvesti gati ons, i ncl udi ng bi opsy. Bi opsy of an oral l esi on that you suspect may be a
carci noma, shoul d be undertaken by the speci al i st who wi l l deci de on defi ni ti ve treatment
and not the general dental practi ti oner. A referral l etter, descri bi ng the pati ent' s hi story and
ful l descri pti on of the ul cer, shoul d be faxed (as wel l as posted) to the speci al i st centre, wi th
a f ol l ow-up tel ephone cal l to check that thi s has been recei ved. The pati ent shoul d be
advi sed of the i mportance of attendi ng hi s speci al ist appoi ntment. Pati ents

may ask i f thei r mouth ul cer coul d be a cancer or mal i gnant or a
tumour, and denti sts may fi nd thi s questi on awkward to answer. In the majori ty of
cases i t i s better to i nform the pati ent that thi s i s a possi bi l i ty and that i t must be rul ed out
by further i nvesti gati ons. It may be hel pf ul , at thi s stage, to poi nt out that there are a
number of di fferent causes of mouth ul cers. If the pati ent' s ul cer i s f ound to be carci noma,
the denti st i s l i kel y to have a rol e to pl ay i n posttreatment rehabi l i tati on.
Projects
1. Read the cl i ni cal gui del i nes, The oral management of oncol ogy pati ents, publ i shed by
the Facul ty of Dental Surgery, Royal Col l ege of Surgeons of Engl and.
2. What i s meant by the term heal th-rel ated qual i ty of l i fe? How has thi s been
measured i n pati ents who have undergone surgery for the treatment of oral carci noma?
Q2 What f eatures of the ul cer are i mportant to note?
Q3 What i s the most l i kel y di f ferenti al di agnosi s i n thi s parti cul ar case?
P. 122
> Tabl e of Cont ent s > 11 - Mucocut aneous di sease and connect i ve t i ssue di sor der s
11
Mucocutaneous disease and connective tissue
disorders
Problem cases
Case 11.1
One of your edentul ous pati ents, a 62-year-ol d l ady, tel ephones the surgery requesti ng an
urgent appoi ntment because she i s af rai d she may have mouth cancer. Whi l st cl eani ng her
dentures the ni ght before, she had noti ced some whi te patches i nsi de her mouth and
remembers readi ng i n a magazi ne that these can be a si gn of mouth cancer. Thi s l ady had
been unabl e to sl eep due to worry. She i s seen l ater that day and on exami nati on you note a
network of whi te l ace-l i ke l esi ons on both buccal mucosae, whi ch are pai nl ess. There
are no other oral l esi ons. On further questi oni ng the pati ent reports an i tchy rash on her
wri sts.
Case 11.2
A 41-year-ol d Jewi sh man presents wi th a 9-month history of mouth ul cerati on, whi ch has
become persi stent and consi derabl y worse. It i s al so causi ng great di scomf ort on eati ng. He
has noti ced that l arge bl i sters occur i nsi de hi s l i ps but these rapi dl y burst. Over the l ast few
days he has devel oped ski n l esi ons and he shows you some eroded ski n l esi ons on hi s back.
There are no ocul ar or geni tal symptoms. Exami nati on of the mouth reveal s wi despread oral
ul cerati on and the Ni kol sky si gn i s posi ti ve.
Case 11.3
A 2-year-ol d boy i s referred to you by a dermatol ogi st for dental care. Hi s parents tel l you
that he was di agnosed at bi rth as havi ng epi dermol ysi s bul l osa (EB).
Mucocutaneous disease
It was poi nted out i n Chapter 1 that the oral mucous membrane, al though si mi l ar to other
l i ni ng mucosae i n structure and general behavi our, al so resembl es the ski n i n some ways.
Q1 What i s the most l i kel y di agnosi s of thi s l ady' s oral l esi ons?
Q2 Are her ski n symptoms rel ated?
Q3 What i nvesti gati ons woul d you carry out?
Q4 What advi ce and treatment woul d you gi ve to thi s l ady?
Q1 How woul d you i nvesti gate and manage thi s gentl eman?
Q1 What are the orodental mani f estati ons of thi s condi ti on? Di scuss the di f fi cul ti es you
mi ght encounter when provi di ng dental treatment for thi s boy.
Thi s i s i n keepi ng wi th the transi ti onal posi ti on and f uncti on of the oral mucous membrane,
l yi ng as i t does i n an i ntermedi ate posi ti on between the ski n and the gut mucosa proper. One
resul t of thi s si tuati on i s that general i zed di seases, both of mucosae and of ski n, may affect
the mouth. In such ski n di seases, however, the oral l esi ons, because of the modi f yi ng effect
of the envi ronment, may bear l i ttl e superf i ci al resembl ance to those of the ski n. The
conti nual presence of sal i va, secondary i nfecti on by oral organi sms, and the repeated
traumas of the oral envi ronment pl ay thei r respective parts i n the modi f i cati on of the oral
l esi ons. Parti cul arl y good exampl es of thi s form of modi fi cati on are shown i n the group of
di seases (i mmunobul l ous) i n whi ch bl i sters or bul l ae are formed. When thi s occurs i n the
mouth the bul l ae rapi dl y break down to f orm ul cerated areas. Such behavi our af fects the
management of these condi ti ons. It i s often necessary to treat the secondary i nfecti on
produced i n thi s way before proceedi ng to more systemi c treatment. A di ff erent form of
modi fi cati on i n oral l esi ons occurs i n some di seases (f or exampl e, l i chen pl anus) i n whi ch the
i ni ti al oral l esi on may bear l i ttl e morphol ogi cal resembl ance to the ski n l esi on, even though
the basi c hi stopathol ogi cal changes are si mi l ar. These vari ants presumabl y depend on the
di f feri ng structures of the ski n and the oral mucosa rather than on the effect of the
envi ronment.
It i s not easy to understand why some ski n di seases commonl y produce oral l esi ons whi l e
others do not. For exampl e, psori asi s, one of the two most common ski n di seases seen i n
European cl i ni cs (the other i s eczema), produces oral l esi ons very rarel y. In fact, there i s
some di f ference of opi ni on as to whether a characteri sti c oral l esi on does exi st. On the other
hand, l i chen pl anus produces oral l esi ons i n a hi gh proporti on of the pati ents wi th ski n
i nvol vement.
Oral l esi ons have occasi onal l y been descri bed i n a very wi de range of ski n di seases, even
though such behavi our may not be characteri sti c of the condi ti on. Thus, i n the di agnosi s of
oral l esi ons of doubtf ul aeti ol ogy, a hi story of ski n di seases i s al ways of i nterest. Perhaps the
most i mportant consi derati on i n thi s context i s the fact that, i n some di seases of the ski n
(i ncl udi ng some

of the most seri ous such as pemphi gus), the oral l esi ons may appear bef ore those of the ski n
and may thus provi de an opportuni ty for earl y di agnosi s and the rapi d i ni ti ati on of treatment.
Defi ni ti ve di agnosi s of the oral l esi ons i n ski n di seases depends l argel y on bi opsy
exami nati on and thi s shoul d be carri ed out on any doubtful l esi on. Thi s i s not, however,
al ways an easy matter, parti cul arl y i n the case of bul l ous l esi ons i n whi ch the mani pul ati ons
of i nci si onal or exci si onal bi opsy may cause vi rtual di si ntegrati on of the l esi on. If such a
l esi on cannot be removed i ntact f or exami nati on, then the edge of the l esi on may often show
the characteri sti c changes better than a central area where secondary changes may obscure
the pi cture. A bi opsy taken f rom an ul cerated area may show nothi ng but non-speci f i c
i nfl ammatory changes. Immunofl uorescent techni ques and, i n parti cul ar, di rect
i mmunofl uorescent studi es of bi opsy materi al have revol uti oni zed the methods of di agnosi s
of i mmunobul l ous ski n di seases and thei r oral l esi ons. For thi s techni que a peri l esi onal
bi opsy i s taken and f resh (unfi xed) ti ssue sent to the pathol ogy l aboratory.
In thi s chapter the condi ti ons chosen for di scussi on are those that are rel ati vel y commonl y
seen i n the oral medi ci ne cl i ni c or those of parti cul ar i nterest or i mportance i n the dental
context. Other condi ti ons that l i nk oral medi ci ne and dermatol ogy i ncl ude the
genatodermatoses (Chapter 9), PeutzJegher' s syndrome (Chapter 9), and Wegener' s
granul omatosi s (Chapter 1).
Lichen planus and lichenoid reactions
Skin lichen planus
In thi s di sease the ski n l esi ons take the form of dusky pi nk papul es that may occur i n any
P. 126
si te, but are most commonl y found on the fl exor surf aces of the wri sts (Fi g 11. 1), on the
geni tal ski n, on the abdomen and l umbar regi ons, and on the ankl es and shi ns. Fi ne whi te
stri ati ons overl i e the papul es. These are the so-cal l ed Wi ckham' s stri ae that are
characteri sti c of the condi ti on (Fi g. 11. 1). In general , the ski n l esi ons are rel ati vel y short-
l i ved, the average durati on bei ng of the order of 9 months. After thi s they fade, l eavi ng
behi nd a f ai ntl y pi gmented patch that may take a consi derabl e ti me to di sappear. In a
si gni f i cant proporti on of the pati ents, however, there may be a recurrence of the l esi ons. The
majori ty of ski n l esi ons cause l i ttl e troubl e apart from i tchi ng, whi ch i s very vari abl e i n i ts
i ntensi tyi n some i nstances i t i s so i nsi gni fi cant that the l esi ons are not noti ced by the
pati ent. In a f ew cases the Kbner phenomenon may be seen wi th l esi ons di stri buted i n a
l i near pattern al ong a scratch mark on the ski n. Scal p l esi ons may al so occur i n a few
pati ents (usual l y femal e). Of ten these are not papul ar, but are represented by patches of
al opeci a. There are wi despread vari ati ons i n the cli ni cal pi cture, shown by occasi onal pati ents
who may present wi th bul l ous, hypertrophi c, or annul ar l esi ons, and, very rarel y, the di sease
may f i rst occur i n an acute form wi th i ni ti al symptoms much more severe than those
descri bed above. The hi stol ogy of a ski n l esi on i s qui te characteri sti c and, i n al l essenti al s,
resembl es that of the oral l esi ons. The most obvi ous f eature i s basal cel l l i quefacti on and the
presence of a narrow dense band of i nf l ammatory cel l s, predomi nantl y l ymphocytes, wi thi n
the dermi s and l yi ng just bel ow the epi thel i um. In the ski n the effect appears to be a pushi ng
up of the epi thel i um to form the papul es, al though thi s may not, i n f act, be the actual
mechani sm of papul e formati on. The overl yi ng epi thel i um may undergo a vari ety of changes,
hyperorthokeratosi s bei ng the most common fi ndi ng in the case of the ski n l esi ons.
In the Kbner phenomenon i ndi vi dual ski n l esi ons devel op on a si te of trauma or an
operati on scar. Thi s i s seen i n a number of dermatol ogi cal condi ti ons i ncl udi ng l i chen pl anus
and psori asi s.
Invol vement of the nai l s i s seen i n about 10 per cent of pati ents and thi s mani f ests as f i ne
ri dgi ng or groovi ng, severe dystrophy, and, i n a few cases, destructi on of the nai l bed. The
cl assi c nai l change of l i chen pl anus i s the pterygium, i n whi ch adhesi on between the dorsal
nai l fol d and the nai l bed l eads to the parti al destructi on of the nai l .
Fig. 11.1 Typi cal ski n l esi ons of l i chen pl anus.
The f l exor surfaces of wri sts, ankl es, and shi ns and l umbar regi ons are the usual si tes of
i nvol vement i n ski n l i chen pl anus.
The geni tal mucosa al so can be i nvol ved i n l i chen pl anus but may be asymptomati c. The
vul vovagi nal gi ngi val (VVG) syndrome i s a rare vari ant of l i chen pl anus, characteri zed by
erosi ons of vul val , vagi nal , and gi ngi val mucosae wi th a predi l ecti on f or scarri ng and stri cture
formati on. Thi s causes consi derabl e probl ems to the pati ent and i s di ffi cul t to manage.
The vul vovagi nal gi ngi val syndrome i s a rare vari ant of l i chen pl anus, whi ch i s di ff i cul t to
manage.
Aetiology of lichen planus
Li chen pl anus i s an i mmunol ogi cal l y medi ated di sease but i n the majori ty of cases does not
appear to be consi stentl y part of an autoi mmune di sturbance. A number of factors have been
i mpl i cated i n the aeti ol ogy of l i chen pl anus and associ ati ons, often unsubstanti ated, made
wi th systemi c di seases such as di abetes mel l i tus and l i ver di sease. Lesi ons that cl i ni cal l y and
hi stol ogi cal l y

resembl e those of oral l i chen pl anus (l i chenoi d reacti on) can be preci pi tated by an ever-
i ncreasi ng range of drugs. Li chenoi d reacti ons wi l l be di scussed i n the next secti on of thi s
chapter.
Oral lichen planus
PREVALENCE
Oral l i chen pl anus affects f rom 0.1 to 4 per cent of i ndi vi dual s dependi ng on the popul ati on
studi ed and i s general l y a di sease of the mi ddl e-aged and el derl y, wi th a f emal e
preponderance of 2:1. The age range i s si mi l ar to that i n pati ents reporti ng wi th ski n l esi ons
onl y, al though, i n the case of pati ents wi th oral lesi ons, there i s a rather hi gher proporti on of
pati ents i n the 60 year pl us group. The authors have seen a smal l number of pati ents wi th
confi rmed oral l esi ons at a very earl y age, the youngest bei ng 7 years ol d. Thi s woul d be
consi dered a great rari ty i n the case of ski n l esi ons.
Oral l i chen pl anus affects 0. 1 to 4 per cent of i ndi vi dual s, dependi ng on the popul ati on
studi ed.
Oral l esi ons occur i n a consi derabl e proporti on of pati ents wi th l i chen pl anus and are seen i n
the oral medi ci ne cl i ni c f ar more frequentl y than the oral l esi ons of other ski n di seases. The
true i nci dence of oral l esi ons i s a l i ttl e di ffi cul t to determi ne si nce the fi gures avai l abl e vary
wi del y, a si gni f i cant factor bei ng whether the patients are fi rst seen i n a dermatol ogi cal cl i ni c
(i n whi ch case oral l esi ons are present i n about 70 per cent of cases) or i n a dental cl i ni c. In
these l atter pati ents, presenti ng because of oral lesi ons, the i nci dence of ski n l esi ons i s of
the order of 40 per cent. Thi s vari ati on i s probabl y due to a number of factors, the most
i mportant bei ng the asymptomati c nature of the oral l esi ons i n many cases. A second
i mportant factor i s the i nconstant sequenti al rel ati onshi p of the l esi onsthe oral l esi ons
may occur before, af ter, or at the same ti me as the ski n l esi ons. In general , however, oral
l esi ons l ast much l onger than ski n l esi ons. A mean durati on of 4. 5 years has been suggested
but there i s no doubt that i n many cases thi s peri od may be greatl y exceeded.
The oral l esi ons are usual l y bi l ateral and i nvol ve the buccal mucosa i n about 90 per cent of
al l cases. In descendi ng order of f requency the tongue, gi ngi vae, al veol ar ri dge, l i ps, and
P. 127
l ess commonl y the pal ate may al so be affected.
In oral l i chen pl anus, the buccal mucosa and tongue are the most common si tes of
i nvol vement and l esi ons tend to be bi l ateral .
A range of cl i ni cal presentati ons can occur i n oral l i chen pl anus and may coexi st i n the same
pati ent. There i s, however, some conf usi on i n the li terature concerni ng the termi nol ogy used
to descri be the cl i ni cal vari ants of oral l i chen planus (Tabl e 11. 1).
Reti cul ar, pl aque-l i ke, and papul ar vari ants tend to be asymptomati c. In these vari ants the
epi thel i al change i s of hyperparakeratosi s or, occasi onal l y, hyperorthokeratosi s. There i s no
atrophy of the epi thel i um and, hence, no ul cerati on. The characteri sti c appearance i s of
whi te streaks on the oral mucosa, arranged i n a reti cul ar l ace-l i ke pattern (Fi g.
11. 2). In some pati ents the l esi ons may be more confl uent (pl aque-l i ke) and resembl e a
l eukopl aki a, whereas i n others there may be papul ar, l i near, or annul ar arrangements of the
whi te areas. These are of ten descri bed as bei ng si mi l ar to the Wi ckham' s stri ae of the ski n
l esi ons, but are, i n fact, much more cl earl y def i ned. In the course of the di sease the
morphol ogy of the l esi ons may change, and there may al so be vari ati ons i n thei r extent and
i ntensi ty. In general , these l esi ons are of ten qui te symptom-free and are often noti ced
i nci dental l y by the pati ent, al though a sensati on of roughness may be present. In the
stri ated f orms the di agnosi s may i ni ti al l y be made wi th some conf i dence on appearance
al one, but i n the case of the confl uent pl aque-l i ke l esi ons the di agnosi s may be made onl y
after bi opsy.
Table 11.1 Clinical variants of oral lichen planus
Reti cul ar
Pl aque-l i ke
Papul ar
Atrophi c
Erosi ve (ul cerati ve)
Bul l ous
Desquamati ve gi ngi vi ti s
In other forms of oral l i chen pl anus, the oral epi thel i um undergoes atrophi c changes and i s
easi l y l ost from the weak and oedematous basal areas. Thi s may resul t i n the formati on of
ul cerati ve l esi ons on the mucosa, these often bei ng associ ated wi th nearby areas i n whi ch
reti cul ar or atrophi c l esi ons occur (Fi g. 11. 3). In a few pati ents the basal and subepi thel i al
oedema may l ead to separati on of the epi thel i um wi th consequent bul l a formati on, but such
bul l ae, devel oped i n i ni ti al l y atrophi c epi thel i um, are very fragi l e and rapi dl y di si ntegrate to
produce the characteri sti c ul cerati ve l esi ons of the condi ti on. In publ i shed studi es of oral
l i chen pl anus the terms ul cerati ve and erosi ve have been used

synonymousl y to descri be a cl i ni cal appearance i n whi ch there has been epi thel i al l oss. Thi s
vari ati on i n descri pti ve termi nol ogy used i n oral medi ci ne l eads to conf usi on and does not
necessari l y refl ect the hi stopathol ogy of the l esi on: i n addi ti on the resul ts of cl i ni cal tri al s
are di ff i cul t to eval uate. In thi s chapter, the cl i ni cal l y descri pti ve term erosi ve wi l l
be used to descri be the i rregul ar areas of oral epi thel i al destructi on (shal l ow or deep) whi ch
are covered i n a yel l ow l ayer of fi bri n. Atrophi c lesi ons, i n whi ch there has been epi thel i al
thi nni ng, present as i rregul ar areas of erythematous mucosaboth erosi ve and atrophi c
vari ants tend to be symptomati c. Unl i ke the si tuati on i n the reti cul ar or pl aque-l i ke f orms of
the di sease, there i s of ten consi derabl e di scomfort to the pati ent, parti cul arl y when eati ng
spi cy or aci d f oods. The mucosa i s al so suscepti bl e to mechani cal i rri tati on and the i ni ti al
symptoms may occasi onal l y appear as denture trauma bef ore any other more characteri sti c
l esi ons appear. Pati ents, of ten i n ol der age groups, may present wi th extensi ve erosi ve
l esi ons, coveri ng l arge surfaces of the tongue and buccal mucosa. These l esi ons have a
gl azed appearance and tend to be separated from the adjacent mucosa by a cl earl y
demarcated edge (Fi g. 11. 4). These areas of erosi on can take months to resol ve and are
of ten repl aced by whi te, confl uent l esi ons, parti cul arl y on the tongue. Needl ess to say, thi s
type of l i chen pl anus causes a great deal of di scomf ort and pati ents have di ffi cul ty when
eati ng.
Al l these cl i ni cal vari ants of oral l i chen pl anus can af fect the gi ngi vae, wi th or wi thout
i nvol vement of other oral si tes. In about 10 per cent of cases gi ngi val i nvol vement al one
occurs and can make the cl i ni cal di agnosi s more di ff i cul t. In addi ti on, gi ngi val archi tecture
can create probl ems when choosi ng a si te to bi opsy and the resul ti ng hi stopathol ogy i s more
di f fi cul t to i nterpret, parti cul arl y i f there i s a superi mposed non-speci f i c gi ngi vi ti s. In
Fig. 11.2 Reti cul ar, non-erosi ve l i chen pl anus of buccal mucosa.
P. 128
pati ents wi th i nvol vement of thi s si te the gi ngi vae often have a red, gl azed appearance
aff ecti ng thei r ful l wi dth (Fi g. 11. 5). Thi s cl i ni cal appearance i s known as desquamati ve
gi ngi vi ti s whi ch may be di ffi cul t to cl i ni cal l y di fferenti ate from i nvol vement wi th other
mucocutaneous di seases, parti cul arl y pemphi goi d. The term desquamati ve gi ngi vi ti s
i s a cl i ni cal descri pti ve term and does not therefore i nf er any speci f i c underl yi ng pathol ogy.
Not al l cases are due to l i chen pl anus.
The defi ni ti ve di agnosi s of al l types of l i chen pl anus i s by bi opsy and hi stopathol ogy shoul d
i ncl ude peri odi c aci d Schi ff (PAS) stai ni ng for Candi da speci es (see Chapter 4). Al though the
appearance of the l esi ons may gi ve an i ndi cati on of the di agnosi s i n some cases, thi s i s not
al ways so and the cl i ni cal features may someti mes be conf used wi th those of i mmunobul l ous
l esi ons or wi th patches of erythropl aki a. The presence of ski n l esi ons i s a usef ul di agnosti c
poi nter but, evi dentl y, not a constant one.
Fig. 11.3 Reti cul ar and erosi ve l esi ons of oral l i chen pl anus.
HISTOPATHOLOGY
The essenti al hi stol ogi cal change i n these l esi ons i s the same as i n those of the ski nthe
Fig. 11.4 Large erosi ve l esi on of l i chen pl anus wi th whi te pl aque at commi sureys
Fig. 11.5 Atrophi c and erosi ve l i chen pl anus af fecti ng the gi ngi vae.
presence of a subepi thel i al band of l ymphocytes, predomi nantl y of the T type, and some
macrophages (Fi g. 11. 6)but i n the case of the oral mucosa there i s a much wi der range of
epi thel i al response, wi th acanthosi s or atrophy, orthokeratosi s, or parakeratosi s. However,
the most common f i ndi ng i s of parakeratosi s, the rete pegs bei ng di storted to gi ve a saw-
tooth or, more commonl y, fl attened appearance. Around the basement membrane there
i s oedema, associ ated wi th degenerati ve changes i n the basal cel l s, an associ ati on that i n
some cases l eads to vi rtual separati on of the epi thel i um from the cori um. The di rect
i mmunofl uorescence fi ndi ngs i n l i chen pl anus are not

hi ghl y speci fi c. A band of f i bri n i s shown up at the basal zone, but no i mmunogl obul i n
deposi ts. Cytoi d ci vatte bodi es may be seen, however, both i n the epi thel i um and i n
the dermi s. These are non-di sease-speci f i c spheri cal structures that contai n vari abl e
i mmunogl obul i ns and compl ement components. A hi gh inci dence of these i s i ndi cati ve, i f not
di agnosti c, of l i chen pl anus.
The pathogenesi s of l i chen pl anus i s not ful l y understood but probabl y i nvol ves a cel l -
medi ated i mmune response to anti geni c changes i n oral mucosa. Thi s woul d be consi stent
wi th the predomi nantl y T-l ymphocyte i nfi l trate.
Lichenoid reactions
In a mi nori ty of cases (but sti l l a substanti al number) l i chen pl anus i s preci pi tated by drugs
or other substances. A number of drugs have been i mpl i cated i n l i chenoi d drug erupti ons
(LDEs) i ncl udi ng nonsteroi dal anti -i nfl ammatory agents (NSAIDs), anti hypertensi ve drugs
(especi al l y angi otensi on converti ng enzyme (ACE) i nhi bi tors), anti mal ari al s, and gol d
i njecti ons (Tabl e 11. 2). Wi thdrawal of the preci pi tati ng drug usual l y resul ts i n resol uti on of
the LDE but not al l l esi ons uni forml y regress.
Local i zed l i chenoi d reacti ons have al so been associated wi th hypersensi ti vi ty reacti ons to
mercuri c sal ts rel eased from amal gam restorati ons. These characteri sti cal l y occur where the
oral mucosa i s i n contact wi th the of fendi ng restorati on. Removal of the restorati on
frequentl y resul ts i n regressi on of the l esi on and thi s woul d suggest a type IV
hypersensi ti vi ty reacti on, si mi l ar to that seen i n contact dermati ti s as a response to metal s
such as ni ckel . A few cases of l i chenoi d reacti ons have, however, al ready been reported i n
response to composi te resi ns. Pati ents undergoi ng bone marrow transpl antati on can devel op
an oral l i chenoi d l esi on as a mani f estati on of a graft-versus-host reacti on.
P. 129
Fig. 11.6 Secti on of oral mucosa i n l i chen pl anus.
Li chenoi d drug erupti ons can be caused by a l arge number of drugs, i ncl udi ng NSAIDs and
anti hypertensi ves.
LDE and i di opathi c oral l i chen pl anus are si mi l ar both cl i ni cal l y and hi stol ogi cal l y. Mucosal
l esi ons i n oral l i chen pl anus tend to be bi l ateral , whereas LDEs have a tendency to occur
uni l ateral l y and may af fect the pal ate. Hi stol ogi cal l y i n LDEs, l ymphoi d fol l i cl es may be
promi nent and there may be a more mi xed cel l popul ati on i ncl udi ng eosi nophi l s and pl asma
cel l s.
Management of lichen planus and lichenoid reactions
INVESTIGATIONS
In addi ti on to standard hi story taki ng and cl i ni cal exami nati on, pati ents shoul d be asked i n
detai l about thei r drug hi story and possi bl e i nvol vement of other si tes, i ncl udi ng the ski n and
other mucous membranes. Mi crobi ol ogi cal sampl i ng of mucosal si tes may reveal candi dal
i nfecti on, whi ch i t may be advi sabl e to treat pri or to bi opsy. Bi opsy shoul d be undertaken for
al l pati ents wi th suspected oral l i chen pl anus and may need to be from more than one
aff ected si te. PAS stai ni ng of hi stol ogi cal sampl es i s essenti al .
THERAPY
Treatment of ski n l i chen pl anus i s di rected at control l i ng the i tch and moderatel y potent
topi cal steroi ds are probabl y the most effecti ve agent for thi s. Sedati ng, systemi c
anti hi stami nes may be hel pful at ni ght. Short courses of systemi c steroi ds have been
suggested f or severe cases but l ong-term steroi ds shoul d not be used f or chroni c cases.
Reti noi ds and ci cl ospori n have been used i n some reported cases, wi th mi xed success.
Therapy for symptomati c oral l i chen pl anus i s ai med at provi di ng rel i ef of di scomfort, heal i ng
of erosi ve l esi ons, and i ncreasi ng epi thel i al thi ckness i n areas of atrophy. It usual l y i ncl udes
the use of an anti septi c mouthwash (to ai d wi th pl aque control and reduce secondary
i nfecti on) and an anal gesi c mouthwash (to reduce di scomfort)see Tabl e 11. 3. Local
steroi ds are the mai nstay of treatment and assi st i n the heal i ng of erosi ons and reducti on of
atrophy (Tabl e 11. 3). Hydrocorti sone l ozenges are of l i mi ted val ue i n oral l i chen pl anus and
tri amci nol one, i n an adhesi ve paste, i s di f fi cul t to appl y to wi despread erosi ve and atrophi c
l esi ons. These preparati ons, however, are unl i kel y to gi ve ri se to probl ems associ ated wi th
Table 11.2 Systemic drug groups commonly implicated in
oral lichenoid drug eruptions
Nonsteroi dal anti -i nfl ammatory drugs (NSAIDs)
Angi otensi n-converti ng enzyme (ACE) i nhi bi tors
Oral hypogl ycaemi cs
Di ureti cs
Anti mal ari al s
Gol d
Peni ci l l ami ne
systemi c absorpti on. Other topi cal preparati ons (Chapter 3) i ncl ude sol ubl e betamethasone
tabl ets, di ssol ved i n water and used as a mouthwash, steroi d sprays (for exampl e,
becl omethasone), potent steroi d oi ntments (for exampl e, fl uoci noni de) mi xed wi th an
adhesi ve base, and tri amci nol one mouthwashes. In al l these preparati ons, systemi c
absorpti on can occur and oral candi dosi s may occasi onal l y compl i cate treatment. Topi cal
anti f ungal therapy i s often i ndi cated f or pati ents wi th

symptomati c oral l i chen pl anus. Not onl y do topi cal steroi ds and other i mmunomodul ators
predi spose to candi dosi s but several studi es have shown an i ncreased preval ence of Candi da
speci esi n both mycol ogi cal and hi stol ogi cal studi es of oral l i chen pl anus. Superi mposed
oral candi dosi s can exacerbate the oral symptoms of oral l i chen pl anus and shoul d be
treated, i n the fi rst i nstance, by appropri ate topical anti f ungal agents (Chapter 4). Nystati n
pasti l l es or amphoteri ci n l ozenges are appropri ate but topi cal nystati n may not be tol erated
by pati ents wi th a sore, atrophi c mucosa. Mi conazole gel may be preferred and i n some cases
a systemi c anti f ungal , such as fl uconazol e, i s i ndi cated. Care must be taken wi th the use of
azol es because of thei r i nteracti ons wi th other drugs, notabl y warf ari n.
P. 130
In severe cases wi th maj or erosi ve l esi ons, short courses of systemi c steroi ds can be
eff ecti ve to al l evi ate acute exacerbati ons of oral l i chen pl anus but are contrai ndi cated i n the
l ong term. Hi gh-concentrati on steroi d mouthwashes in some cases appear to be equal l y
eff ecti ve. Azathi opri ne may be requi red as a steroid-spari ng drug. Other systemi c drugs,
i ncl udi ng ci cl ospori n, reti noi ds, anti mal ari al s, and dapsone (Tabl e 11. 3), have been
advocated f or the treatment of severe oral l i chen pl anus wi th varyi ng reported resul ts. There
i s, however, a pauci ty of evi dence-based treatment protocol s for thi s condi ti on. Nearl y al l
these systemi c f orms of medi cati on have si gni fi cant si de-eff ects.
OTHER ASPECTS OF MANAGEMENT
If hi stopathol ogy i ndi cates a si gni fi cant degree of dyspl asi a, then surgi cal (or l aser) exci si on
of the af fected si te may need to be consi dered. Li chenoi d l esi ons due to drug therapy may be
di f fi cul t to manage i f the drug i s requi red for a pati ent' s general medi cal condi ti on and an
al ternati ve cannot be submi tted. Li chenoi d drug erupti ons can onl y be rel i abl y confi rmed i f
they resol ve after wi thdrawal or return after re-chal l enge wi th the drug. In most cases, thi s
Table 11.3 Therapeutic options for oral lichen planus
Type Examples
Anti septi c Chl orhexi di ne gl uconate (mouthwash)
Anal gesi cs Benzydami ne hydrochl ori de (mouthwash)
Li gnocai ne ri nse
Anti f ungal s Nystati n pasti l l es (topi cal )
Amphoteri ci n l ozenges (topi cal )
Mi conazol e gel (topi cal )
Fl uconazol e (systemi c)
Topi cal corti costeroi ds Hydrocorti sone hemi succi nate (pel l ets)
Tri amci nol one acetoni de (i n adhesi ve paste)
Fl uoci noni de (i n adhesi ve paste)
Betamethasone sodi um phosphate (mouthwash)
Tri amci nol one mouthwash
Becl omethasone di propi onate (spray)
Budesoni de (spray)
Systemi c i mmunomodul ators Predni sol one
Azathi opri ne
Ci cl ospori n
Other reported therapi es Anti mal ari al s (hydroxychl oroqui ne)
Topi cal tacrol i mus
Reti noi ds
Dapsone
i s di ffi cul t or unethi cal to do. Dental restorati ons, parti cul arl y l arge, corrodi ng amal gams i n
posteri or mol ars, shoul d be consi dered for repl acement as thi s may resul t i n resol uti on of an
adjacent l i chenoi d reacti on. Stress has been i mpl i cated as a factor i n oral l i chen pl anus but
there are f ew studi es that have proved a di rect rel ati onshi p. The chroni c di scomfort
associ ated wi th symptomati c oral l i chen pl anus i s undoubtedl y goi ng to be a stressf ul factor
and af fect a pati ent' s qual i ty of l i fe.
Pati ents shoul d be encouraged to stop smoki ng and gi ven advi ce about sensi bl e dri nki ng.
Oral and denture hygi ene shoul d be addressed and attenti on pai d to the pati ent' s peri odontal
heal th. Al l these factors can del eteri ousl y affect oral l i chen pl anus and may predi spose to
oral candi dosi s.
LONG-TERM REVIEW OF ORAL LICHEN PLANUS
The great majori ty of oral l i chen pl anus cases run a compl etel y beni gn course and some wi l l
go i nto remi ssi on after a vari abl e number of years. However, i n a smal l proporti on of cases
(0.43. 3 per cent i n reported studi es, over a foll ow-up peri od of 0.520 years), oral
l esi ons undergo mal i gnant change. For thi s reason, l ong-term revi ews of al l cases shoul d be
undertaken and re-bi opsy performed i f there are any suspi ci ous cl i ni cal changes, such as a
nodul ar, verrucous, speckl ed, or vel vety-red appearance of the mucosa. Pati ents
shoul d be advi sed to report any si gni fi cant changes i n thei r l esi ons or symptoms. Ideal l y, a
photographi c record of the pati ents l esi ons shoul d be taken at each f ol l ow-up vi si t. It
has been suggested that i t i s the erosi ve/atrophi c l esi ons of oral l i chen pl anus that are more
l i kel y to transform and that these cases shoul d theref ore undergo more stri ngent f ol l ow-up.
There are, however, no control l ed studi es to ref ute or substanti ate thi s cl ai m.
A smal l proporti on of oral l i chen pl anus cases undergo mal i gnant change. Long-term revi ew
i s therefore essenti al .
Immunobullous disease
Pemphigus
Pemphi gus i s a group of i mmunobul l ous di sorders that affect the ski n and mucous
membranes. It i s characteri zed by the devel opment of autoanti bodi es to anti gens on the
kerati nocyte cel l surface. The bul l ae are i ntraepi thel i al and therefore l i e above the basal
l ayer. Pemphi gus i s a di sease of mi ddl e age wi th most pati ents between the ages of 40 and
60 years wi th an even gender di stri buti on. There i s a raci al factor i nvol ved i n that there i s a
hi gh i nci dence of the condi ti on among peopl e of Jewi sh ori gi n,

al though pati ents are by no means restri cted to this group. The oral mucous membrane i s
i nvol ved i n a hi gh proporti on of the pati ents and, i n fact, hal f of al l i ni ti al l esi ons are found
i n the mouth. Before the i ntroducti on of steroi ds the prognosi s i n thi s di sease was very poor.
The i ntroducti on of treatment by systemi c steroi ds, however, has made the outl ook
consi derabl y l ess gl oomy, al though pemphi gus must sti l l be consi dered a very seri ous
condi ti on. It i s, therefore, parti cul arl y i mportant that the oral l esi ons shoul d be recogni zed
earl y i n order that treatment may be started at the earl i est opportuni ty. There are four mai n
vari eti es of pemphi gus: pemphi gus vul gari s, vegetans, fol i aceus, and erythematosus.
Pemphi gus vul gari s i s the most common vari ety and accounts f or about 70 per cent of al l
cases. It usual l y begi ns wi th shal l ow erosi ons on the ski n and ruptured bl i sters on mucosal
surfaces. In pemphi gus vegetans, the rupture of bul l ae i s accompani ed by exuberant
granul ati on ti ssue, the vegetati ons of the nomencl ature. A rare heredi tary form i s
known as f ami l i al beni gn chroni c pemphi gus (or Hai l eyHai l ey di sease). In thi s condi ti on
the hi stol ogy of the l esi ons cl osel y resembl es that of pemphi gus proper, wi th marked
acanthol ysi s. The i mmunol ogi cal fi ndi ngs are negative. Thi s f orm i s, however, a much l ess
aggressi ve di sease than pemphi gus and runs a protracted chroni c course. The oral l esi ons
P. 131
cl osel y resembl e those of pemphi gus and the rare occurrence of thi s vari ant shoul d be borne
i n mi nd i n di f ferenti al di agnosi s.
Oral mucosal i nvol vement i n pemphi gus vul gari s i s common and frequentl y precedes
i nvol vement at other si tes.
Clinical presentation
The cl i ni cal pi cture i n pemphi gus i s of wi despread bul l ae formati on on the ski n and mucous
membranes. The bul l ae, even of the ski n, are fragi le and break down rapi dl y to f orm crusted,
eroded l esi ons (Fi g. 11. 7). The l esi ons of mucous membranes are even more fragi l e and
rapi dl y break down wi th the f ormati on of i rregul ar ul cers, often wi th a ragged edge as a
resul t of the spl i t and fragi l e epi thel i um. The oral l esi ons may occur i n any si te wi thi n the
mouth and oropharynx and may be accompani ed by si mi l ar l esi ons of other mucosae. The
vul va i s often i nvol ved i n thi s way.
Immunopathology (Table 11.4)
Bul l ae are produced as a resul t of acanthol ysi sthe breakdown of the i ntercel l ul ar
connecti ons, usual l y i n the stratum spi nosum of the epi thel i um. Immunopathol ogy shows the
presence of autoanti bodi es di rected agai nst the epidermal and i ntercel l ul ar materi al . The
autoanti body i s usual l y an IgG and compl ement i s also fi xed at the si te (Tabl e 11. 4). The
target anti gens are now recogni zed to be desmogl ei n I and desmogl ei n III, transmembrane
gl ycoprotei ns bel ongi ng to the cadheri n fami l y of adhesi on mol ecul es.
Management
Pati ents f requentl y present wi th a severel y ul cerated mouth wi th very l i ttl e normal mucosa,
between the l esi ons. Bi opsy may prove to be di ffi cul t as the epi thel i um i s readi l y detached
from the underl yi ng mucosa and i t i s i mportant that peri l esi onal ti ssue i s obtai ned. The
fragi l i ty of the epi thel i um may be confi rmed i n some pati ents by the posi ti ve Ni kol sky si gn;
the epi thel i um can be detached by l ateral pressure. Immunol ogi cal methods are now
empl oyed for the di agnosi s of pemphi gus, as autoanti bodi es are found i n both the affected
Fig. 11.7 Mul ti pl e ruptured bul l ae of the l abi al mucosa and tongue i n oral pemphi gus
vul gari s.
oral (and ski n) ti ssue and ci rcul ati ng i n the pati ent' s serum. Di rect i mmunofl uorescence
(IMF) of the

peri l esi onal oral ti ssue demonstrates IgG-cl ass anti bodi es bi ndi ng to the i ntercel l ul ar
substances and cel l membranes i n the stratum spi nosum of affected epi thel i um. Posi ti ve
di rect IMF i s essenti al for di agnosi s. Ci rcul ati ng autoanti bodi es are demonstrated by i ndi rect
i mmunofl orescence i n approxi matel y 90 per cent of pati ents wi th pemphi gus vul gari s.
The treatment of pemphi gus i s a mul ti di sci pl i nary matter, i nvol vi ng oral physi ci ans,
dermatol ogi sts, and other medi cal speci al i ti esas requi red. The parti cul ar rol e of the oral
P. 132
Table 11.4 The immunopathology of bullous diseases
Disease Direct IMF:
site
Indirect IMF*
(circulating
autoantibodies)
Target antigens
Pemphi gus Epi thel i al -
bound, IgG, C3:
Intercel l ul ar
Posi ti ve IgG (90%) Desmogl ei n I
and III
Bul l ous
pemphi goi d
Li near, IgG C3:
BM zone
Posi ti ve IgG (75%) Bul l ous
pemphi goi d
anti gens, BP180
and BP230
Mucous
membrane
pemphi goi d
Li near IgG, IgA
C3: BM zone
Posi ti ve IgG (75%),
IgA (50%)
BP180 i n the
majori ty;
l ami ni n 5; 4
i ntegri n
Li near IgA
di sease
Li near, IgA, C3:
BM zone
Posi ti ve IgA (30%
of adul ts)
A number of
target anti gens
i ncl udi ng BP180
Dermati ti s
herpeti f ormi s
Granul ar, IgA,
C3: ti ps of
dermal papi l l ae
Negati ve for
epi thel i al
autoanti bodi es
Unknown
Epi dermol ysi s
bul l osa
acqui si ta
Li near IgG, C3 Occasi onal l y dermal
bi ndi ng IgG
Type 7 col l agen
*The presence of ci rcul ati ng autoanti body depends on ski n substrate used, i . e.
monkey oesophagus for pemphi gus and sal t-spl i t ski n f or pemphi goi d and l i near IgA
di sease.
BM, basement membrane.
physi ci an i s, f i rst of al l , to enabl e earl y di agnosi s by the recogni ti on of the oral l esi ons and,
l ater, to hel p i n the management of the often very severe oral di sease. The prognosi s has
been compl etel y al tered by the i ntroducti on of systemi c steroi ds and many pati ents may l ead
reasonabl e l i ves mai ntai ned on substanti al doses of predni sol one, often wi th the use of
azathi opri ne as a steroi d-spari ng drug. Very hi gh dosages are used i ni ti al l y to suppress bul l a
formati on (of the order of 1 mg/kg predni sol one dail y), but thi s may often be sl owl y reduced
to a mai ntenance dose of 15 mg dai l y or thereabouts. Other steroi d-spari ng,
i mmunosupressi ve drugs such as cycl ophosphami de or ci cl ospori n have al so been used to
treat pemphi gussevere cases may requi re pl asmapheresi s. The ti tre of ci rcul ati ng
anti bodi es refl ects the di sease severi ty and i s therefore a usef ul gui de f or therapy.
Systemi c steroi d therapy may be suppl emented by hi gh-concentrati on steroi d mouthwashes,
si nce the oral mucosa i s often l ess responsi ve to treatment than the ski n. Just as the oral
mucosa seems to be parti cul arl y prone to attack by the anti bodi es earl y i n the course of the
di sease, i t al so often remai ns qui te severel y affected when the ski n l esi ons are i n remi ssi on.
Thi s may be the case even when the l evel of the anti body ti tre i s unmeasurabl y l ow. Long-
term, topi cal steroi d therapy may be requi red, suppl emented by such measures as anti fungal
therapy and the use of anaestheti c mouthwashes as necessary. Oral hygi ene may present a
great probl em and care must be taken i f the teeth are not to be l ost, an i mportant dental
factor, si nce the weari ng of dentures may be di ffi cul t, i f not i mpossi bl e.
Pemphigoid
Thi s group of i mmunobul l ous di sorders i s characterized by the f ormati on of subepi dermal
bul l ae and the presence of i mmunoreactants at the basement membrane zone. There are two
broad cl i ni cal subtypes of thi s condi ton: the fi rst, whi ch predomi nantl y affects the ski n wi th
occasi onal mucosal i nvol vement, i s ref erred to as bul l ous pemphi goi d (BP). There i s a f urther
cutaneous subgroup occurri ng i n pregnancy known as pemphi goi d gestati oni s. The second
subtype predomi nantl y i nvol ves the mucous membranes wi th onl y occasi onal ski n
i nvol vement and i s now referred to as mucous membrane pemphi goi d (MMP)thi s was
formerl y referred to as beni gn mucous membrane pemphi goi d or ci catri ci al pemphi goi d.
Bullous pemphigoid
The pati ents wi th pemphi goi d are, i n the mai n, el derl y, most bei ng over the age of 60 years,
al though some very f ew may be consi derabl y younger. As i n pemphi gus, the gender
di stri buti on i s even. There i s no raci al factor i nvol ved. Bul l ous pemphi goi d i s the most
common i mmunobul l ous di sease i n Western Europe.
The condi ti on starts wi th a pruri ti c, someti mes urti cari al rash, often on the l i mbs but i t may
al so i nvol ve the trunk. Wi thi n these erythematous areas devel op tense bul l ae at an i nterval
varyi ng from a f ew days to a few weeks. Bul l ae may remai n l ocal i zed to the l i mbs or may
become more general i zed. They are f l ui d-fi l l ed but may l ater become haemorrhagi c. Mucous
membrane l esi ons are usual l y conf i ned to the mouth and occur i n up to 20 per cent of
pati entsi n some cases the gi ngi vae are i nvol ved (Fi g. 11. 8). Intraoral l y, i n contrast to
pemphi gus vul gari s, bl i sters may occasi onal l y be seen. The cl i ni cal course of BP i s usual l y
l ess severe than that of pemphi gus wi th many pati ents havi ng a resol uti on wi thi n 2 to 5
years. Neverthel ess, whi l e pati ents wi th l ocal i zed di sease may be control l ed adequatel y wi th
topi cal corti costeroi d creams, pati ents wi th more wi despread l esi ons wi l l requi re systemi c
i mmunosuppressi on. In thi s el derl y popul ati on, patients are at a parti cul ar ri sk from the
compl i cati ons of systemi c drug therapy.
IMMUNOPATHOLOGY
The bl i sters i n bul l ous pemphi goi d are subepi dermal and may therefore remai n i ntact f or a
number of days. Di rect i mmunofl uorescence on bi opsy materi al shoul d demonstrate IgG or C3
i mmunoreactants i n a l i near di stri buti on al ong the basement membrane zone (Fi g. 11. 9).
Ci rcul ati ng anti -basement membrane zone IgG autoanti bodi es (75 per cent of pati ents) and
occasi onal IgA may al so be detected by i ndi rect i mmunofl uorescence techni ques to bi nd on to
the basement membrane of the epi thel i um (Tabl e 11. 4). Li near deposi ti on of IgA anti bodi es
al one, i n the context of a predomi nantl y cutaneous bl i steri ng di sease, i s more suggesti ve of
l i near IgA di sease. The mai n target anti gens i n bul l ous pemphi goi d are the BP230 and BP180
anti gens. These are consi dered to be i mportant protei ns i n mai ntai ni ng the structural
i ntegri ty of the basement membrane, that i s, i n provi di ng cohesi on of the dermi s to the
epi dermi s.
MANAGEMENT
Di agnosi s i s based upon a combi nati on of cl i ni cal si gns (ski n and oral ) and
i mmunofl uorescent fi ndi ngs. To i mprove the success of

IMF techni ques, a peri l esi onal bi opsy i s essenti al . Wi th the use of systemi c and topi cal
steroi ds most pati ents can be kept i n reasonabl e comfort. Steroi ds can, i n most cases of
bul l ous pemphi goi d, be reduced to a mi ni mum or compl etel y tai l ed of f, as most pati ents wi l l
eventual l y achi eve remi ssi on, tai l ed of f after 36 years (unl i ke pemphi gus). For l ong-term
use of steroi ds, addi ti onal i mmunosuppressi ve agents such as azathi opri ne shoul d be added
as steroi d-spari ng agents. Dapsone may be an al ternati ve fi rst-l i ne agent i n BP. Topi cal
steroi d preparati ons and anal gesi c mouthwashes may be benefi ci al for oral l esi ons.
Fig. 11.8 Desquamati ve gi ngi vi ti s i n a pati ent wi th bul l ous pemphi goi d.
P. 133
Mucous membrane pemphigoid (MMP)
Thi s compri ses a heterogeneous group of di sorders that may be di sti ngui shed f rom cl assi cal
bul l ous pemphi goi d by havi ng a predi l ecti on for mucosal si tes and a tendency to resul t i n
fi brosi s.
Mucous membrane pemphi goi d i s a cl i ni cal l y heterogeneous di sease, whi ch may present to a
wi de range of speci al i sts i ncl udi ng oral physi ci ans, dermatol ogi sts, ophthal mol ogi sts,
gynaecol ogi sts, and ENT speci al i sts. Whi l e the majori ty of pati ents have oral l esi ons, wi th
addi ti onal mucosal si tes frequentl y i nvol ved, there are subgroups of pati ents wi th di sease
l i mi ted to one si te, for exampl e, pure ocul ar pemphi goi d or pure oral pemphi goi d. Ski n
l esi ons are i nfrequent and are present i n 1025 per cent of cases, usual l y appeari ng on the
face, neck, or scal p.
Intraoral l y, i ntact bul l ae may someti mes be present, for exampl e, on the gi ngi vae or soft
pal ate. More often pati ents present wi th ul cerated areas of mucosa i nvol vi ng the buccal ,
pal atal , or occasi onal l y l i ngual or l i p mucosa (Fi g. 11. 10). Desquamati ve gi ngi vi ti s i s a
frequent presentati on and may be l ocal i zed or general i zed. The di f ferenti al di agnosi s of thi s
i ncl udes l i chen pl anus and pemphi gus. Intraoral scarri ng may occur i n MMP but i t may be a
si gni f i cant fi ndi ng i n other mucosal si tes such as the conjuncti va or i n the ski n. The term
ci catri ci al pemphi goi d has thus been f avoured i n the past for thi s subgroup of
pemphi goi d and remai ns the preferred term i n the ophthal mol ogy l i terature.
Onset of MMP vari es from under 30 years of age to over 70, but i s more common i n l ate
mi ddl e to ol d age (5070 year age group). There i s a 2:1 preponderance of femal e
pati ents. The frequency and extent of bl i steri ng vary f rom vi rtual l y conti nuous and mul ti pl e,
to i ntermi ttent and si ngl e. The bul l ae, when establ i shed, are i n general pai nl ess, but there
may be di scomf ort when the bul l a i s f ormi ng and af ter rupture.
Fig. 11.9 Immunofl uorescent demonstrati on of IgG-group anti body compl exes al ong the
basal zone i n pemphi goi d.
Mucous membrane pemphi goi d may present as a desquamati ve gi ngi vi ti s.
In a consi derabl e proporti on of pati ents (as much as 75 per cent) the conjuncti va are
eventual l y i nvol ved and, si nce the l esi ons i n thi s si te heal wi th scarri ng, vi si on may be
aff ected. A combi nati on of subconjuncti val f i brosi s l eadi ng to adhesi ons of the conjuncti va,
symbl epharon formati on (adhesi on of bul bar conjuncti va to the eye-l i d), and l oss of the tear
fi l m resul ts i n opaci fi cati on of the cornea and bl indness.
Al l pati ents di agnosed as havi ng mucous membrane pemphi goi d shoul d be ref erred to an
ophthal mol ogi st, as earl y treatment of ocul ar i nvol vement, whi ch may be asymptomati c, i s
essenti al .
Ocul ar i nvol vement i n mucous membrane pemphi goi d i s common and may l ead to bl i ndness.
Al l pati ents shoul d be referred for ophthal mol ogi cal assessment.
Other mucous membranes such as vul val , nasal , pharyngeal , l aryngeal , oesophageal , and
anogeni tal may be af fected. Pati ents shoul d therefore be asked about symptoms (such as
hoarseness, dysphagi a, and geni tal di scomfort) that mi ght i ndi cate i nvol vement of other
mucous membranes.
IMMUNOPATHOLOGY
As i n bul l ous pemphi goi d, the oral bl i sters are subepi thel i al . Di rect i mmunofl uorescence on
unf i xed, f resh bi opsy materi al shows IgG, IgA, or C3 i n a l i near di stri buti on al ong the
basement membrane zone. Not al l pati ents wi th the cl i ni cal di sease have posi ti ve
i mmunofl uorescence.
Immunopathol ogi cal f i ndi ngs concerni ng the detecti on of ci rcul ati ng anti -basement
membrane zone autoanti bodi es i n MMP have shown wi de vari ati on i n the past and i n many
previ ous

studi es autoanti bodi es were i nfrequentl y detected. However, wi th the use of a battery of ski n
substrates and, i n parti cul ar, wi th the use of sal t-spl i t ski n substrate, detecti on of both IgG
Fig. 11.10 Ruptured gi ngi val bul l ae i n mucosal pemphi goi d.
P. 134
and IgA ci rcul ati ng anti bodi es i n MMP has consi derabl y i ncreased (see Tabl e 11. 4). Mol ecul ar
techni ques have recentl y demonstrated that autoanti bodi es target speci fi c protei ns i n the
basement membrane. The majori ty of sera react to the bul l ous pemphi goi d anti gen, BP180.
However, addi ti onal subgroups react to al mi ni n 5 and 4 i ntegri n (ocul ar subgroup).
MANAGEMENT
Treatment of oral i nvol vement i s i ni ti al l y on a l ocal basi s, usi ng topi cal steroi ds i n a
mouthwash, spray or i ncorporated i n a paste. Symptomati c treatment f or the rel i ef of oral
erosi ons caused by the perforati on of the bul l ae may al so be necessary. Pati ents wi th
recurrent mul ti pl e bul l ae may need l ong-term steroid mouthwashes. Of ten, however, control ,
even wi th hi gh concentrati ons of l ocal steroi ds, i s i nadequate. Dapsone has been advocated
as a second-l i ne treatment f or mi l d to moderate mucosal pemphi goi d. It i s, i n general , a
wel l -tol erated drug, but may cause a haemol yti c anaemi a. Therefore, careful moni tori ng of
the pati ent' s bl ood count i s requi red. In the most severe cases of mucosal di sease, for
exampl e, i n the presence of acti ve conjuncti val i nfl ammati on and scarri ng, systemi c steroi ds
combi ned wi th a steroi d-spari ng agent such as azathi opri ne or cycl ophosphami de may be
requi red.
The cl i ni cal course of MMP i s vari abl e but for the majori ty of pati ents i t i s a chroni c di sorder
that rel apses and remi ts over many years. For those wi th mi l d di sease l i mi ted to the oral
mucosa, the condi ti on may burn out af ter a f ew years. Among these pati ents i n
whom, for exampl e, mi ni mal desquamati ve gi ngi vi ti s or bul l ae occur once every few months,
oral di scomfort may be rel i eved by the i ntermi ttent use of topi cal steroi ds and anti septi c or
anal gesi c mouthwashes al one. For the remai nder of pati ents attendi ng oral medi ci ne cl i ni cs
wi th mul ti si te di sease or severe oral di sease, l ong-term dapsone may be requi red wi th
occasi onal addi ti onal short courses of systemi c steroi ds. It i s i mportant to appreci ate that
some of these pati ents may requi re mul ti di sci pl i nary management to achi eve opti mal care.
Oral immunobullous diseases: summary
It shoul d perhaps be added that the concept of ri gid cl assi fi cati ons of bul l ous di seases,
cl earl y defi ned on cl i ni cal and hi stol ogi cal grounds, i s to some extent gi vi ng way to the i dea
that overl ap condi ti ons may occur. Thi s recogni ti on by dermatol ogi sts of a spectrum of
presentati on of bul l ous ski n di sease i s paral l el ed by si mi l ar observati ons i n the case of oral
l esi ons. Al though the great majori ty of these may be cl assi f i ed wi th some degree of
confi dence i nto one or other of the accepted di sease patterns, there remai n some l esi ons
that both cl i ni cal l y and hi stol ogi cal l y seem equi vocal .
Dermatitis herpetiformis
Dermati ti s herpeti formi s i s an uncommon bul l ous ski n di sease, i nvari abl y associ ated wi th
gl uten enteropathy. The cutaneous presentati on i s of groups of i tchy vesi cl es that
characteri sti cal l y affect the knees, el bows, and buttocks. Two types of oral l esi on have been
descri bedone consi sti ng of fragi l e vesi cl es and the second of keratoti c patches cl i ni cal l y
somewhat resembl i ng l i chen pl anus. However, ski n and oral l esi ons share the same
characteri sti c i mmunopathol ogi cal f eaturesof granul ar l gA deposi ts l ocal i zed to the apex
of the papi l l ae of the l ami na propri a (Tabl e 11. 4).
Linear IgA disease
A menti on shoul d be made of l i near IgA di sease, a rare bul l ous di sease of ski n that cl i ni cal l y
overl aps wi th dermati ti s herpeti formi s and bul l ous pemphi goi d. There are two types of l i near
IgA di sease: one affecti ng chi l dren (chroni c bul l ous di sease of chi l dhood) and the other
adul ts (adul t l i near IgA di sease). Ski n l esi ons are cl assi cal l y seen as tense bul l ae on the
trunk, l i mbs, or scal p, someti mes arranged i n groups known as rosettes. In many
pati ents the cl i ni cal pi cture i s di sti ngui shabl e f rom that of bul l ous pemphi goi d. Oral l esi ons
appear to be uncommon but are si mi l ar to those seen i n pemphi goi d. As the name suggests,
the characteri sti c i mmunohi stol ogi cal feature i s of l i near deposi ts of IgA at the basement
zone (Tabl e 11. 4). The IgA anti bodi es are di rected at a number of target anti gens, i ncl udi ng
the bul l ous pemphi goi d anti gen, BP180. Thi s fi ndi ng emphasi zes the broad overl ap between
the subepi dermal bl i steri ng di seases. Dapsone i s the systemi c drug of choi ce for the
treatment of l i near IgA di sease.
Epidermolysis bullosa
In these groups of di sorders there i s ski n and mucosal f ragi l i ty wi th bl i steri ng fol l owi ng
mechani cal traumahence they are someti mes ref erred to as mechanobul l ous
di seases.
Inherited forms
Inheri ted f orms of epi dermol ysi s bul l osa (EB) compri se a rare but seri ous group of bl i steri ng
di sorders, of whi ch over 20 types have been reported. They are, however, hi ghl y unl i kel y to
be di agnosed on the basi s of oral symptoms al one but may be of great si gni f i cance to dental
treatment.
The vari ous types of EB have been di vi ded i nto three mai n subgroups, based on the
hi stol ogi cal l evel of bul l a formati on, the mol ecul ar basi s of the defect, and mode of
i nheri tance (Tabl e 11. 5).
The predomi nati ng f eature i s extreme fragi l i ty of the epi thel i um, the resul t of bul l ae
formati on occurri ng ei ther spontaneousl y or as a response to mi ni mal degrees of trauma. In
the si mpl ex form of the di sease there i s l ess severe bul l a formati on and the mucous
membranes and teeth are rarel y i nvol ved. Many pati ents onl y have bl i sters on the sol es of
thei r feet. In the l ethal juncti onal and dystrophi c type of EB, there i s extensi ve i nvol vement
of mucous membrane and the oral mucosa i s extremel y f ragi l e. Bul l ae break down to form
pai nful erosi ons and there i s scar f ormati on. In the juncti onal type of EB the aff ected chi l d at
bi rth presents wi th an extreme degree of fragi l i ty of the ski n and mucous membranes that i s
i ncompati bl e wi th l i fe, and death i s

common i n i nf ancy. In the nonl ethal j uncti onal type, there i s extensi ve ski n and mucosal
bl i steri ng but scarri ng i s much l ess severe than i n recessi ve dystrophi c EB.
P. 135
Table 11.5 Subgroups of epidermolysis bullosa
Clinical features Site of blister Inheritance
Si mpl ex epi dermol ysi s bul l osa
Ski n bl i sters at bi rth, mai nl y i nduced by
fri cti on.
Oral i nvol vement absent or mi l d. Teeth
normal
Basal cel l s Mai nl y
autosomal
domi nant
Juncti onal epi dermol ysi s bul l osa
Herl i tz (l ethal ) form resul ts i n extensi ve Lami na l uci da Al l autosomal
In recessi ve dystrophi c EB, bul l ae are seen at, or soon after, bi rth. The l esi ons are produced
i n response to the most mi nor degrees of trauma and eventual l y heal wi th scar formati on,
thi s l eadi ng eventual l y to gross ti ssue def ormi ty, parti cul arl y of the extremi ti es. The oral
mucous membrane i s equal l y suscepti bl e to damage and, as a resul t of the repeated scar
formati on, openi ng of the mouth may become greatl y restri cted and the tongue bound down.
The essenti al pathol ogy i n thi s form of the di sease i s dermal ; the f ragi l i ty of the ti ssues i s
due to defi ci ent col l agen f ormati on i n the subepi thel i al structures. Thi s si tuati on i s paral l el ed
i n the teeth, there bei ng abnormal i ti es i n denti ne formati on that l ead to hypopl asi a and a
hi gh suscepti bi l i ty to cari es. Si nce conservati ve dental treatment or even ef fecti ve oral
hygi ene measures may be al most i mpossi bl e i n these pati ents, the resul ti ng grossl y cari ous
teeth, associ ated wi th a restri cted access and extreme mucosal fragi l i ty, present a major
probl em to the dental surgeon. It has al so been reported that the oral scars have, i n some
pati ents, been fol l owed by the onset of l eukopl aki a and, f i nal l y, carci noma, but thi s cannot
be regarded as a characteri sti c of the di sease.
In epi demol ysi s bul l osa the oral mucosa and teeth may be af fected.
Epidermolysis bullosa acquisita
Epi dermol ysi s bul l osa acqui si ta i s a rare autoi mmune subepi dermal bul l ous di sorder i nvol vi ng
the ski n and mucous membranes. There are two broad cl i ni cal subgroupsan i nfl ammatory
type remi ni scent of BP and a mechanobul l ous subtype i n whi ch bul l ae occur i n response to
trauma and l esi ons are therefore most promi nent on the knees el bows, hands, and feet. Oral
l esi ons, where present, may vary i n severi ty f rom mi l d desquamati ve gi ngi vi ti s to severe
general i zed ul cerati ve and of ten scarri ng mucosal invol vement. Di rect i mmunofl uorescence i s
posi ti ve f or l i near basement membrane IgG and C3. On sal t-spl i t ski n, ci rcul ati ng IgG
anti bodi es bi nd to the base of the spl i t correspondi ng wi th the target anti gen, type 7
col l agen. Treatment i s often very unsati sfactory despi te systemi c i mmunosuppressi on, and
pati ents f requentl y devel op promi nent mucosal and ski n scarri ng.
Erythema multiforme
Erythema mul ti forme (EM) i s an acute vesi cul obul l ous di sease of ski n and mucous
membranes wi th a wi de range of cl i ni cal presentati onshence the term mul ti forme.
It may be preci pi tated by a range of f actors, i ncl udi ng i nfecti ons (parti cul arl y vi ral ), drugs of
vari ous ki nds, neopl asi a, and pregnancy (Tabl e 11. 6). In l ess than hal f of the cases no such
ski n and mucosal i nvol vement, dental
abnormal i ti es andof ten wi th death i n
i nfancy. The nonl ethal form produces
wi despread ski n and vari abl e mucosal
i nvol vement
recessi ve
Dystrophi c epi dermol ysi s bul l osa
Domi nant form i s often mi l d. Recessi ve
form i s very severe wi th extensi ve
bl i sters and scarri ng of ski n, l oss of
nai l s, severe oral mucosal bl i steri ngand
scarri ng, and hypopl asti c teeth
Immedi atel y bel ow
the l ami na densa of
the basement
membrane
Domi nant
and recessi ve
forms
i nduci ng factor i s f ound. In the most ful l y devel oped form (al ternati vel y known as the
StevensJohnson syndrome), there i s wi despread i nvol vement of the ski n and mucous
membranes but, i n the more usual restri cted f orm, the oral mucous membrane i s mai nl y
i nvol ved, wi th no more than mi nor l esi ons i n other si tes. The pati ents are predomi nantl y
young adul ts, mal es bei ng affected more frequentl y than femal es.
Erythema mul ti forme i s an acute vesi cul obul l ous di sease of ski n and mucous membranes.
The mai n f eature of an attack i s the sudden devel opment of wi despread erosi ons of the oral
mucosa, characteri sti cal l y i nvol vi ng the l i ps. The erosi ons are produced by the di si ntegrati on
of subepi dermal bul l ae, l esi ons that onl y rarel y l ast l ong enough to become a di agnosti c
feature. The erosi ons on the l i ps (especi al l y the lower l i p) are accompani ed by crusti ng and
bl eedi ng and are, i f not absol utel y di agnosti c, strong poi nters to the nature of the condi ti on
(Fi g. 11. 11). There i s often a cervi cal l ymphadeni ti s wi th

pyrexi a and the pati ent feel s unwel l . The accompanyi ng ski n l esi ons, when present, may have
a characteri sti c target (or i ri s-l i ke) appearance that i s di agnosti c. Ocul ar
i nvol vement, i f present, may resul t i n conjuncti val damage but thi s i s unusual . An attack
gradual l y subsi des after some 10 days, but there i s a consi derabl e l i kel i hood of recurrence of
EM after a peri od varyi ng from no more than a few weeks to a year or so. Thi s i s more l i kel y
i f EM i s associ ated wi th recurrent herpes si mpl ex vi rus (HSV) i nfecti on.
P. 136
Table 11.6 Some precipitating factors in erythema
multiforme
Inf ecti ons
Herpes si mpl ex vi ral (HSV) i nfecti ons
Mycopl asma pneumoni a (rarel y)
Drugs
Sul f onami des
Anti convul sants
Pregnancy
Recurrent erythema mul ti forme i s associ ated parti cul arl y wi th HSV i nfecti on and pati ents
may requi re l ong-term prophyl acti c aci cl ovi r.
The i ni ti al di agnosi s i s enti rel y cl i ni cal , the i mportant di ff erenti al di agnosi s bei ng from a
pri mary herpeti c stomati ti s. In the case of a recurrent attack, however, herpeti c stomati ti s
may be confi dentl y excl uded si nce thi s i s an i sol ated event i n i mmunocompetent i ndi vi dual s.
Si mi l arl y, a hi story of recurrent herpes si mpl ex renders the di agnosi s of herpeti c stomati ti s
unl i kel y. The i nvol vement of the l i ps i s a strong indi cati on of the di agnosi s of erythema
mul ti forme and the presence of target l esi ons of the ski n can be taken as al most
concl usi ve evi dence for the di agnosi s. Bi opsy of the oral l esi ons shows a rather non-speci f i c
hi stol ogi cal pi cture wi th degenerati ve changes i n the epi thel i um and subepi thel i al bul l a
formati on. Because of the acute cl i ni cal features, however, the di agnosi s does not often
depend on the hi stol ogi cal appearance of the l esi ons. Other i mmunobul l ous di seases may
need to be excl uded by i mmunof l uorescence studi es.
Treatment of the cases restri cted to the mouth depends on the use of l ocal or systemi c
steroi ds, to whi ch there i s usual l y a rapi d response. A steroi d mouthwash i s l i kel y to gi ve
symptomati c rel i ef and ef fecti vel y reverse the process i n a few days. In the more severe
cases (parti cul arl y when other mucous membranes are i nvol ved), when the ski n or oral
l esi ons are severe or when the eyes are af fected, a short course of systemi c steroi ds may be
necessary to shorten the attack. The use of systemi c steroi ds for EM remai ns controversi al .
However, a course of 40 mg predni sol one dai l y for 14 weeks, rapi dl y reduci ng over the
next f ew weeks, may be benef i ci al for symptomati c rel i ef. A topi cal or systemi c anti fungal
agent may al so be requi red. Such pati ents may be suf fi ci entl y i l l to requi re hospi tal
admi ssi on, parti cul arl y i f they become dehydrated. Pati ents wi th recurrent EM requi re l ong-
term prophyl acti c aci cl ovi r.
Some authori ti es have suggested that EM and StevensJohnson syndrome are di sti nct
cl i ni cal di sorders, on the basi s of thei r cl i ni cal features and speci f i c causes. However, others
have chal l enged thi s concept as some pati ents wi th EM and mi l d symptoms may subsequentl y
devel op severe attacks, necessi tati ng hospi tal admissi on.
Idiopathic oral blood blisters (angina bullosa
haemorrhagica)
Some pati ents devel op spontaneous bl ood-fi l l ed bul l ae (bl ood bl i sters) of the oral
Fig. 11.11 Crusted l esi ons of the l i ps i n erythema mul ti forme.
mucosa from ti me to ti me. These have been descri bed under the rather unfortunate and
i nappropri ate ti tl e of angi na bul l osa haemorrhagi ca. The usual pattern i s that the
pati ent feel s a sharp pri cki ng sensati on i n the mouth (most usual l y on the pal ate) and fi nds
that a bl ood-fi l l ed bl i ster has suddenl y devel oped. Thi s most commonl y occurs when the
pati ent i s eati ng. The bul l ae may be qui te l arge (up to 23 cm i n di ameter) and the pati ent
may be i n consi derabl e f ear of obstructi on. The bl i ster eventual l y ruptures or i s perforated by
the pati ent and heal i ng occurs uneventf ul l y.
These pati ents have no demonstrabl e abnormal i ty of the bl ood-cl otti ng mechani sm, al though
pati ents wi th thrombocytopeni a may al so devel op bl ood bl i sters. Both mal e and femal e
pati ents have been descri bed, over a wi de age range. The method of formati on of the bl i sters
i s not known. It i s specul ated that the basi c mechani sm i s of bl eedi ng from the capi l l ary bed
bel ow a basal zone that i s for some reason weakened. Thi s may i ndeed be the mechani sm,
but the reasons f or i t are far from cl ear.
The pati ent' s hi story i s often suggesti ve of the di agnosi s, but i t i s i mportant to excl ude an
i mmunobul l ous condi ti on and al so to carry out a ful l bl ood count and cl otti ng screen.
Perforati on of a l arge, i ntact, bl i ster to rel ease the contents may be necessary and anti septi c
or anal gesi c mouthwashes can be prescri bed. Often, however, onl y a ruptured bul l a i s
presented for exami nati on, the pati ent havi ng perf orated i t as a fi rst measure. No preventi ve
treatment i s known.
Connective tissue diseases
Thi s i s a compendi um term used to descri be a number of di seases wi th a si mi l ar, but by no
means i denti cal i mmunol ogi cal background. They are not ski n di seases, but there are ski n
l esi ons i n a number of them, and i t i s common practi ce to group them wi th the ski n di seases
for descri pti ve purposes. The group i ncl udes Sjgren' s syndrome and rheumatoi d arthri ti s,
whi ch do not have speci f i c ski n l esi ons. These are di scussed i n Chapters 8 and 15. Apart from
these two condi ti ons, l upus erythematosus has i mportant oral mani f estati ons as do mi xed
connecti ve ti ssue di sease and systemi c scl erosi s, al though these l ast two are rel ati ve
rari ti es.

Lupus erythematosus
The group of di seases i ncl uded under thi s headi ng presents wi th a wi de range of symptoms,
but al l resul t from abnormal i ti es of the connecti ve ti ssues brought about by an autoi mmune
process. Two mai n cl i ni cal enti ti es are recogni zed, al though there are many vari ati ons. These
are systemi c l upus erythematosus (SLE) and di scoi d l upus erythematosus (DLE).
Systemic lupus erythematosus
SLE tends to occur i n adul t l i fe and femal es are aff ected much more than mal es. In thi s
condi ti on, there are wi despread changes i n the connecti ve ti ssues wi th secondary effects i n
the cardi ovascul ar, muscul oskel etal , and other systems, as wel l as i n the ski n. Cutaneous LE
cl assi cal l y presents as a photosensi ti ve erupti on of the f ace (butterfl y-pattern) and hands.
The course of the di sease vari es from a rel ati vel y mi l d chroni c condi ti on to a rapi dl y fatal
process, and an equal l y wi de range of ski n reacti ons may occur. These are paral l el ed by an
equal l y di verse range of oral symptoms, the most commonl y descri bed bei ng superfi ci al
erosi ons and erythematous patches on the mucosa. It woul d seem very unl i kel y that the
i ni ti al di agnosi s of the di sease woul d be made on the grounds of oral l esi ons al one, but the
possi bi l i ty of such an aeti ol ogy f or unrecogni zed oral l esi ons shoul d be borne i n mi nd. In
parti cul ar, i t shoul d be remembered that most cl i nical descri pti ons of the oral l esi ons of
l upus vari ants resembl e those of l i chen pl anus. Hi stol ogi cal l y, al so, there i s a cl ose
resembl ance between these condi ti ons. The fi nal di agnosi s of SLE i s l i kel y to be made as a
resul t of the i mmune abnormal i ti es presenti n parti cul ar, a wi de (and vari abl e) range of
P. 137
anti nucl ear autoanti bodi es may be found i n the serum. In SLE, ci rcul ati ng anti bodi es to DNA
are al most al ways present and thi s i s the most si gni f i cant i mmunol ogi cal screeni ng test. If
there are ski n l esi ons, the hi stol ogy and i mmunofl uorescent fi ndi ngs on bi opsy are as i n
di scoi d l upus erythematosus (bel ow).
Just as a l i chenoi d reacti on may occur as a response to drugs, SLE may be preci pi tated i n the
same way and by an equal l y wi de range of drugs. Hydral l azi ne, used i n the management of
refractory hypertensi on, i s the most quoted exampl e, but other drugs i ncl ude beta-bl ockers,
anti convul sants, and qui ni di ne.
SLE may be preci pi tated by drugs.
Treatment of SLE i s essenti al l y wi th steroi ds, of ten requi red i n hi gh doses, and wi th the
addi ti on of steroi d-spari ng drugs such as azathi opri ne. The oral l esi ons may be both very
pai nful and di ff i cul t to treat. Hi gh-concentrati on steroi danti bi oti c mouthwashes may be
useful , together wi th such supporti ng measures as anal gesi c mouthwashes.
Discoid lupus erythematosus (DLE)
Thi s i s a much more restri cted form of the di sease, whi ch presents as a ski n di sorder and
wi thout the wi despread general i zed abnormal i ti es found i n the systemi c form. The ski n
l esi ons, whi ch resul t from degenerati ve changes i n the subepi thel i al connecti ve ti ssues,
present as scal y red patches that l ater heal wi th scar formati on. The face i s the area most
commonl y affected, and ci rcumscri bed l esi ons occur bi l ateral l y. Al opeci a can occur i f the
scal p i s i nvol ved. Fol l i cul ar pl uggi ng of the ski n i s an i mportant cutaneous feature to el i ci t
the di agnosi s. The pati ents are predomi nantl y female (femal e:mal e, 2:1), the age of
i nci dence bei ng wi del y di stri buted, but havi ng a peak i n the fourth decade of l i f e. The fi rst
appearance of the l esi ons may fol l ow some form of trauma (such as an unusual degree of
exposure to sunl i ght) and l ater exacerbati ons may fol l ow repeated trauma of thi s ki nd. Oral
l esi ons are found i n a consi derabl e proporti on of pati ents wi th DLE, al though the esti mated
i nci dence vari es very wi del y from 3 to 50 per cent accordi ng to the source quoted. Al though
l esi ons may be f ound on any part of the oral mucosa, the characteri sti c si te i s on the l i ps.
The l esi on starts as an area of erythema that devel ops to a thi ckened, rather crusted, l esi on
wi th a whi te margi n. The hi stol ogi cal appearances are of epi thel i al atrophy at the centre of
the l esi on wi th hyperkeratosi s at the margi ns wi th a cl ose resembl ance to the changes i n
l i chen pl anus. The f undamental di fference between the hi stol ogi cal f i ndi ngs i n l i chen pl anus
and i n LE (and other connecti ve ti ssue di seases wi th ski n and mucosal l esi ons) i s that the
subepi thel i al band of l ymphocytes, rel ati vel y evenl y di stri buted i n l i chen pl anus, has a
tendency to a fol l i cul ar di stri buti on i n DLE. Di rect i mmunofl uorescence i n LE gi ves vari abl e
resul ts wi th homogeneous or granul ar deposi ts of IgG, someti mes wi th IgM and compl ement
components, at the dermo-epi dermal juncti on or bel ow the basal zone. Ci rcul ati ng
autoanti bodi es are found i n approxi matel y one-thi rd of pati ents wi th ski n l esi ons of DLE.
Treatment of DLE i s often symptomati c wi th the use of potent topi cal steroi ds to suppress the
l esi ons. Parenteral treatment, oddl y enough, i s wi th the anti mal ari al drugs, such as
hydroxychl oroqui ne, whi ch may compl etel y suppress the symptoms but whi ch may al so
i ntroduce a wi de range of si de-eff ects, some mi nor and some more seri ous, for exampl e, the
producti on of corneal deposi ts and reti nopathy. The most si gni fi cant consi derati on, so far as
the oral l esi ons are concerned, i s the possi bi l i ty of mal i gnant change. It i s di f fi cul t to assess
the i nci dence of thi s f rom the publ i shed fi gures, but there i s no doubt that cases i nvol vi ng
mal i gnant transformati on i n l i p l esi ons have been documented. It i s, therefore, necessary to
observe the l esi ons on a l ong-term basi s.
Morphoea and systemic sclerosis
Morphoea i s a purel y cutaneous di sease, i n whi ch there i s a spontaneous appearance of a
scar-l i ke band or pl aque. Systemi c scl erosi s i s a mul ti system di sease i n whi ch there i s
wi despread fi brosi s of the ski n and gut, together wi th other organs. There may al so be other
el ements of connecti ve ti ssue di sease present, such as SLE and occasi onal l y Sj gren' s
syndrome (see Chapter 8). Femal es are more commonl y af fected and the earl i est feature i s
usual l y Raynaud' s phenomenon. Invol vement of the peri oral ti ssues can l ead to restri cted
mouth openi ng and di ff i cul ti es wi th oral hygi ene and dental treatment. Pati ents may devel op
an expressi onl ess mask-l i ke faci al expressi on.

In systemi c scl erosi s there may be restri cted mouth openi ng due to i nvol vement of the
peri oral ti ssues.
Wi deni ng of the peri odontal membrane space, parti cul arl y i n posteri or teeth i s the
characteri sti c radi ol ogi cal dental fi ndi ng. A vari ant of systemi c scl erosi s has been named the
CRST or CREST syndrome (C, cal ci fi cati on; R, Raynaud' s phenomenon; E, oesophageal
dysfuncti on; S, scl erodactyl y; T, tel angi ectasi a). Most pati ents wi th systemi c scl erosi s have
hi gh ti tres of anti nucl ear anti body (ANA), usual l y of the speckl ed vari ety, al though other
types can al so be f ound. Treatment of systemi c scl erosi s i s essenti al l y symptomati c.
Wi deni ng of the peri odontal membrane space i s a characteri sti c radi ol ogi cal feature of
scl eroderma.
Mixed connective tissue disease
Mi xed connecti ve ti ssue di sease i s an overl ap condi ti on i n whi ch a number of the
characteri sti cs of other di seases i n the group are found i n a si ngl e pati ent. Pati ents are
predomi nantl y f emal e and may have f eatures of SLE, systemi c scl erosi s, dermatomyosi ti s,
and pol ymyosi ti s. It i s a rare condi ti on, but of i nterest i n the present context si nce there are
a number of oral di agnosti c i ndi cators of the di sease. The f i rst i s a l i chen pl anus-l i ke
l esi on of the oral mucosa, but wi th a hi stol ogi cal appearance resembl i ng that of LE. The
second i s the i nvol vement of the tri gemi nal nerve in the neurol ogi cal changes that may occur
i n mi xed connecti ve ti ssue di sease. Thi s may l ead to a compl ai nt of f aci al anaesthesi a, due to
a tri gemi nal neuropathy. In addi ti on, mi xed connecti ve ti ssue di sease may be associ ated wi th
secondary Sjgren' s syndrome (Chapter 8). The most i mportant i mmunol ogi cal i ndi cator i n
thi s condi ti on i s the presence of anti nucl ear anti bodi es of the speckl ed type and hi gh l evel s
of a speci fi c anti body to RNAase-sensi ti ve, extractabl e nucl ear ri bonucl eoprotei n (RNP)
anti gen.
The most l i kel y di agnosi s of thi s l ady' s oral condi ti on i s l i chen pl anus i n i ts non-erosi ve,
reti cul ar f orm. She shoul d be speci f i cal l y asked about medi cati on, as l i chenoi d erupti ons can
be drug-i nduced, al though these may be uni l ateral . Femal e pati ents may be rel uctant to
report geni tal i nvol vement, i f present. The fl exor surfaces of the wri sts are cl assi cal si tes for
ski n l i chen pl anus. Inci si onal bi opsy of the oral mucosa i s advi sabl e to conf i rm the cl i ni cal
di agnosi s. Thi s l ady shoul d be reassured about the di agnosi s but advi sed about the need for
P. 138
Q1 What i s the most l i kel y di agnosi s of thi s l ady' s oral l esi ons?
Q2 Are her ski n symptoms rel ated?
Q3 What i nvesti gati ons woul d you carry out?
Q4 What advi ce and treatment woul d you gi ve to thi s l ady?
l ong-term fol l ow-up. The oral l esi ons are asymptomati c and, therefore, no treatment i s
requi red. A topi cal steroi d preparati on can be prescri bed for her ski n.
The hi story and cl i ni cal exami nati on are suggesti ve of an i mmunobul l ous condi ti on wi th
i nvol vement of the ski n and oral mucosa. Oral bi opsy, wi th di rect i mmunofl uorescence of
peri l esi onal ti ssue shoul d be arranged and bl ood taken for i ndi rect i mmunofl uorescence.
Bi opsy of the oral mucosa may be di ffi cul t because of the extreme ti ssue fragi l i ty and
extensi ve i nvol vement of the oral mucous membrane.
Pemphi gus vul gari s i s the most l i kel y di agnosi s i n thi s case and there i s a hi gher i nci dence
amongst the Jewi sh race. The pati ent' s age and hi story of oral l esi ons, precedi ng
i nvol vement of the ski n, together wi th a posi ti ve Ni kol sky si gn are more suggesti ve of
pemphi gus than bul l ous pemphi goi d. Mucous membrane pemphi goi d tends to affect an ol der
age group, parti cul arl y women, and does not commonly i nvol ve the ski n. The protracted
hi story of oral ul cerati on and cl i ni cal appearance of ski n l esi ons i s not consi stent wi th
erythema mul ti f orme, whi ch tends to be epi sodi c and mani fest as target l esi ons on the ski n.
The cl i ni cal di agnosi s of pemphi gus vul gari s i s confi rmed by the presence of autoanti bodi es
di rected agai nst epi dermal i ntercel l ul ar substance of the epi thel i um. Routi ne hi stol ogy i n
pemphi gus reveal s bul l ous formati on wi th acanthol ysi s. Posi ti ve di rect IMF wi l l demonstrate
i ntercel l ul ar deposi ti on of IgG and usual l y C3 i n the epi thel i um (the so-cal l ed fi shnet
or chi cken wi re appearance). Urgent treatment i s requi red and the pati ent i s
managed i n conjuncti on wi th a dermatol ogi st. Admi ssi on to hospi tal i s usual l y requi red.
Ini ti al l y hi gh doses of steroi ds are gi ven, wi th the l ater addi ti on of a steroi d-spari ng drug, i f
necessary. Anti f ungal medi cati on i s usual l y requi red. Anal gesi c mouthwashes, contai ni ng
l i gnocai ne, provi de symptomati c rel i ef and hel p the pati ent mai ntai n fl ui d and nutri ti onal
i ntake. Ski l l ed nursi ng care i s essenti al i f there i s wi despread ski n i nvol vement. Attenti on
shoul d be pai d to l ong-term mai ntenance of oral hygi ene. Dental treatment i s often di ffi cul t.
Oral mucosal i nvol vement occurs i n the dystrophi c and juncti onal types of epi dermol ysi s
bul l osa (EB). In chi l dren wi th the juncti onal type, there are often severe dental
abnormal i ti es. Hypopl asti c teeth have been descri bed i n pati ents wi th dystrophi c EB. Mi ni mal
trauma from toothbrushi ng and eati ng causes oral bul l ae and scarri ng, as does suckl i ng i n
neonates. As a resul t of repeated scarri ng, mouth openi ng becomes restri cted and the

tongue and l i ps may become i mmobi l e. Cracki ng at the corners of the mouth frequentl y
occurs.
Oral hygi ene i s di f fi cul t f or these chi l dren and i nadequate pl aque control , superi mposed on
def ecti ve teeth and a restri cted di et often resul ts i n rampant cari es. Physi cal access for
dental treatment becomes di f fi cul t because of scarri ng. Due to excessi ve ti ssue f ragi l i ty
aff ecti ng al l mucous membranes, l ocal and general anaesthesi a are probl emati cal and
extracti ons must be carri ed out wi th extreme care. Parents of affected chi l dren need to be
gi ven preventi ve advi ce concerni ng di et, oral hygi ene, and fl uori de suppl ementati on before
the deci duous teeth erupt. Regul ar dental revi ews and empatheti c support for parents are
essenti al . Most cases are treated by a speci al i st paedodonti st.
Project
Q1 How woul d you i nvesti gate and manage thi s gentl eman?
Q1 What are the orodental mani festati ons of thi s condi ti on? Di scuss the di ffi cul ti es you
mi ght encounter when provi di ng dental treatment for thi s boy.
P. 139
1. Fi nd out about the short- and l ong-term ef fects of systemi c steroi ds and how these may
be prevented and/or managed. Di scuss the i mpl i cati ons of provi di ng oral and dental
care for pati ents who are taki ng systemi c steroi ds.
> Tabl e of Cont ent s > 12 - Gast r oi nt est i nal di sease
12
Gastrointestinal disease
Problem case
Case 12.1
A 26-year-ol d femal e pati ent vi si ts your dental practi ce for the fi rst ti me si nce movi ng i nto
the l ocal i ty. She compl ai ns of soreness of the mouth and ul cerati on. Her medi cal hi story
reveal s that she has been di agnosed by her general practi ti oner (GP) as suf feri ng from
i rri tabl e bowel syndrome (IBS), for whi ch she takes anti spasmodi cs, to no eff ect. She has
recentl y noti ced i ncreasi ng bouts of bl oody di arrhoea and stomach pai n. You note that she i s
pal e and has cervi cal l ymphadenopathy. The angl es of the mouth are cracked. There i s
marked i rregul ari ty of both buccal mucosae wi th associ ated l i near ul cerati on. There are no
si gns of aphthous stomati ti s but you note smal l ti ssue tags i n the retromol ar regi on. The
pati ent i s extremel y anxi ous and has no energy at work. Her appeti te i s poor and she has
l ost over a stone i n wei ght over the l ast coupl e of months. Her GP has tol d her that her gut
probl ems are stress-rel ated and prescri bed some vi tami ns. The l ady wants to know why she
has recentl y devel oped ul cerati on of her mouth.
Introduction
Oral l esi ons may occur i n a number of gastroi ntestinal tract di seases. In a few, these are
pri mary oral l esi ons resembl i ng those of the l ower gut, but, i n al most al l , secondary l esi ons
may be i nduced by factors such as mal absorpti on. Not onl y the di sease process, but al so
surgi cal resecti on of parts of the gut may resul t in these secondary changes.
Some di seases of the gastroi ntesti nal tract are of parti cul ar si gni f i cance i n the fi el d of oral
medi ci ne and are di scussed bel ow.
Coeliac disease (gluten-sensitive enteropathy)
Coel i ac di sease i s a permanent i ntol erance to gl i adi nthe protei n component of wheat. It i s
a l i f el ong i nfl ammatory condi ti on of the gastroi ntesti nal tract that af fects the smal l i ntesti ne
i n geneti cal l y suscepti bl e i ndi vi dual s. Coel i ac di sease i s characteri zed by mal absorpti on due
to morphol ogi cal abnormal i ti es i n the smal l i ntestinal mucosa. These changes are reversi bl e
on wi thdrawal of gl uten from the di et. The fi rst detai l ed descri pti on of coel i ac di sease i n
chi l dren was gi ven i n 1887. Cl assi cal l y, coel i ac disease i s characteri zed by the devel opment
of di arrhoea, l oss of appeti te, and wasti ng when a weani ng di et contai ni ng gl uten i s
i ntroduced, together wi th secondary effects of mal absorpti on such as anaemi a. Coel i ac
di sease i s al so known as coel i ac sprue and i t i s i nteresti ng that the name
sprue was deri ved from the Dutch word f or aphthous ul cer, thi s i ndi cati ng the hi gh
Q1 Coul d thi s l ady' s oral condi ti on be rel ated to her gut symptoms?
Q2 What are the symptoms of IBS?
Q3 How woul d you manage thi s l ady?
proporti on of suff erers wi th oral ul cerati on.
Coel i ac di sease i s a permanent i ntol erance to gl i adi n.
It was not unti l the mi ddl e of the twenti eth century that the of fendi ng di etary product i n
coel i ac di sease was found to be wheat fl our. Damage to the i ntesti nal mucosa i s caused
predomi nantl y by the gl i adi n fracti on of gl uten. Gl uten i s present i n wheat, rye, and barl ey
(and possi bl y oats).
In adul t coel i ac di sease, di arrohea, wei ght l oss, and weakness are the cl assi c si gns and
symptoms.
Si nce the 1980s i t has become apparent that the cl i ni cal pattern of coel i ac di sease i s
changi ng and that i t i s frequentl y underdi agnosed. It i s becomi ng i ncreasi ngl y appreci ated
that i t may become apparent or be di agnosed i n chi ldren or adul ts l ong after the i ni ti al
i ntroducti on of gl uten i nto the di et. In the l ate 1990s thi s l ed to the concept of the
coel i ac i ceberg (Fi g. 12. 1). At the ti p are those wi th cl i ni cal l y overt di sease fol l owed
by, i n order, si l ent coel i ac di sease, l atent coel i ac di sease, and heal thy i ndi vi dual s who may
have the suscepti bi l i ty genes for the di sease.
The i nci dence of bi opsy-proven coel i ac di sase i n the UK i s 1 i n 2000, whereas the i nci dence
when screeni ng for the di sease usi ng such markers as anti -endomysi al anti bodi es actual l y
approaches 1:300. Thi s l atter group of coel i ac patients may be the popul ati on that gets
aphthous ul cers, despi te not havi ng any of the more general i zed gastroi ntesti nal symptoms
(see Chapter 5). Approxi matel y 510 per cent of pati ents wi th coel i ac di sease have an
aff ected fi rst-degree rel ati ve. There i s al so a recogni zed

associ ati on of coel i ac di sease wi th other autoi mmune di seases such as i nsul i n-dependent
di abetes mel l i tus. Approxi matel y 510 per cent of peopl e wi th thi s sort of di abetes wi l l al so
have coel i ac di sease.
Defi ni ti ve di agnosi s of coel i ac di sease requi res the demonstrati on of characteri sti c mucosal
P. 144
Fig. 12.1 The coel i ac i ceberg and spectrum of gl uten sensi tivi ty.
abnormal i ti es i n bi opsi es of the smal l bowel (usuall y obtai ned vi a endoscopy). The earl y
l esi on compri ses i ncreased l ymphocyti c i nfi l trati on i nto the epi thel i um and then the l ami na
propri a. Crypt hyperpl asi a occurs next and i s fol l owed by the appearance of vi l l ous atrophy.
The actual appearance of total or subtotal vi l l ous atrophy i s not vi l l ous atrophy at al l but i s,
i n fact, due to crypt hyperpl asi a fi l l i ng i n the spaces between the vi l l i (see Fi g. 12. 2). IgA
anti -gl i adi n, IgA anti -reti cul i n, and IgA endomysi al autoanti bodi es are bl ood tests that can be
used as a fi rst-l i ne i nvesti gati on i n those peopl e suspected of havi ng coel i ac di sease or as a
screeni ng tool . However, by defi ni ti on, the f i nal di agnosi s rests on the mi croscopi c
appearance of the smal l bowel bi opsy. Those pati ents i n whom the bl ood tests are posi ti ve
but the bi opsy i s essenti al l y normal are defi ned as havi ng l atent coel i ac di sease. It i s
uncertai n what proporti on of these wi l l progress to meet the ful l di agnosti c cri teri a of coel i ac
di sease.
Smal l bowel bi opsy sampl i ng i s essenti al for the diagnosi s for coel i ac di sease. Di agnosi s
shoul d not be based on symptoms or serol ogi cal tests al one.
Pati ents wi th coel i ac di sease wi l l undergo cl i ni cal remi ssi on on a gl uten-free di et but they
may i ni ti al l y need i ron and fol ate suppl ements to correct mi crocyti c/macrocyti c anaemi as
caused by mal absorpti on. Dermati ti s herpeti f ormi s, a ski n di sorder characteri zed by
bl i steri ng, (see Chapter 11), i s the cl assi cal non-gastroi ntesti nal mani festati on of coel i ac
di sease, and both the i ntesti nal l esi on and rash respond to a gl uten-free di et. Dermati ti s
herpeti f ormi s i s more preval ent i n the adul t popul ati on wi th coel i ac di sease and i s hardl y
ever seen i n chi l dren.
Dermati ti s herpeti formi s i s the cl assi cal non-gastroi ntesti nal mani f estati on of coel i ac
di sease.
Oral manifestations of coeliac disease
The associ ati on of recurrent aphthous stomati ti s (RAS) wi th coel i ac di sease i s wel l
establ i shed but i ni ti al studi es of thi s rel ati onshi p i mpl i ed that the proporti on of RAS-aff ected
i ndi vi dual s wi th coel i ac di sease was of the order of 25 per cent. Recent studi es i ndi cate that
Fig. 12.2 Hi stol ogi cal appearance of vi l l ous atropl y (jejunum) i n a pati ent wi th coel i ac
di sease.
thi s fi gure i s much l ower and l i kel y to be l ess than 5 per cent.
Adul ts wi th undi agnosed coel i ac di sease may present wi th recurrent aphthous stomati ti s.
Mal absorpti on, as a resul t of coel i ac di sease, may resul t i n a haemati ni c def i ci ency,
parti cul arl y of i ron and fol i c aci d. Pati ents wi th coel i ac di sease may therefore present wi th
angul ar chei l i ti s, gl ossi ti s, or RAS. It i s, therefore, essenti al to undertake a f ul l bl ood count
and haemati ni c assay for pati ents presenti ng wi th these oral condi ti ons. Serol ogi cal testi ng
for anti bodi es i s i ndi cated i f there are gut symptoms suggesti ve of coel i ac di sease or
evi dence of mal absorpti on. Whether or not serol ogi cal testi ng shoul d be recommended for
pati ents wi th RAS, i n the absence of these features, i s a matter for debate but i n some cases
aphthous ul cers may be the onl y mani f estati on of the di sease. The sensi ti vi ty and speci fi ci ty
of these tests are not, however, 100 per cent and smal l bowel bi opsy i s requi red for a
di agnosi s of coel i ac di sease. Interesti ngl y, denti sts may be al erted to the possi bi l i ty of
coel i ac di sease by f i ndi ng dental enamel def ects (that i s, hypopl asi a) on the permanent
teeth. The presence of dental enamel hypopl asi a i s thought to i ndi cate that the coel i ac
di sease has been present at l east i n the f i rst 2 years of l i f e even though i t mi ght have been
cl i ni cal l y si l ent.
Denti sts may suspect coel i ac di sease because of enamel def ects on permanent
teethparti cul arl y l ower i nci sors.
Inflammatory bowel disease (IBD)
The i nfl ammatory bowel di seases are l i f el ong condi ti ons resul ti ng f rom aberrant i nf l ammati on
of the mucosal l i ni ng of the

gastroi ntesti nal tract. The two mai n categori es are Crohn' s di sease, whi ch may aff ect the gut
anywhere f rom mouth to anus, and ul cerati ve col i ti s, whi ch i s predomi nantl y wi thi n the
col on.
Oral manifestations of coeliac disease
Oral ul cerati onRAS
Gl ossi ti s
Angul ar chei l i ti s
Enamel hypopl asi a
Oral i nvol vement has for some ti me been recogni zed i n pati ents sufferi ng from both Crohn' s
di sease (CD) and, to a l esser extent, ul cerati ve col i ti s (UC). Oral l esi ons may precede or
accompany gastroi ntesti nal di sease and can be the onl y si te of i nvol vement.
Crohn' s di sease and ul cerati ve col i ti s are the two major f orms of non-speci f i c i nfl ammatory
bowel di sease (IBD).
Crohn's disease
Crohn' s di sease was f i rst descri bed i n 1932 as regi onal i l ei ti s, that i s, i nfl ammati on of the
i l eum. The ori gi nal paper i ncl uded a descri pti on of mucosal i nfl ammati on and occasi onal
ul cerati on of the affected gut, l ymph node hyperpl asi a l eadi ng to obstructi ve oedema, and
the producti on of granul omatous l esi ons. Shortl y after the f i rst descri pti on of the di sease, i t
became cl ear that i t was not of necessi ty conf i ned to the i l eum, and l esi ons have si nce been
descri bed throughout the whol e of the gastroi ntestinal tract. The cl i ni cal progress of the
P. 145
di sease i s very vari abl e wi th i nacti ve phases. However, i n the more aggressi ve stages of the
di sease, there may be abdomi nal pai n, di arrhoea, and mal ai se and i nf l ammati on of the
aff ected gut may progress to the producti on of fi stul ae. There may be arthropathi es and ski n
granul omas associ ated wi th the di sease, as wel l as probl ems ari si ng f rom mal absorpti on. The
aeti ol ogy of the di sease i s not known and the eponymous descri pti on has, therefore, been
mai ntai ned. Treatment i s medi cal , usi ng steroi ds, azathi opri ne (and other
i mmunosuppressi ve agents), ami nosal i cyl ates, di etary treatment strategi es, and repl acement
therapy to correct mal absorpti on. Di etary i nterventi on al one has been demonstrated to be
eff ecti ve and i s especi al l y f avoured i n chi l dren as i t may avoi d the use of steroi ds. In some
cases where the di sease i s resi stant to medi cal therapy, surgery i s necessary.
Oral Crohn's disease
Oral l esi ons, i n pati ents suf feri ng from CD, whi ch hi stol ogi cal l y resembl ed those el sewhere i n
the gut were fi rst descri bed i n 1969. The fi rst cases i n whi ch oral l esi ons of CD were
descri bed i n the absence of gastroi ntesti nal l esi ons were reported i n 1973. The orofaci al
features of Crohn' s di sease are descri bed bel ow and summari zed i n Tabl e 12. 1.
Orofacial granulomatosis
Over the l ast decade, there has been i ncreasi ng attenti on pai d to non-i nfecti ous
granul omatous di sorders of the orof aci al regi on, whi ch i ncl ude: oral Crohn' s di sease and oral
sarcoi d, as wel l as cl i ni cal enti ti es, known as the Mel kerssonRosenthal syndrome
and Mi eschener' s chei l i ti s granul omatosa (granul omatous chei l i ti s).
The term orof aci al granul omatosi s (OFG) was i ntroduced to encompass these
di sorders and to descri be a cl i ni cal syndrome presenti ng wi th swel l i ng of the face, l i ps, or
oral ti ssues i n associ ati on wi th hi stol ogi cal evi dence of noncaseati ng granul omatous
i nfl ammati on wi thi n these ti ssues.
Clinical manifestations of orofacial granulomatosisoral
Crohn's disease (Table 12.1)
Orofaci al swel l i ng, parti cul arl y i nvol vi ng the l i ps, i s the most consi stent feature of OFG and
the most common reason for pati ents presenti ng f or i nvesti gati on and treatment. The
Table 12.1 Clinical features of orofacial
granulomatosisoral Crohn's disease
Swel l i ng of l i ps and face*
Mucosal tags or cobbl estoni ng
Oral ul cerati on (RAS and non-RAS)
Angul ar chei l i ti s
Li p fi ssures
Persi stent l ymphadenopathy
Peri oral erythema and scal i ng of ski n
Ful l -wi dth gi ngi vi ti s
*Fi ssured tongue (l i ngua pl i cata) and f aci al pal sy are other mani festati ons of the
Mel kersonRosenthal syndrome.
RAS, Recurrent aphthous stomati ti s.
swel l i ng of the l i ps i s pai nl ess, has a f i rm rubbery consi stency; and can i nvol ve both
upper and l ower l i ps, i ndi vi dual l y or together. It can be uni l ateral or bi l ateral . The l i ps can
be mi l dl y swol l en or grossl y enl arged l eadi ng to severe cosmeti c di sfi gurement (Fi g. 12. 3).
The f l oor of the mouth i s occasi onal l y i nvol ved. Faci al swel l i ng can af fect one or both si des of
the face and may i nvol ve the peri orbi tal ti ssues and chi n.
Li p fi ssures and angul ar chei l i ti s are commonl y seen i n OFG.
Pati ents of ten gi ve a hi story of recurrent or persistent swel l i ng of the submandi bul ar of
cervi cal nodes. Persi stent verti cal fi ssuri ng, chappi ng of the l i ps, and angul ar chei l i ti s are
al so seen i n OFG. There may al so be persi stent erythema and scal i ng of the peri oral ti ssues.
The buccal mucosa i s thi ckened and fol ded, gi vi ng a corrugated or characteri sti c
cobbl estone appearance (Fi g. 12. 4). Mucosal tags may be present, parti cul arl y i n the
retromol ar regi ons.
Pati ents may present wi th recurrent aphthous stomati ti s (RAS), but thi s condi ti on i s common
i n the popul ati on general l y

and not speci fi c to OFG (see Chapter 5). Persi stent l i near ul cers (non-RAS) tend to occur at
the base of hyperpl asti c ti ssue f ol ds, parti cul arl y i n the buccal and l abi al sul ci , and can be
pai nful , parti cul arl y when eati ng. Thi ckened buccal mucosa can al so become traumati zed
al ong the occl usal ri dge, resul ti ng i n ul cerati on. There i s frequent gi ngi val i nvol vement i n
OFG, whi ch i s qui te di sti nct f rom that seen i n a non-speci f i c i nf l ammatory gi ngi vi ti s. The
gi ngi vae may appear hyperpl asti c and oedematous and the abnormal ti ssue usual l y extends
from the gi ngi val margi ns on to the non-kerati ni zed mucosa of the sul ci a ful l
thi ckness pattern (Fi g. 12. 5).
P. 146
Fig. 12.3 Gross swel l i ng of l ower l i p i n pati ent wi th establ i shed Crohn' s di sease
aff ecti ng l ower gut.
Hi stol ogi cal l y, noncaseati ng and epi thel i oi d granul omas, wi th or wi thout mul ti nucl eated gi ant
cel l s, are seen i n about 90 per cent of cases. Granul omas are not al ways present and thei r
absence does not excl ude the cl i ni cal di agnosi s of OFG.
OFG as a predictor of Crohn's disease
The i nterrel ati onshi p between oral Crohn' s di sease and OFG i s a matter for debate. In one
study, 10 per cent of 60 OFG pati ents had def i ni te evi dence of Crohn' s di sease af fecti ng the
i ntesti ne but al l si x pati ents had reported gastrointesti nal symptoms. Another study
demonstrated asymptomati c i ntesti nal i nvol vement i n 37 per cent who presented sol el y wi th
features of OFG. The preval ence of asymptomati c i ntesti nal di sease i n pati ents wi th OFG has
been reported as between 10 and 50 per cent i n various seri es. In i ndi vi dual cases of OFG i t
i s di ffi cul t to predi ct whether there wi l l be subsequent devel opment of CD el sewhere i n the
gut.
Fig. 12.4 Corrugated (cobbl estoned) appearance of the buccal mucosa i n orofaci al
granul omatosi s (OFG).
OFG and oral sarcoid
Pati ents suff eri ng f rom sarcoi dosi s can present wi th OFG mani f esti ng as regi onal
l ymphadenopathy, orofaci al swel l i ng, gi ngi val enl argement, or ti ssue tags. Oral l esi ons can
al so occur as mul ti pl e or sol i tary, pai nl ess mucosal l esi ons that appear as red nodul es
aff ecti ng the gi ngi va, l i ps, pal ate, and buccal mucosa. Invol vement of the major sal i vary
gl ands may occasi onal l y present as a pai nl ess persistent enl argement of the gl and resul ti ng
i n a reducti on i n sal i vary fl ow. Sarcoi d can therefore present as a Sjgren' s-type
condi ti on. Invol vement of the tongue wi th sarcoi d i s rare. Paroti d gl and i nvol vement,
together wi th uvei ti s and l ow-grade fever, i s characteri sti c of Heerfordt' s syndrome.
Characteri sti c ski n, ophthal mi c, and upper respi ratory tract l esi ons of sarcoi d may be present
and conf i rm the di agnosi s.
OFG: MelkerssonRosenthal syndrome (MRS) and cheilitis
granulomatosa
The term OFG encompasses the Mel kerssonRosenthal syndrome (MRS), both i n i ts
cl assi cal presentati on and the ol i gosymptomati c and monosymptomati c vari ants. The cl assi cal
presentati on of MRS i s as recurrent orofaci al oedema, rel apsi ng faci al paral ysi s, and fi ssured
tongue. These symptoms may occur at the same ti me or at i nterval s of months or years. Thi s
rare syndrome affects both sexes equal l y and most commonl y occurs i n the second decade of
l i f e. Most revi ews of MRS cases i ndi cate that pati ents sel dom report al l the components of
the syndrome. The i ncompl ete expressi on of MRS has been recogni zed as havi ng
monosymptomati c and ol i gosymptomati c forms. Orof aci al swel l i ng, parti cul arl y of the l i ps, i s
the most i mportant and consi stent symptom of MRS and i s descri bed as a pai nl ess, non-
pruri ti c, fi rm oedema.

The cl assi c presentati on of MRS i s of recurrent orof aci al oedema (often af fecti ng the l i ps),
rel apsi ng f aci al pal sy, and fi ssured tongue.
The recurrent peri pheral f aci al paral ysi s seen i n MRS i s characteri sti cal l y sudden i n onset and
i ndi sti ngui shabl e f rom Bel l ' s pal sy. The pal sy i s usual l y uni l ateral and i n the majori ty of
pati ents cl ears spontaneousl y. The fi ssured tongue (l i ngua pl i cata) appears to be the l east
common and l east i mportant f eature of MRS. A number of associ ated di sorders have been
reported i n MRS and i ncl ude other neurol ogi cal manif estati ons and ophthal mi c fi ndi ngs, such
as kerati ti s, retrobul bar neuri ti s, di pl opi a, and paral ysi s of the medi al rectus muscl e.
Mi escher i n 1945 descri bed a characteri sti c di ffuse swel l i ng of one or both l i ps, whi ch may be
fol l owed by permanent enl argement. Mi eshcer' s granul omatous chei l i ti s i s characteri zed
hi stol ogi cal l y by aggregates of noncaseati ng granul omas and i s consi dered by some to be an
ol i gosymptomati c (or monosymptomati c) f orm of the Mel kerssonRosenthal syndrome.
Chei l i ti s granul omatosa i s the most frequent si gn of OFG.
The term OFG therefore encompasses MRS and i ts ol i gosymptomati c or monosymptomati c
vari ants, such as chei l i ti s granul omatosa, al l of whi ch are characteri zed hi stol ogi cal l y by the
presence of noncaseati ng granul omata i n orofaci al bi opsy speci mens.
OFG: aetiology and other associations
The aeti ol ogy of OFG unrel ated to systemi c di sease remai ns uncl ear. However, al l ergy,
i nfecti ons, and heredi tary causes have al l been i mpl i cated, together wi th i nfecti ous agents
Fig. 12.5 Gi ngi val appearance i n a chi l d wi th OFG.
P. 147
such as Saccharomyces cerevi si ae, Mycobacteri um paratubercul osi s, and Campyl obacter-
l i ke bacteri a.
Cl i ni cal atopy i s more preval ent i n OFG. Recent studi es have i nvesti gated the associ ati on of
OFG wi th i ntol erance to speci fi c f oods, food addi ti ves, fl avouri ngs, and the
consti tuents of toothpastes. Ci nnamonal dehyde and sodi um benzoates have been parti cul arl y
i mpl i cated i n thi s respect and, i n some seri es, there was a cl i ni cal response to speci fi c
el i mi nati on di ets. It remai ns unknown whether sensi ti vi ty to food addi ti ves i s the pri mary
factor f or some pati ents wi th OFG or a secondary aggravati ng f actor to some underl yi ng
process.
Management of OFG
Pati ents wi th OFG must be appropri atel y i nvesti gated, not onl y to confi rm the di agnosi s but
to i denti f y any provoki ng factors and si gns and symptoms, suggesti ve of an underl yi ng
systemi c condi ti on, such as CD or sarcoi dosi s. A ful l range of haematol ogi cal and bi ochemi cal
i nvesti gati ons, i ncl udi ng i nfl ammatory markers, shoul d be undertaken, together wi th an
esti mati on of the serum angi otensi n-converti ng enzyme (ACE) and a chest radi ograph. Bi opsy
of an affected si te (usual l y the l abi al or buccal mucosa and occasi onal l y the gi ngi vae) shoul d
be carri ed out by an experi enced operator. Granul omata may onl y be present i n the
underl yi ng muscl e and i t i s therefore advi sabl e to extend the bi opsy deeper beyond the
superfi ci al ti ssue.
Some authori ti es regard compl ete gastroi ntesti nal eval uati on by
oesophagogastroduodenoscopy, i l eocol onoscopy, and smal l bowel radi ographs as mandatory
i n al l pati ents wi th OFG. Others argue that these potenti al l y unpl easant i nvesti gati ons are
unwarranted for al l cases of OFG, unl ess the hi story, cl i ni cal exami nati on, or prel i mi nary
i nvesti gati ons are suggesti ve of l ower gastroi ntesti nal i nvol vement. The authors opi ni on
i s that al l pati ents wi th OFG shoul d be seen by a gastroenterol ogi st, who can deci de on the
appropri ateness of future gastroi ntesti nal i nvesti gati ons.
Whether or not al l pati ents wi th OFG shoul d be patch-tested to i denti f y possi bl e al l ergi es to
foods or f ood addi ti ves i s debatabl e. To date, there i s no total l y convi nci ng evi dence of a
cl i ni cal response to el i mi nati on di ets, but there may be a therapeuti c rol e for di etary
mani pul ati on i n some pati ents.
Pati ents wi th OFG often seek treatment for l i p swel l i ng, whi ch causes di stress and
embarrassment.
The outl ook for pati ents wi th OFG i s vari abl e and treatment i s often unrewardi ng. Pati ents,
and thei r parents, usual l y seek treatment because of unsi ghtl y l i p-swel l i ng, whi ch can cause
consi derabl e embarrassment and di stress to chi l dren and teenagers. Occasi onal , short
courses of systemi c steroi ds can be eff ecti ve at reduci ng the swel l i ng and the dose can be
gradual l y reduced over a number of weeks, dependi ng on the severi ty of the epi sode. Long-
term systemi c steroi ds are, however, contrai ndi cated, parti cul arl y i n chi l dren. Intral esi onal
steroi ds can be i njected i nto the l i ps or face but l ocal anaestheti c bl ocks shoul d be gi ven
bef orehand, to make the procedure more acceptabl e to pati ents. These i njecti ons may need
to be repeated. A l arge number of systemi c drugs have been tri ed for OFG wi th mi xed
success and i ncl ude azathi opri ne, cl ofazi mi ne, hydroxychl oroqui ne, danazol , ci cl ospori n,
sul azosul fapyri di ne, thal i domi de, and anti mi crobi als such as metroni dazol e and co-
tri moxazol e.
Treatment of OFG i s unsati sfactory and response to drug-therapy i s di sappoi nti ng.
Topi cal steroi ds and anti septi c and anal gesi c mouthwashes can be hel pful for managi ng the
oral ul cerati on, associ ated wi th OFG. Angul ar chei li ti s and l i p fi ssures frequentl y become
secondari l y i nfected but i n many cases can be i mproved by the appl i cati on of

an anti fungal or anti bi oti c cream (for exampl e, nystati n, fusi di c aci d, mi conazol e), as
i ndi cated by mi crobi ol ogy. Topi cal steroi d creams may al so be requi red but hi gh-potency
preparati ons are best avoi ded, parti cul arl y i n the l ong term. Dry, chapped l i ps wi l l usual l y
benefi t from regul ar appl i cati on of an emol l i ent. Surgi cal reducti on of the l i p swel l i ng i n OFG
has not been rel i abl y ef fecti ve and i s not usual l y recommended.
The management of OFG necessi tates a mul ti di sci pl i nary approach.
Chi l dren wi th OFG and thei r parents requi re a great deal of ti me and support from a cl i ni ci an
who i s ful l y cogni zant of the condi ti on. OFG i s a compl ex condi ti on and i ts management
necessi tates a mul ti di sci pl i nary approach. Persi stent swel l i ng of the l i ps and f ace and pai nful
oral ul cerati on can cause consi derabl e embarrassment and di stress to chi l dren and adul t
pati ents; both requi re sympatheti c management.
Ulcerative colitis
Ul cerati ve col i ti s i s a di sease, usual l y begi nni ng i n young adul t l i fe, i n whi ch i nfl ammatory
changes i n the col oni c mucosa and submucosa l ead to wi despread ul cerati on. Thi s ul cerati on
may be compl i cated by haemorrhage, perf orati on, and, occasi onal l y, by the eventual onset of
mal i gnancy. Pai n, di arrhoea, and general i zed abdominal di scomfort are the predomi nant
symptoms. The aeti ol ogy of ul cerati ve col i ti s i s not cl ear, al though i t seems to be one of the
di seases of ci vi l i zati on. The treatment of ul cerati ve col i ti s i s by the use of
sul f asal azi ne and steroi ds, used ei ther l ocal l y (as pessari es or enemas) or systemi cal l y.
Surgery may be necessary i f medi cal treatment fai l s.
Crohn' s di sease can aff ect any part of the gastroi ntesti nal tract. Ul cerati ve col i ti s onl y
aff ects the l arge bowel .
Stomatitis and inflammatory bowel disease
Pyostomatitis vegetans
Pyostomati s vegetans i s a rare oral di sorder that is consi stentl y associ ated wi th chroni c
i nfl ammatory bowel di sease and consi dered to be a hi ghl y speci fi c marker f or IBD. The bowel
P. 148
Table 12.2 Summary of OFG
OFG i s a cl i ni copathol ogi cal enti ty descri bi ng orofaci al l esi ons wi th noncaseati ng
granul omas. As a uni fyi ng concept i t i ncl udes:
Pati ents wi th establ i shed CD and those who subsequentl y devel op CD
Pati ents wi th oral sarcoi d
Pati ents di agnosed as havi ng MRS or chei l i ti s granul omatosa
A smal l , heterogeneous group of pati ents wi th orofaci al mani f estati ons of
del ayed-type hypersensi ti vi ty reacti ons to food or food addi ti ves
symptoms often precede oral i nvol vement by several months or years. Pyostomati s vegetans
has a gender predi l ecti on wi th a mal e:femal e rati o of 3:1 and can af fect al l age groups. It i s
the oral equi val ent of pyoderma vegetans. Oral and cutaneous l esi ons are si mi l ar and may be
seen concurrentl y or i n i sol ati on.
Pyostomati ti s vegetans i s an i mportant oral marker f or i nf l ammatory bowel di sease.
Pyostomati ti s vegetans has a di sti nct cl i ni cal appearance wi th mi l i ary abscesses and pustul ar
l esi ons af fecti ng the oral mucosa and gi ngi va, whi ch become thi ckened, erthematous, and
may exhi bi t vegetati ons or cobbl estoni ng. Pustul ar l esi ons often rupture, whi ch l eads
to erosi ons and ul cerati on, wi th fi ssuri ng, i n a pattern descri bed as snai l -track
ul cerati on. The oral l esi ons predomi nantl y af fect the l abi al and buccal mucosa and the l abi al -
attached gi ngi vae (Fi g. 12. 6), al though the hard and soft pal ate, vesti bul e, and tonsi l l ar
regi on can al so be af fected. The hi stol ogi cal features of pyostomati s vegetans are often
characteri sti c, al though not pathognomoni c, showi ng i ntraepi thel i al and subepi thel i al
mi croabscesses contai ni ng l arge numbers of eosi nophi l s.
Topi cal steroi d therapy has been successful for the treatment of pyostomati ti s vegetans but
i n many cases systemi c treatment, wi th or wi thout azathi opi ne or sul famethoxypyri dazi ne, i s
requi red. Management of the associ ated IBD may al so resul t i n i mprovement of the oral
l esi ons. In vi ew of the cl ose associ ati on of pyostomati ti s vegetans wi th IBD, i t has been
suggested that al l pati ents shoul d be i nvesti gated for bowel di sease, even i n the absence of
gastroi ntesti nal symptoms.
Pyostomatitis gangrenosum
Other forms of stomati ti s have been reportedl y associ ated wi th IBD and i ncl ude i rregul ar,
deep, foul -smel l i ng ul cers of varyi ng si ze, wi th rol l ed margi ns and a greyi sh, fi bri nous base.
Gastro-oesophageal reflux disorder (GORD)
Heal thy i ndi vi dual s experi ence gastro-oesophageal ref l ux af ter a meal and thi s i s due to the
rel axati on of the l ower oesophageal sphi ncter. In pati ents wi th gastro-oesophageal ref l ux
di sorder (GORD), there i s i ncreased frequency and durati on of refl ux and damage i s caused
Fig. 12.6 Pyostomati ti s vegetans af fecti ng l abi al mucosa and gi ngi vae i n a pati ent wi th
ul cerati ve col i ti s.
to the oesophageal mucosa by regurgi tati on of gastri c contents. As a resul t there can be
refl ux oesophagi ti s, ul cerati on, stri cture, or epi thel i al metapl asi a (Barrett' s oesophagus).
Symptoms of GORD i ncl ude heart-burn, epi gastri c pain, and regurgi tati on. However, some
pati ents, the si l ent ref l uxers, have no symptoms. Drug therapy i s usual l y successful
for GORD and consi sts of si mpl e antaci ds (or coveri ng agents), H
2
receptor bl ockers (f or
exampl e, ci metedi ne) that

i nhi bi t gastri c aci d secreti on, or proton-pump i nhi bi tors (for exampl e, omeprazol e) that
i nhi bi t aci d producti on. Surgery i s rarel y i ndi cated but pati ents who devel op oesophageal
stri ctures may need peri odi c di l atati on. Pati ents wi th GORD may devel op dental erosi on (see
Chapter 18), parti cul arl y of the pal atal aspects of the teeth, and, i n pati ents who are
asymptomati c, thi s erosi on may be the onl y i ndi cator of pathol ogi cal GORD. Pati ents who
regul arl y chew antaci d preparati ons may show si gns of toothwear and some al gi nate-
contai ni ng antaci ds have a hi gh sugar content that can predi spose to cari es. Occasi onal l y,
pati ents present wi th orof aci al mani f estati ons of iron defi ci ency (Chapter 13), i f there has
been bl eedi ng from oesophageal ul cerati on.
Pati ents presenti ng wi th pal atal dental erosi on shoul d be assessed f or gastro-oesophageal
refl ux (GORD).
Discussion of problem case
Thi s l ady' s orofaci al si gns are suggesti ve of OFG, whi ch can be a mani f estati on of Crohn' s
di sease. The gastroi ntesti nal symptoms, previ ousl y di agnosed as IBS, may al so be i ndi cati ve
of i nf l ammatory bowel di sease (IBD), parti cul arl y Crohn' s di sease.
IBS i s a functi onal bowel di sease, i n whi ch pati ents report vari abl e degrees of abdomi nal
pai n, di stensi on, and bl oati ng. The pai n i s cl assi cal l y i n the l ef t i l i ac fossa and usual l y
rel i eved by defecati on or passage of wi nd. Pati ents often compl ai n of consti pati on or
di arrhoea and many report frequent bowel acti ons i n the morni ng. Women are affected more
than men. Psychol ogi cal factors are i mportant and stress often exacerbates symptoms.
The pri ori ty, i n thi s parti cul ar case, i s for thi s l ady to be referred as soon as possi bl e for ful l
i nvesti gati ons by a gastroenterol ogi st, to excl ude IBD, parti cul arl y Crohn' s di sease. It i s
i mportant that you l i ai se wi th her GP and di scuss your suspi ci ons about thi s l ady' s condi ti on.
Referral to a gastroenterol ogi st i s preferabl y made by her GP. The si tuati on shoul d be
expl ai ned to the pati ent, who shoul d be gi ven reassurance and appropri ate topi cal therapy
for her angul ar chei l i ti s and oral ul cerati on. Referral to an oral physi ci an shoul d be made for
confi rmati on of your di agnosi s and provi si on of defi ni ti ve treatment. Al ternati vel y, the
pati ent can be ref erred di rectl y to an oral medi ci ne uni t and they wi l l arrange f or appropri ate
i nvesti gati ons, i n conjuncti on wi th a department of gastroenterol ogy. It i s i mportant,
however, that the pati ent' s GP i s kept apprai sed of the si tuati on, as thi s l ady' s general
condi ti on may deteri orate, whi l st she i s awai ti ng appoi ntments.
Project
1. Intesti nal pol yps occur i n a number of syndromes that have orof aci al mani f estati ons.
P. 149
Q1 Coul d thi s l ady' s oral condi ti on be rel ated to her gut symptoms?
Q2 What are the symptoms of IBS?
Di scuss the f eatures of these and comment on thei r propensi ty for mal i gnant change.
> Tabl e of Cont ent s > 13 - Bl ood and nut r i ti on, endocri ne di sturbances, and renal di sease
13
Blood and nutrition, endocrine disturbances, and
renal disease
Problem cases
Case 13.1
A 40-year-ol d l ady reports to her denti st wi th a 2-month hi story of spontaneous bl eedi ng
from her gums, whi ch al so bl eed excessi vel y when toothbrushi ng. Her mouth has become
very sore over the previ ous 23 weeks. She compl ai ns of feel i ng ti red and l ooks very pal e.
On cl oser questi oni ng the pati ent reports frequent attacks of si nusi ti s and chest i nfecti on. In
addi ti on, she has noti ced that she tends to brui se easi l y. She i s not taki ng any medi cati on.
Case 13.2
A 45-year-ol d mal e pati ent wi th a hi story of chronic renal fai l ure presents wi th severe
toothache. He i s not a regul ar dental attender. This gentl eman undergoes regul ar
haemodi al ysi s and has an i ndwel l i ng arteri ovenous shunt. He l ooks very ti red and has come
di rectl y from the renal di al ysi s uni t, where he had been gi ven some anal gesi cs to take f or hi s
dental pai n. On exami nati on there i s a grossl y cari ous maxi l l ary fi rst mol ar that requi res
extracti on. There i s no associ ated soft-ti ssue swell i ng but you noti ce whi te pl aques on the
tongue and buccal mucosae. These do not wi pe off and the pati ent reports that they
come and go.
Disorders of the blood
It i s wel l known that l esi ons of the oral mucosa may occur i n pati ents wi th abnormal i ti es of
the bl ood. In parti cul ar, the appearance of gl ossi ti s or angul ar chei l i ti s i n anaemi c pati ents
has often been descri bed. However, i t has more recentl y become recogni zed that such oral
symptoms may be the resul t of rel ati vel y mi nor changes i n the condi ti on of the bl ood and
that they may occur earl y i n the di sease process, even before abnormal i ti es can be
demonstrated by a si mpl e bl ood exami nati on. Thus, an earl y di agnosi s of the bl ood di sorder
may depend on a recogni ti on of the si gni f i cance of the oral symptoms. The great majori ty of
these pati ents are suff eri ng f rom anaemi as of vari ous ki nds and, hence, the major i nterest i s
centred on thi s group of condi ti ons, but i t must be borne i n mi nd that abnormal i ti es of the
whi te cel l and pl atel et components of the bl ood may al so be refl ected i n oral changes.
Q1 What di agnosi s must be excl uded i n thi s l ady' s case? What other oral mani festati ons
of thi s condi ti on mi ght you fi nd on exami nati on?
Q1 How woul d you manage the dental extracti on of this medi cal l y compromi sed pati ent?
Q2 What i s the most l i kel y di agnosi s of the whi te plaques on the pati ent' s oral mucosa?
Anaemias
The characteri sti c feature of anaemi a i s a reducti on i n the l evel of haemogl obi n, whi ch i s
usual l y accompani ed by a decreased number of erythrocytes. The red cel l s (erythrocytes) are
the ci rcul ati ng cel l s predomi nantl y concerned wi th the transport of oxygen to the ti ssues by
means of the i ron-contai ni ng substance haemogl obi n wi thi n them. They are normal l y regul ar,
bi concave di scs but, i f di sturbances of f ormati on occur, they may become qui te i rregul ar i n
si ze and shape. Such i rregul ari ty i s often a si gn of i mpai red functi on. The formati on of the
erythrocytes wi thi n the bone marrow i s sti mul ated by a number of nutri ti onal f actors, the two
of greatest si gni fi cance bei ng vi tami n B
12
and fol i c aci d. Both these substances are absorbed
from the gut and must be present i n bal anced quantiti es for normal red cel l producti on to
take pl ace, even when suf fi ci ent i ron i s avai l abl e for the synthesi s of haemogl obi n. Iron, fol i c
aci d, and vi tami n B
12
are known as haemati ni cs and are essenti al for normal erythropoi esi s.
Absorpti on depends on normal mucosa i n the smal l i ntesti ne and, i n parti cul ar, on the
presence of i ntri nsi c f actor, whi ch i s synthesi zed i n the gastri c mucosa and whi ch must be
present before absorpti on of vi tami n B
12
can take pl ace. If there are abnormal i ti es that l ead
to fai l ure of i ntri nsi c f actor synthesi s, vi tami n B
12
cannot be absorbed from the gut and must
be repl aced parenteral l y. Lack of ei ther vi tami n B
12
, fol i c aci d, or i ntri nsi c factor wi l l
theref ore aff ect red cel l producti on i n the bone marrow. The erythrocytes formed under these
condi ti ons are l arger than normal (macrocyti c) and thei r f uncti on i s severel y di sturbed. The
resul tant cl i ni cal condi ti ons are known as megal oblasti c anaemi as.

Megaloblastic anaemias
Vi tami n B
12
or fol i c aci d def i ci ency are the most common causes of anaemi as wi th
macrocytosi s. Perni ci ous anaemi a i s an autoi mmune condi ti on causi ng atrophy of the gastri c
mucosa (atrophi c gastri ti s) and consequent f ai l ure to secrete i ntri nsi c factor due to an anti -
i ntri nsi c factor anti body. Theref ore, i n pati ents wi th thi s condi ti on anti bodi es to i ntri nsi c
factor may be detected i n the bl ood. More compl ex mal absorpti on syndromes may al so occur,
i nvol vi ng fai l ure to absorb not onl y vi tami n B
12
, but al so fol i c aci d and i ron compounds. The
term megal obl asti c anaemi a refers to the change i n si ze and structure of the basi c marrow
cel l from whi ch the erythrocytes are deri vedthe l arge ci rcul ati ng erythrocytes formed from
these abnormal stem cel l s are macrocytes. Si mi l ar large ci rcul ati ng red cel l s may be f ound i n
other anaemi as that are not dependent on abnormal i ti es of the vi tami n B
12
/fol i c aci d
metabol i sm (for i nstance, i n some i ron defi ci ency anaemi as) and such macrocyti c anaemi as
form a separate group that, evi dentl y, wi l l not respond to treatment wi th vi tami n B
12
or fol i c
aci d. Other causes of vi tami n B
12
and fol ate defi ci ency i ncl ude mal absorpti on and dietary
def i ci ency. Fol ate defi ci ency can be due to drugs (for exampl e, phenytoi n) or the resul t of
the i ncreased physi ol ogi cal demand duri ng pregnancy.
The si tuati on i s compl i cated by the fact that, i n mul ti pl e defi ci enci es, the tendency to
mi crocytosi s as a resul t of i ron def i ci ency may be counteracted by a tendency to
macrocytosi s caused, say, by fol ate defi ci ency. The resul t may be a normal mean corpuscul ar
vol ume (MCV) i n a pati ent wi th both defi ci enci es present. In these ci rcumstances a routi ne
bl ood count wi l l be returned as normal . It i s al so cl earl y establ i shed that pati ents wi th fol ate
or B
12
defi ci enci es may wel l devel op oral si gns and symptoms before the erythrocytes are
aff ected and bef ore anaemi a devel ops. Agai n, thi s i s an argument for the necessi ty for a f ul l
haematol ogi cal exami nati on i n these pati ents. It may wel l be that the pati ents devel opi ng
oral si gns at an earl y stage of a haematol ogi cal abnormal i ty represent a sel ected group wi th
an unusual l y sensi ti ve mucosal response to the changes.
Iron deficiency anaemia
A much more common cause of anaemi a than fai l ure to absorb vi tami n B
12
or fol i c aci d i s i ron
def i ci ency, whi ch l eads to i nadequate haemogl obi n synthesi s. The defi ci ency may be due
P. 154
ei ther to i nadequate i ntake of i ron or to excessi ve bl ood l oss as i n menstrual abnormal i ty or
gastroi ntesti nal bl eedi ng. In i ron defi ci ency anaemi a, the essenti al f eature observed i s a
reducti on i n the haemogl obi n concentrati on wi thi n the erythrocytes (hypochromi c). They
appear pal e on mi croscopi c exami nati on, and there may be vari ati ons i n si ze and shape. In
i ron defi ci ency the erythrocytes are usual l y smal l (mi crocyti c). However, the number of
erythrocytes per uni t vol ume may not vary greatl y from i ts normal val ue. The erythrocyte
count i s not consi dered to be a parti cul arl y i mportant di agnosti c test i n most cases.
Of the total i ron content of the bl ood, by f ar the greater proporti on i s combi ned i n the form
of haemogl obi n wi thi n the red bl ood cel l s. A smal l f racti on i s present i n the pl asma, bound to
a speci f i c protei n, transf erri n, and represents the transport i ron made avai l abl e f rom the
body reserves to repl ace haemogl obi n l osses. If the stores become exhausted, there i s a
peri od of l atent i ron defi ci ency i n whi ch the haemogl obi n concentrati on i s wi thi n normal
l i mi ts and the erythrocytes are of normal si ze and f orm, but the serum i ron concentrati on i s
reduced. Thi s i s si deropeni a, an i ron defi ci ency that may af fect the ti ssues and i s capabl e of
causi ng oral symptoms, but whi ch does not produce anaemi a si nce the haemogl obi n remai ns
unaff ected. When the serum i ron i s depl eted i n thi s way, the degree of saturati on of the
transferri n by the i ron wi l l evi dentl y be reduced. Thi s f orms the basi s of a val uabl e di agnosti c
test. In more compl ex condi ti ons than i ron def i ci ency, there may al so be a reducti on i n the
ci rcul ati ng transferri n and so the degree of saturati on may remai n hi gh i n spi te of a l ow
serum i ron val ue. As was poi nted out i n Chapter 2, the test f or serum ferri ti n l evel s i s now
regarded as the best general i ndi cator of body i ron stores.
The stages of i ron defi ci ency are summari zed i n Tabl e 13. 1. The serum ferri ti n l evel s, as a
measure of the overal l body i ron status, woul d be expected to fal l over these three stages.
Oral symptoms may appear i n the second and thi rd of these stages.
Haemolytic anaemias
There i s a f urther group of anaemi as, the haemol ytic anaemi as, i n whi ch the essenti al
abnormal i ty i s an i ncrease i n the rate of erythrocyte destructi on. Under normal condi ti ons,
the red cel l s l ast f or about 100 days, but i n haemol yti c anaemi as thei r l i f e may be reduced to
onl y a f ew days. Haemol yti c anaemi as may be due to an i ntri nsi c def ect or may be
acqui redan i mportant, al though rel ati vel y uncommon, cause of the acqui red form bei ng
the effect of some drugs (Tabl e 13. 2). However, the most i mportant haemol yti c anaemi a i n
terms of dental practi ce i s si ckl e-cel l anaemi a, although thi s condi ti on i s somewhat di ff erent
from the others under consi derati on i n that i ts major si gni fi cance to the denti st i s not i n the
producti on of oral l esi ons.
SICKLE-CELL DISEASES
The si ckl e-cel l di seases are a group of geneti cal l y determi ned condi ti ons i n whi ch the red
cel l s contai n an abnormal haemogl obi n, HbS. When HbS l oses oxygen i t undergoes changes
that

produce di storti on of the cel l s, the si ckl e effect. HbS i s i nheri ted as an autosomal recessi ve
condi ti on preval ent i n those wi th Bl ack Af ri can/Cari bbean ancestry and i n some f ami l i es from
the Mi ddl e and Far Eastern countri es. The carri er state (i n whi ch the pati ent i nheri ts the
condi ti on f rom onl y one parent) i s known as the si ckl e-cel l trai t and i s by far the more
common condi ti on. In thi s trai t the proporti on of HbS i n the red cel l s i s l ow and si ckl i ng does
not take pl ace under normal ci rcumstances. However, si ckl i ng may occur i n condi ti ons of l ow
oxygen tensi on and, i f thi s change does take pl ace, the oxygen-carryi ng capaci ty of the bl ood
i s greatl y reduced wi th the consequent possi bi l i ty of anoxi a i n the pati ent. The ful l y
devel oped si ckl e-cel l anaemi a i s the resul t of the presence of two HbS genes, one from each
parent. In thi s case, the proporti on of HbS i s hi gh and si ckl i ng occurs under normal body
condi ti ons. In these pati ents, the oxygen-carryi ng capaci ty of the bl ood i s poor, there i s
i mpai rment of vascul ar fl ow, and a haemol yti c anaemi a resul ts f rom the shortened l i fe of the
P. 155
abnormal erythrocytes. Thi s i s a severe condi ti on and the symptoms of general i l l heal th are
so marked as to make i t very unl i kel y that a pati ent woul d present for dental treatment
undi agnosed. However, there i s no such guarantee i n the case of the si ckl e-cel l trai t and the
onl y way to i denti fy these pati ents i s to carry out a test for the presence of HbS.
Fortunatel y, the i ni ti al screeni ng test f or thi s condi ti on i s rel ati vel y si mpl e and i s easi l y
performed i n the l aboratory. It i s al so true that modern techni ques of anaesthesi a, desi gned
to avoi d even transi ent and mi nor degrees of hypoxia, are much l ess l i kel y to cause probl ems
i n these pati ents than previ ousl y was the case.
Orofaci al mani festati ons of si ckl e-cel l anaemi a have been reported and i ncl ude promi nence of
the maxi l l a and mandi bl e and orofaci al pai n. These occur as a resul t of marrow hyperpl asi a
and expansi on of the marrow space due to a l ongstandi ng haemol yti c anaemi a. Destructi ve
osteomyel i ti s i s a recogni zed compl i cati on of dental i nfecti on i n si ckl e cel l di sease. Peri pheral
neuropathi es associ ated wi th si ckl e cel l cri si s have al so been reported, i ncl udi ng mental
anaesthesi a.
Table 13.1 Stages of iron deficiency
1. Pre-l atent i ron defi ci ency, i n whi ch the body stores of i ron are depl eted, but
the ci rcul ati ng haemogl obi n and serum f erri ti n remai n wi thi n normal l i mi ts
2. Latent i ron def i ci ency, i n whi ch the body stores are exhausted and the serum
ferri ti n reduced. The haemogl obi n concentrati on remai ns unaffected
3. Latent i ron def i ci ency, i n whi ch the body stores are exhausted and the serum
ferri ti n reduced. The haemogl obi n concentrati on remai ns unaf fected
Table 13.2 Oral signs and symptoms of haematinic
deficiencies
Gl ossi ti s
Smooth, depapi l l ated tongue (i ron def i ci ency)
Raw, beefy tongue (vi tami n B
12
and fol ate defi ci enci es)
Oral candi dosi s (i ncl udi ng angul ar chei l i ti s)
Exacerbati on of RAS
Pl ummerVi nson (PattersonKel l y) syndrome (i ron defi ci ency)
Normocytic anaemias
Normocyti c anaemi as may be secondary to systemi c di seases, such as scurvy (see bel ow) or
associ ated wi th pri mary or secondary bone marrow apl asi a and neopl asi a. Apl asti c anaemi as
can be caused by a number of cytotoxi c drugs.
Oral signs and symptoms in anaemias
A wi de range of oral si gns and symptoms may appear i n anaemi c pati ents but these are due
to the underl yi ng defi ci ency of i ron, vi tami n B
12
, or fol i c aci dfor mucosal pal l or to be
noti ceabl e i n the mouth the pati ent' s haemogl obi n needs to be l ow (< 8 g/dl ). The oral si gns
and symptoms are a resul t of basi c changes i n the metabol i sm of the oral epi thel i al cel l s,
whi ch are parti cul arl y suscepti bl e to mi nor vari ations i n the qual i ty of the bl ood suppl y.
These changes gi ve ri se i n thei r turn to abnormal i ti es of cel l structure and of the
kerati ni zati on pattern of the oral epi thel i um, the end resul t often bei ng atrophy. Thi s atrophy
seems parti cul arl y to aff ect the compl ex fi l i f orm papi l l ae of the tongue, whi ch may be al most
compl etel y l ost. However, the changes are by no means restri cted to the tongue and
ul cerati on or general i zed soreness may occur over the whol e of the oral mucosa. Apart from
thi s type of symptom, the pati ent wi th anaemi a or l atent i ron def i ci ency i s parti cul arl y
suscepti bl e to i nfecti on by Candi da al bi cans and angul ar chei l i ti s or thrush may occur. In
addi ti on to these changes, the pati ents may compl ai n of di sturbances of taste sensati on. It
has been suggested that thi s i s due to atrophy of the tongue epi thel i um wi th resul ti ng
di sturbance of the underl yi ng nerve endi ngs, but such a di sturbance of taste has been
observed i n pati ents wi th apparentl y cl i ni cal l y normal tongue epi thel i um. A number of reports
have descri bed the resul ts of the i nvesti gati on of oral l esi ons i n anaemi c pati ents and i t has
become evi dent that there i s no cl ear correl ati on between the oral symptoms and the basi c
aeti ol ogy. Sore tongue, taste di sturbance, general i zed stomati ti s, candi dosi s, angul ar
chei l i ti s (Fi g. 13. 1), gi ngi vi ti s, and an exacerbati on of recurrent aphthous stomati ti s (RAS)
may occur i n any of these pati ents (see Tabl e 13. 2).
The same consi derati ons appl y i n rel ati on to f ol ate and B
12
defi ci ency. It has been poi nted
out above that very earl y defi ci enci es of ei ther of these factors may resul t i n oral mucosal
changes. These are certai nl y not due to secondary anaemi a si nce none may be present. The
preci se reason for these changes i s not known.
In the Pl ummerVi nson (PattersonKel l y) syndrome, pati ents present wi th dysphagi a,
caused by an oesophageal web, i ron

def i ci ency anaemi a (hence the term si deropeni c dysphagi a), and gl ossi ti s. They may al so
devel op oral candi dosi s and are predi sposed to the devel opment of postcri coi d and oral
carci noma (see al so Chapter 10).
P. 156
The rel ati onshi p between RAS and coel i ac di sease was di scussed i n Chapter 5. It i s general l y
accepted that RAS i s not di rectl y associ ated wi th iron defi ci ency (as may occur i n coel i ac
di sease), al though pre-exi sti ng RAS may wel l be exacerbated by i t. It i s l ess easy to def i ne
the rol e of fol ate or B
12
defi ci enci essome def i ci ent pati ents wi th RAS show an i mmedi ate
response to suppl ementati on whi l st, qui te cl earl y, those wi th coel i ac di sease respond
pri mari l y to the gl uten-free di et. Thi s, however, resul ts i n the correcti on of the
mal absorpti on process and the restorati on of defi ci ent l evel s. It i s, therefore, di ff i cul t to tel l
preci sel y what part each component pl ays i n the reversal of the RAS.
Further uncertai nty i s i ntroduced by the fact that some pati ents wi th advanced
haematol ogi cal di sturbances do not show oral changes. Some pati ents, f or exampl e, those
wi th advanced megal obl asti c anaemi a, the resul t of fol ate defi ci ency, may have no oral
compl ai nts and the mucosa may appear to be wi thi n normal cl i ni cal l i mi ts. It woul d seem
reasonabl e to revi se the l ong-hel d vi ew that cl ear-cut oral si gns and symptoms may be
ascri bed to speci fi c bl ood defi ci enci es, and to adopt the al ternati ve vi ew poi nt that a wi de
range of oral changes may ari se f rom any of the condi ti ons under di scussi on.
Management of patients with anaemias and haematinic
deficiencies
In the majori ty of the pati ents descri bed, treatment of the underl yi ng haematol ogi cal
def i ci ency l eads to a rapi d resol uti on or i mprovement i n the oral symptoms. In pati ents wi th
l ongstandi ng l atent i ron defi ci ency, however, the response may be a sl ow one. Such
refractory behavi our i s wel l recogni zed and may i ndi cate poor drug compl i ance or i mpai red
pati ent absorpti on of repl acement therapy.
Leukaemia
Leukaemi a represents a mal i gnant prol i ferati on of whi te cel l s, repl aci ng thei r normal
devel opment i n the bone marrow. Thi s process may affect any of the whi te cel l strai ns, but
the most usual forms are l ymphocyti c, monocyti c, and myel oi d, dependi ng on whether
l ymphocytes, monocytes, or granul ocytes are i nvol ved. Each of these forms of l eukaemi a
present ei ther i n an acute or a chroni c form (see Tabl e 13. 3).
It i s not uncommon for oral si gns and symptoms to be the fi rst i ndi cati on of the presence of
Fig. 13.1 Bi l ateral angul ar chei l i ti s and a general i zed stomati ti s i n uncontrol l ed
perni ci ous anaemi a.
l eukaemi a, parti cul arl y of one of the acute types (see Tabl e 13. 4). The gi ngi vae are
frequentl y i nvol ved wi th a hyperpl asti c gi ngi vi ti s. The hyperpl asti c gi ngi vi ti s resul ts i n fragi l e
red spongy gi ngi vae that bl eed spontaneousl y or f oll owi ng sl i ght i njury. In a few cases, a
mi l d gi ngi val hyperpl asi a may be the fi rst i ndi cati on of an acute l eukaemi a (Fi g. 13. 2). In
acute monobl asti c l eukaemi a (a subgroup of acute myel obl asti c l eukaemi a), the monocytes
are known to i nfi l trate si tes of i nf l ammati on such as the gi ngi va. In more acute cases, the
hyperpl asti c nature of the gi ngi vi ti s may not be evi dent and the condi ti on may show as
spontaneous haemorrhage from the gi ngi val margi ns (Fi g. 13. 3). The gi ngi vae are hi ghl y
suscepti bl e to i nfecti on and a secondary acute ul cerati ve gi ngi vi ti s i s common. Thi s
suscepti bi l i ty to i nf ecti on occurs al so i n more chroni c cases when the oral symptoms may
consi st of recurrent attacks of acute ul cerati ve gingi vi ti s. These may occur wi thout any of the
hyperpl asti c changes menti oned above and so may appear to be not of great si gni fi cance.
Unexpl ai ned or repeated recurrence of acute ul cerati ve gi ngi vi ti s shoul d be treated wi th
suspi ci on and bl ood exami nati on arranged i n order to el i mi nate the possi bi l i ty of the bl ood
dyscrasi a. The orofaci al mani festati ons of chroni c

l eukaemi as are si mi l ar to those of acute l eukaemi as but are of ten l ess fl ori d and may present
i n an i nsi di ous manner. In chroni c l ymphocyti c l eukaemi a there i s i nvari abl y l ymph node
i nvol vement. Leukaemi c i nf i l trati on of the sal i vary and l acri mal gl ands i s occasi onal l y seen.
P. 157
Table 13.3 Classification of leukaemias
Acute Chronic
Lymphobl asti c* Lymphocyti c
Nonl ymphobl asti c (myel obl asti c) Myel oi d
*Most common acute l eukaemi a i n chi l dren.
Most common acute l eukaemi a i n adul ts
Table 13.4 Orofacial manifestations of acute leukaemia*
Spontaneous haemorrhage for gi ngi vae (reducti on i n pl atel ets)
Oral purpura and petechi ae
Gi ngi val swel l i ng (l eukaemi c i nf i l trati on)
Oral ul cerati on (l eukaemi c deposi ts, i nfecti ons, haemati ni c def i ci enci es)
Mucosal swel l i ng and l ooseni ng (exfol i ati on) of teeth (l eukaemi c deposi ts)
Opportuni sti c i nf ecti ons (e.g. herpes, Candi da)
Lymph node enl argement
*N.B. Chemotherapy to treat l eukaemi a may al so produce oral si de-eff ects.
Fig. 13.2 Mi l d gi ngi val hyperpl asi athe fi rst i ndi cati on of acute myel obl asti c
l eukaemi a.
In more advanced cases of l eukaemi a, oral ul cerati on i s very common. The ul cers, produced
by the breakdown of the ti ssues overl yi ng deposi ts of l eukaemi c cel l s, may be l arge, pai nful ,
and di ffi cul t to treat. The mai ntenance of oral hygi ene may be of great hel p i n reduci ng the
di stressi ng oral symptoms i n these pati ents. Coveri ng agents and anti septi c mouthwashes
are al so hel pf ul i n easi ng the pai nful symptoms duri ng the l ate stages of the di sease.
Leukopenia
Leukopeni a represents a fal l i n the whi te cel l content of the bl ood. Thi s may be a
spontaneousl y ari si ng condi ti on, but may occur al so as a response to drug therapy.
Carbamazepi ne has been associ ated wi th severe haematol ogi cal effects, i ncl udi ng l eukopeni a
and apl asti c anaemi a. Leukopeni a may al so occur as a transi ent stage i n the devel opment of
l eukaemi a and other di seases af fecti ng the bone marrow. It may al so resul t from
autoi mmune di sorders such as systemi c l upus erythematosus (SLE) and vi ral i nfecti ons;
parti cul arl y HIV/EBV i nfecti ons.
Al though i t i s not a parti cul arl y common condi ti on, the most usual cl i ni cal presentati on i s of
agranul ocytosi s. Thi s represents a reducti on i n the number of granul ocytes formed i n the
marrow and ci rcul ati ng i n the bl ood. The eff ect of thi s i s to i ncrease the suscepti bi l i ty of the
pati ent to i nfecti ons of vari ous ki nds. In the case of the oral mucosa thi s may l ead to
wi despread i nf ecti on and ul cerati on of al l parts of the mucosa. These changes may not be
di ssi mi l ar to those occurri ng i n l eukaemi a. The aeti ol ogi cal process i s si mi l ar i n that the
protecti ve functi on of the whi te cel l component of the bl ood i s reduced, i n the one case by
the excessi ve producti on of abnormal cel l s and, i n the other case, by an i nadequate
producti on of normal cel l s.
In cycl i c neutropeni a, a rare condi ti on i n whi ch neutrophi l producti on i s i ntermi ttentl y
def i ci ent, gi ngi vi ti s and RAS-l i ke ul cerati on may occur duri ng the neutropeni c epi sodes (see
Chapter 5).
Myelodysplastic syndromes
Thi s i s a group of stem cel l di sorders i n whi ch there i s a suppressi on of one or more cel l l i nes
i n the bone marrow. In some cases thi s l eads to bone marrow fai l ure and i n others to
l eukaemi a. Myel odyspl asti c syndromes are more common i n mal es over 60 years of age and
may be detected i n the oral medi ci ne cl i ni c when pati ents undergo routi ne haematol ogi cal
screeni ng. An abnormal bl ood count i n an el derl y pati ent shoul d therefore suggest the
Fig. 13.3 Spontaneous haemorrhage f rom the gi ngi val margi n in acute l eukaemi a.
possi bi l i ty of a myel odyspl asti c syndrome. Oral ul cerati on and gi ngi val i nf i l trati on have been
reported i n a f ew cases.
Platelet abnormalities
When the functi on or the number of the pl atel ets i n the ci rcul ati ng bl ood i s reduced, there i s
a tendency for spontaneous haemorrhage to occur wi thi n the ti ssues. Thi s may wel l show
i ni ti al l y i n the form of petechi al haemorrhages on the oral mucosa and these are, i n fact, a
wel l -known si gn of earl y i mmune thrombocytopeni a. In l eukaemi as of vari ous ki nds, both
pl atel et functi on and numbers are greatl y reduced, and haemorrhages of mucosa and ski n
may theref ore be an earl y warni ng si gn. It i s advi sabl e to carry out a ful l bl ood screen,
i ncl udi ng a pl atel et count, on any pati ent wi th otherwi se unexpl ai ned areas of haemorrhage
aff ecti ng the oral mucosa. It shoul d be remembered, however, that transi ent haemorrhages
of thi s ki nd may occur on the soft pal ate i n pati ents wi th a severe col d. Idi opathi c oral bl ood
bl i sters (wi thout any associ ated haematol ogi cal defect) were descri bed i n Chapter 11. Si mi l ar
bl ood-fi l l ed bl i sters may al so be produced i n thrombocytopeni a, parti cul arl y on the pal ate,
al though they may occur el sewhere on the oral mucosa.
The selection of patients for haematological examination
Pati ents wi th the symptoms descri bed i n Tabl e 13. 5 form a consi derabl e proporti on of those
referred to the oral medi ci ne cl i ni c for i nvesti gati on and, as a consequence, a ful l
haematol ogi cal exami nati on must be carri ed out i n al l these cases. The accepted routi ne
bl ood screen, consi sti ng of a ful l bl ood cel l count (haemogl obi n (Hb), di f ferenti al
whi te cel l count (WCC), and pl atel et esti mati on) and the exami nati on of a bl ood f i l m, i s
someti me i nsuffi ci ent to demonstrate anaemi as i n thei r earl y or l atent stages but i t i s
i mportant to extend the i nvesti gati on further i n sel ected pati ents (Tabl e 13. 5). The rati onal e
for thi s has al ready been di scussed i n Chapter 2. When an extended i nvesti gati on i s deci ded
upon, a reasonabl e scheme of i nvesti gati on i s that shown i n Tabl e 13. 6.
It i s evi dent that further, more speci al i zed tests may be necessary for a ful l di agnosi s of
some of the pati ents i nvol ved. A number of these were di scussed i n Chapter 2.
It i s essenti al that the presence of some haematol ogi cal abnormal i ty shoul d be fol l owed up
wi th appropri ate i nvesti gati ons accordi ng to the cl i ni cal ci rcumstances. Si mpl e repl acement
therapy for a def i ci ency i s unacceptabl e unl ess attempts are made

to defi ne the underl yi ng cause. It must be sai d, however, that, even after carryi ng out ful l
i nvesti gati ons, a smal l number of pati ents presenting wi th oral symptoms attri buted to
def i ci enci es remai n a di agnosti c puzzl e i n terms of the aeti ol ogy of the defi ci ency.
P. 158
Disorders of nutrition
The i ntegri ty of the oral mucous membrane i s mai ntai ned by a wi de-rangi ng compl ex of
factors, i ncl udi ng those dependent on adequate nutri ti on. The si gni f i cance of i ron metabol i sm
and associ ated factors was previ ousl y di scussed, as was the rel ati onshi p between
gastroi ntesti nal di sease and nutri ti on.
Nutritional deficiencies
A wi de range of condi ti ons that depend on the absence or reducti on of certai n speci f i c
nutri ti onal factors, parti cul arl y vi tami ns, has been descri bed i n the past. Wi th a few
excepti ons these speci f i c condi ti ons are now rarel y seen under European condi ti ons, al though
thi s may certai nl y be f ar from the case i n other si tuati ons. It shoul d be remembered that a
Table 13.5 Oral medicine patients who should be
considered for an extended haematological examination
Pati ents wi th recurrent aphthous stomati ti s (RAS)
Pati ents wi th a persi stentl y sore and/or dry mouth
Pati ents wi th oral l esi ons wi th an atypi cal hi story or unusual l y resi stant to
treatment
Pati ents compl ai ni ng of a sore or burni ng mouth or tongue, or abnormal taste
sensati on, even though no mucosal changes can be seen
Al l pati ents wi th persi stent oral and orofaci al candi dosi s
Pati ents showi ng abnormal i ti es fol l owi ng an i ni ti al screeni ng
Table 13.6 Haematological investigations for oral
medicine patients
Ful l bl ood count and fi l m exami nati on. Thi s i s the routi ne screen procedure,
and from i t evi dent anaemi as are demonstrated by vari ati ons i n red cel l
morphol ogy and l owered haemogl obi n val ues. Abnormal i ti es of the whi te cel l s
and pl atel ets are al so shown
Esti mati on of serum ferri ti n as an i ndi cator of full -body i ron status. Thi s has
repl aced the previ ousl y used serum i ron/i ron bi ndi ng capaci ty/saturati on tests
when used for thi s purpose. Thi s l atter group of tests, however, i s sti l l used
i n the i nvesti gati on of compl ex i ron defi ci ency states
Serum B12, serum fol ate, and red cel l fol ate esti mati ons. The necessi ty for
carryi ng out both fol ate esti mati ons has been di scussed i n Chapter 2
As an addi ti onal test an erythrocyte sedi mentati on rate (ESR) measurement i s
useful as a non-speci f i c gui de to underl yi ng pathol ogi cal processes such as
chroni c i nfl ammatory condi ti ons or neopl asi aal ternati vel y, measurement of
C-reacti ve protei n (CRP) may be used as a marker f or pre-exi sti ng di sease
nutri ti onal defi ci ency may occur i n three ways: (1) as a resul t of reduced i ntake; (2) as a
resul t of faul ty absorpti on or metabol i sm; and (3) as a resul t of i ncreased excreti on. The
rel ati onshi p of i ron defi ci ency anaemi a to these three factors i s a good and si mpl e exampl e.
Most pati ents seen i n the oral medi ci ne cl i ni c wi th f ol ate defi ci ency have thi s as a resul t of
some form of mal absorpti on rather than poor i ntake. It shoul d be remembered, however, that
a hi gh al cohol i ntake may resul t i n l ow f ol ate l evel s, as may some drugsphenytoi n i n
parti cul ar. Onl y rarel y, and then usual l y i n stri ct vegans, i s vi tami n B
12
defi ci ency a resul t of
poor di etary i ntake. On the whol e, those pati ents who adopt unusual di ets do so on a
reasonabl y i nformed basi s and are found to have sati sfactory haematol ogi cal i ndi ces.
There has recentl y been consi derabl e i nterest i n the rol e of other nutri ti onal el ements i n the
i ntegri ty of the oral mucosathe B compl ex of vi tami ns and a number of trace el ements
(parti cul arl y zi nc) have been the subject of i nvesti gati ons. Serum zi nc l evel s have been
i nvesti gated i n pati ents wi th burni ng mouth syndrome (BMS) and geographi c tongue,
but there i s no convi nci ng evi dence that zi nc defi ci ency i s i nvol ved i n the pathogenesi s of
these condi ti ons.
It was previ ousl y poi nted out that, i n pati ents wi th nutri ti onal def i ci enci es, secondary eff ects
may f ol l ow. Predomi nant amongst these i s the suppressi on of the normal i mmune response.
Thi s was descri bed i n Chapter 4 i n the case of nutri ti onal l y defi ci ent chi l dren af fected by
cancrum ori s. Nutri ti onal def i ci enci es dependent on faul ty di et are rarel y si mpl e ones and the
pati ent suff eri ng f rom any speci fi c def i ci ency shoul d be consi dered a candi date f or a more
compl ete i nvesti gati on of nutri ti onal standards.
A speci al case i s that of the anorexi c pati entmost commonl y, but not excl usi vel y, young
and femal e. Such pati ents may come to the oral medi ci ne cl i ni c as a resul t ei ther of general
nutri ti onal defi ci enci es, whi ch may resul t i n stomati ti s or some other mucosal probl em, or of
the paroti d swel l i ng that i s a f eature i n some patients. The aci d-i nduced erosi on of the teeth
that may occur i n pati ents wi th the bul i mi c form of anorexi a i s di scussed i n Chapter 17.
Scurvy
Scurvy (ascorbi c aci d def i ci ency) i s now an uncommon di sease i n Europe, but i s not by any
means unknown, bei ng the most commonl y recogni zed condi ti on associ ated wi th a si ngl e
vi tami n defi ci ency. Al though the di sease occurs more often i n ol d and negl ected pati ents,
there are occasi onal cases of much younger i ndi vi dual s who adopt such a restri cted form of
di et that cl i ni cal si gns of ascorbi c aci d def i ci ency appear.
The predomi nant oral symptom i n a severe vi tami n C def i ci ency i s a hyperpl asti c gi ngi vi ti s,
the gi ngi vae becomi ng swol l en and f ri abl e and purple-red i n col our. There i s marked f al se
pocketi ng and thi s, together wi th general l ack of ti ssue resi stance, may l ead to secondary
i nfecti on. There may be, therefore, a superi mposed acute gi ngi vi ti s. General i zed symptoms
i ncl ude ti redness and mal ai se. Capi l l ary fragi l i ty i s a feature of thi s condi ti on and may l ead
to the appearance of spontaneous haemorrhage and widespread brui si ng, parti cul arl y around
the joi nt areas.

Investi gati on of such a pati ent must i ncl ude a bl ood exami nati on i n order to el i mi nate the
possi bi l i ty of a bl ood di sease, si nce the most i mportant di f ferenti al di agnosi s i s from
l eukaemi a. The mai n bl ood change i n scurvy i s the presence of a secondary anaemi a. The
posi ti ve di agnosi s i s by l aboratory tests for l eukocyte or pl asma ascorbi c aci d l evel s.
However, a sati sfactory cl i ni cal di agnosti c test i s deri ved from the response of the symptoms
to therapeuti c doses of vi tami n C, a response that occurs wi thi n a few days and that i s
accompani ed by a dramati c reversal of al l the symptoms.
The treatment of scurvy i s a general medi cal and, someti mes, soci al probl em. Al though the
admi ni strati on of a hi gh dose of ascorbi c aci d (1 g dai l y) f or a few days may i mprove the
condi ti on of the pati ent remarkabl y, the further management must be a matter f or the
P. 159
general medi cal practi ti oner. Not onl y the defi ci ency state i tsel f, but al so the condi ti ons
l eadi ng to i ts appearance must be corrected. It shoul d al so be remembered that, al though
the symptoms presenti ng may be of ascorbi c aci d defi ci ency, there i s every l i kel i hood that, i n
fact, mul ti pl e di etary defects exi st.
Scurvy (vi tami n C defi ci ency) i s rare. In advanced cases there may be a marked gi ngi vi ti s
wi th swel l i ng or bl eedi ng.
Endocrine disturbances
In general , changes i n the oral mucosa di ctated by endocri ne abnormal i ti es are not common.
Perhaps the most frequentl y ci ted changes are those due to the endocri ne di sturbances f ound
i n normal l i feespeci al l y duri ng pregnancy, and at the menopause. However, a few wel l -
establ i shed oral changes occur i n some endocri ne pathol ogi es and these wi l l be outl i ned
bel ow. It shoul d be remembered that endocri ne di sorders are hi ghl y compl ex and of ten
i nvol ve a number of systems because of the feedback mechani sms that control the endocri ne
system as a whol e. It i s, therefore, often di f fi cult to determi ne the exact ef fect of a si ngl e
endocri ne abnormal i ty on any structure. Endocri ne di sorders affecti ng bone and teeth
(gi ganti sm, acromegal y, and hyperparathyroi di sm) are di scussed i n Chapter 18.
Hypoparathyroi di sm resul ti ng from parathyroi d or thyroi d surgery has no parti cul ar eff ects on
the orofaci al ti ssues but resul ts i n a l ow serum cal ci um. Tetany i s the cl i ni cal mani festati on
of reduced serum cal ci um. Tetany may al so be encountered i n anxi ous dental pati ents who
hyperventi l atethi s resul ts i n al kal osi s and a reduced pl asma i oni zed cal ci um.
Normal endocrine changes
Pregnancy
Duri ng pregnancy, the hormonal changes that occur may have the eff ect of exacerbati ng a
previ ousl y exi sti ng chroni c gi ngi vi ti s that may have been previ ousl y symptom-free and
unrecogni zed. The resul ti ng gi ngi vi ti s i s essenti all y hyperpl asti c, al though there i s mi ni mal
prol i ferati on of f i brobl asts. The most marked prol iferati on i s of capi l l ari es and thi s l eads to
the typi cal l y purpl e col orati on of the gi ngi val papi l l ae (Fi g. 13. 4). These papi l l ae tend to be
fragi l e and may bl eed at the l east i njury. Because of the presence of fal se pocketi ng and
bl eedi ng, stagnati on and secondary i nf ecti on may occur, and may l ead to hal i tosi s.
Occasi onal l y, a si ngl e papi l l a may become consi derabl y enl arged and present as an epul i s.
Thi s i s the so-cal l ed pregnancy epul i s (Chapter 9). The cl i ni cal characteri sti cs and
ti mi ng of the occurrence of these pregnancy l esi ons are suff i ci ent to gi ve a strong
presumpti ve di agnosi s. However, shoul d any doubt occur as to the nature of the condi ti on,
ful l i nvesti gati on i s essenti al , i ncl udi ng exci si onal bi opsy of any doubtful overgrowth.
Pregnancy i s, i n i tsel f, no contrai ndi cati on to such a bi opsy, but i t shoul d be remembered
that the l esi on i s l i kel y to be extremel y vascul ar and prof use bl eedi ng i s to be expected. On
bal ance, i t i s often better, i f a confi dent cl i ni cal di agnosi s has been made, to avoi d bi opsy,
si nce the condi ti on i s l i kel y to regress consi derabl y, i f not compl etel y, after pregnancy. The
rel ati onshi p of RAS to pregnancy and the menstrual cycl e has not yet been establ i shed.
Treatment duri ng pregnancy shoul d consi st of the appl i cati on of stri ct oral hygi ene measures.
Thi s, i n i tsel f , i s often suf fi ci ent to hal t the progress of the gi ngi vi ti s. However, oral hygi ene
measures al one are unl i kel y to l ead to the compl ete resol uti on of a di screte epul i s-
l i ke mass and eventual exci si on may be necessary.
Menopause
There i s no substanti ve evi dence that the hormonal changes occurri ng duri ng and af ter the
menopause di rectl y affect the oral mucosa. The questi on of oral symptoms of vari ous ki nds
wi th no i denti f i abl e physi cal cause or abnormal i ty i s further addressed i n Chapter 17. These
have a tendency to occur i n menopausal women.
Adrenocortical diseases
Addison's disease
Addi son' s di sease i s the resul t of l ack of functi on of the adrenal cortex, usual l y the resul t of
an autoi mmune di sorder, but wi th other possi bl e aeti ol ogi es. As a resul t of thi s destructi on,
the

feedback mechani sm between the adrenal s and the pi tui tary i s di sturbed and a wi de-rangi ng
seri es of endocri ne changes resul ts. The oral change of si gni fi cance i n Addi son' s di sease i s
mel anoti c pi gmentati on of the oral mucosa, whi ch may i ncl ude the buccal mucosa, gi ngi vae,
and pal ate. It shoul d be remembered that thi s form of pi gmentati on i s by no means speci fi c,
but none the l ess the appearance of such si gns i n a pati ent known previ ousl y to be f ree of
pi gmentati on shoul d al ways be consi dered as of si gni f i cance. The mechani sm of mel ani n
producti on i n thi s way i s not cl earl y known. Al though a mel ani n-sti mul ati ng hormone i s
secreted by the pi tui tary gl and, i t seems that the actual onset of pi gmentati on may be
associ ated wi th vari abl e adrenocorti cotrophi c hormone (ACTH) l evel s. In more f ul l y
devel oped Addi son' s di sease, oral candi dosi s may al so occur. The associ ati on of oral
mel anoti c pi gmentati on wi th candi dosi s i s evi dentl y a f urther, more marked i ndi cati on f or
endocri ne studi es. Investi gati on of a pati ent wi th suspected Addi son' s di sease i s shown i n
Tabl e 13. 7.
Al though Addi son' s di sease i s a wel l -known cause of oral mucosal pi gmentati on, i t i s, i n fact,
very unusual for the di sease to be recogni zed i n thi s way. There are many much more
common causes of mel ani n pi gmentati on, whi ch were consi dered i n Chapter 9.
Cushing's syndrome
Thi s i s due to hyperfuncti on of the adrenal cortex, usual l y as a resul t of an ACTH-secretory
Fig. 13.4 Pregnancy gi ngi vi ti s, i ncl udi ng the formati on of a pregnancy epul i s.
P. 160
pi tui tary adenoma. Corti costeroi d therapy, parti cularl y i f prol onged, can have physi ol ogi cal
eff ects si mi l ar to those of Cushi ng' s syndrome (see Chapter 2).
Phaeochromocytoma and the adrenal medulla
Phaeochromocytoma i s a tumour of the adrenal medul l a, whi ch secretes catechol ami nes. It
can be associ ated wi th neurofi bromatosi s and the mul ti pl e endocri ne adenoma syndrome
(type III). Neurofi bromas of the oral mucosa or l i ps shoul d al ert the cl i ni ci an to the rare
possi bi l i ty of a phaeochromocytoma.
Thyroid disease
Hyperthyroidism
Excessi ve producti on of thyroi d hormones (hyperthyroi di sm) does not appear to have any
di rect ef fect on the oral mucosa but may cause probl ems of dental management. Dental
practi ti oners may be the f i rst to observe pati ents wi th exophthal mos who may al so appear
hyperexci tabl e and report wei ght l ossthey may also have a tremor and tachycardi a.
Treatment i s usual l y by drugs such as carbi mazol e or use of radi oacti ve i odi ne. Parti al
thyroi dectomy i s rarel y requi red unl ess the thyroi d i s causi ng compressi on symptoms.
Hypothyroidism
Hypothyroi di sm i n adul ts i s often autoi mmune but may occur as a consequence of excessi ve
removal of the thyroi d gl and to treat hyperthyroi dism. Acqui red hypothyroi di sm
(myxoedema) mani fests as wei ght gai n, i nabi l i ty to tol erate col d, dry ski n, l oss of hai r, and a
sl owi ng down of acti vi ty and mental processes. Hypothyroi di sm i s associ ated wi th an
i mpai red i mmune mechani sm and oral candi dosi s may be the resul t. There i s often a
rel ati onshi p between perni ci ous anaemi a and hypothyroi di smpossi bl y i n the fami l y i f not
i n the i ndi vi dual .
Congeni tal hypothyroi di sm (creti ni sm) i s characteri zed by dwarfi sm and mental retardati on.
Orofaci al si gns i ncl ude enl argement of the tongue (see Chapter 6), defecti ve faci al
devel opment, and del ayed erupti on of the teeth.
Diabetes mellitus
Table 13.7 Investigation of patient with melanotic
pigmentation due to possible Addison's disease
Impai red adrenal response to syntheti c anal ogue of ACTH (Synacthen test)*
Measurement of serum el ectrol ytes (Na , K )
Measurement of pl asma corti sol () and ACTH( )
Bl ood pressure measurement (hypotensi onespeci al l y postural )
*Defi ni ti ve test for Addi son' s di sease.
May be normal .
Di abetes mel l i tus i s a common endocri ne di sorder that occurs as a resul t of a defi ci ency of
i nsul i n or resi stance to i nsul i n. Two cl i ni cal types are recogni zed: juveni l e onset (i nsul i n-
dependent, type 1) and maturi ty onset di abetes (type 2).
Di abetes mel l i tus has no speci fi c oral si gns or symptoms. However, possi bl y because of the
general l ack of resi stance to i nfecti on of the di abeti c pati ent, peri odontal di sease processes
may become exaggerated (Tabl e 13. 8). It i s not uncommon to f i nd that an undi agnosed
di abeti c presents wi th advanced peri odontal di sease. The pri nci pl es of treatment of thi s are
si mpl y those of peri odontal treatment i n the nondi abeti c pati ent. However, i n thi s and i n al l
other treatment, the dental surgeon must al ways remember that the di abeti c pati ent i s more
than normal l y suscepti bl e to i nfecti on. The f requent occurrence of oral candi dosi s i n di abeti c
pati ents was di scussed i n Chapter 4. Pati ents wi th undi agnosed or poorl y control l ed di abetes
may report xerostomi a, whi ch i s due to dehydrati on, secondary to pol yuri a. Oral
hypogl ycaemi c drugs may cause l i chenoi d drug

reacti ons and, occasi onal l y, pati ents present wi th swel l i ng of the sal i vary gl ands (si al osi s).
Gl ossodyni a (burni ng mouth syndrome, BMS) may be an earl y mani festati on of undi agnosed
di abetes (Chapter 17).
The pati ent wi th undi agnosed or i nadequatel y treated di abetes may have a general i zed
stomati ti s and, i n parti cul ar, a sore tongue. Thi s i s probabl y at l east partl y due to
dehydrati on and partl y due to candi dal i nf ecti on. The possi bi l i ty of l atent di abetes shoul d be
consi dered, amongst others, i n the pati ent wi th non-speci f i c gl ossi ti s or a candi dosi s. A
fami l y hi story of di abetes i s of parti cul ar si gni fi cance. Just as i n the case of the sore tongue
due to anaemi a, a sore tongue resul ti ng from di abetes may occur earl y i n the di sease
process, before substanti al amounts of gl ucose are passed i nto the uri ne. In order to provi de
a suff i ci ent screeni ng procedure, i t i s necessary in these pati ents to carry out a bl ood
gl ucose esti mati on, pref erabl y f asti ng, rather than a si mpl e uri ne screen (see Chapter 2).
Renal disease
Renal di sease i s of great si gni f i cance i n oral medi ci ne and general dental practi ce. Oral
changes occur not onl y as a resul t of chroni c renal fai l ure but al so as a consequence of the
medi cal management of renal di sease. The dental management of pati ents wi th renal di sease
may al so need to be modi f i ed to prevent compl i cati ons.
Chronic renal failure
P. 161
Table 13.8 Oral features of diabetes mellitus
Dry mouth
Compromi sed peri odontal heal th*
Oral candi dosi s
Gl ossodyni aBMS
Li chenoi d drug reacti ons (oral hypogl ycaemi c drugs)
*Depends on oral hygi ene.
Chroni c renal fai l ure (CRF) i s i rreversi bl e deteri orati on i n renal f uncti on. When pl asma
creati ni ne persi stentl y exceeds 300 mol /l (normal range 80120 mol /l ), there i s
progressi ve deteri orati on i n renal functi on, i rrespecti ve of aeti ol ogy. When the pl asma
creati ni ne reaches 1000 mol /l , di al ysi s wi l l be requi red. The gl omerul ar fi l trati on rate
(GFT; normal , 120 ml /mi n) measures renal functi on. Progressi ve i mpai rment i n functi on of
the ki dneys l eads to the devel opment of the cl i ni cal syndrome of uraemi a.
Orofacial manifestations of chronic renal failure
Chroni c renal fai l ure and i ts resul tant uraemi a cause a number of physi ol ogi cal changes that
can cause oral symptoms (Tabl e 13. 9). In addi ti on, restri cted fl ui d i ntake, as part of the
medi cal management of thi s condi ti on, can cause oral dryness and, i n some pati ents,
i nfecti ons of the paroti d gl ands. In chroni c renal fai l ure, uraemi c stomati ti s occurs ei ther i n
the form of wi despread and pai nf ul oral ul cerati ons (ul cerati ve uraemi c stomati ti s) or as
whi te pl aques af fecti ng the oral mucosa. The l atter are pai n-free and transi ent. Once the
underl yi ng metabol i c dysf uncti on i s treated, the oral mucosa reverts back to normal . In
pati ents wi th anaemi a, pal l or of mucosa may be evi dent and purpura or haemorrhage can
occur as a resul t of pl atel et defi ci enci es. Oral candi dosi s i s frequentl y a probl em and
contri butes to oral symptoms i n pati ents wi th chroni c renal fai l ure.
Gi ant cel l l esi ons of the jaws may occur as a result of hyperparathyroi di sm secondary to
renal f ai l ure (or prol onged di al ysi s) and gi ve ri se to oral l esi ons or osteol yti c l esi ons i n the
bone. Pati ents wi th CRF are now treated wi th potent al pha-hydroxyl ated vi tami n D
suppl ements to prevent secondary hyperpathyroi di sm.
Great care must be taken when prescri bi ng drugs for pati ents wi th renal f ai l ure and the
safest drugs to use are those that are metabol i zed pri mari l y by the l i ver. Dosages of other
drugs need to be adjusted to compensate for decreased renal excreti on.
Renal patients undergoing dialysis (Table 13.10)
When pati ents reach a stage of renal fai l ure i n whi ch the ki dney functi on i s i nadequate, they
are assessed for di al ysi s. Haemodi al ysi s i s a techni que whereby the pati ent' s bl ood i s
detoxi fi ed by passi ng i t through a machi ne. Access to the pati ent' s ci rcul ati on i s achi eved by
fashi oni ng an arteri ovenous shunt, usual l y on the forearm. Pati ents undergoi ng
haemodi al ysi s are hepari ni zed and dental extracti ons are best del ayed unti l at l east 12 hours
afterwards. The opti mum ti me for dental procedures i s 1224 hours posthaemodi al ysi s.
Table 13.9 Orofacial manifestations of chronic renal
failure
Dry mouth
Mucosal ul cerati on
Bacteri al and f ungal pl aques
Pal l or of the mucosa (anaemi a)
Oral purpura
Whi te pl aques (uraemi c stomati ti s)
Gi ant cel l l esi onsosteol yti c l esi ons i n jaws
Immedi atel y after di al ysi s, pati ents al so tend to be extremel y ti red. Indwel l i ng arteri ovenous
shunts may become i nfected and many authori ti es consi der that anti bi oti c prophyl axi s i s
advi sabl e f or orodental procedures l i kel y to produce a bacteraemi a i n di al ysed pati ents. As
for those wi th chroni c renal fai l ure, care must be taken when prescri bi ng drugs f or these
pati ents. Haemodi al ysed pati ents have been reported as havi ng an i ncreased carri age rate of
hepati ti s B and C. However, fol l owi ng fatal outbreaks of hepati ti s B amongst staff and
pati ents i n renal uni ts, di al ysed pati ents are regul arl y screened for hepati ti s B. Uni versal
precauti ons for cross-i nfecti on control shoul d, however, be i nsti tuted for al l dental pati ents,
regardl ess of thei r medi cal or ri sk status.

The opti mum ti me f or renal pati ents to undergo dental procedures i s 1224 hours
posthaemodi al ysi s.
An al ternati ve form of di al ysi s i s CAPD (conti nuous ambul atory peri toneal di al ysi s), whi ch
does not requi re hospi tal i zati on. Indi vi dual s on CAPD shoul d be treated as haemodi al ysed
pati ents but do not have the probl ems associ ated with arteri ovenous shunts and
heparani zati on. CAPD pati ents are, however, at ri sk from peri toni ti s.
Renal transplant patients
Renal transpl antati on, ei ther f rom a ti ssue-matched cadaver or cl ose rel ati ve, off ers the best
hope of a normal l i fe. However, i t i s not wi thout probl ems, mai nl y due to the si de-eff ects of
i mmunosuppressi ve regi mens gi ven to prevent rejection of the transpl anted ki dney (Tabl e
13. 11). In the past l arge doses of corti costeroi ds gave ri se to consi derabl e short- and l ong-
term probl ems (see Chapter 3). Renal transpl ant pati ents pose a number of dental and
medi cal management probl ems.
Table 13.10 Dental management of haemodialysed
patients: some important considerations
Impai red excreti on of drugs by ki dney
Bl eedi ng tendency
Hepari ni zati on pri or to di al ysi s
Arteri ovenous shunts suscepti bl e to i nfecti on
Anaemi a
Increased carri age of hepati ti s B and C
Hypertensi on
P. 162
Table 13.11 Oral complications of renal transplantation
Drug-i nduced gi ngi val overgrowth (hyperpl asi a)
Ci cl ospori n A i s wi del y used i n al l branches of transpl ant surgery yet, paradoxi cal l y, thi s i s a
nephrotoxi c drug. Gi ngi val overgrowth i s a wel l -recogni zed compl i cati on of ci cl ospori n (Fi g.
13. 5), but can al so occur wi th cal ci um channel bl ockers, such as ni fedi pi ne, whi ch may be
prescri bed f or concomi tant hypertensi on i n renal pati ents (see Chapter 14). Recent studi es
have shown that pretranspl ant gi ngi val hyperpl asi a appears to be potenti ated by ci cl ospori n
therapy, resul ti ng i n severe posttranspl ant overgrowth.
Immunosuppressi ves, gi ven to prevent ti ssue rejection, may cause a number of oral
compl i cati ons i ncl udi ng the devel opment of bacteri al (or candi dal ) pl aques, wi despread oral
candi dosi s, herpes si mpl ex vi rus i nfecti ons (secondary), suscepti bi l i ty to dental i nf ecti ons,
and, l ess commonl y, hai ry l eukopl aki a. Lesi ons presenti ng as superfi ci al pl aques on
the oral mucosa may resembl e acute pseudomembranous candi dosi s (thrush) and can be
wi ped of f when fi rml y scraped. However, i t i s now recogni zed that the i nfecti ve organi sm
may be bacteri al (for exampl e, staphyl ococci , streptococci , col i f orms) and wi l l not respond to
anti -fungal therapy. These pl aques grow i n vi rtual ly pure cul ture and i n adherent pl aque-l i ke
l esi ons. Treatment of these l esi ons depends on cul ture and sensi ti vi ty of putati ve bacteri a.
There i s al so a reported i ncreased i nci dence of oral carci noma i n renal pati ents taki ng
i mmunosuppressi ve drugs. Carci noma ari si ng i n ci cl ospori n-i nduced gi ngi val overgrowth has
al so been reported.
Bacteri al and f ungal pl aques
Increased i nci dence of oral mal i gnancy (reported)
Oral candi dosi s
Herpes si mpl ex i nfecti ons (secondary)
Fig. 13.5 Gi ngi val hyperpl asi a i nduced by ci cl ospori n i n a renal transpl ant pati ent.
Controversy sti l l exi sts as to whether renal transpl ant pati ents shoul d be gi ven anti bi oti c
cover f or orodental procedures l i kel y to generate a bacteraemi a. It i s essenti al that the
denti st l i ai ses wi th the pati ent' s renal transpl ant team, many of whom advi se prophyl acti c
anti bi oti c cover. Anti mi crobi al s and other drugs that are potenti al l y nephrotoxi c to the
transpl anted ki dney shoul d be avoi ded. Every attempt must be made to keep these pati ents
dental l y f i t and free f rom i nfecti ons.
The most l i kel y di agnosi s i n thi s l ady' s case i s an acute form of l eukaemi a. If she presented
on medi cati on, i t woul d be i mportant to consi der a drug-i nduced agranul ocytosi s. Pal l or and
fati gue are both mani festati ons of i ron defi ci ency and a hi story of suscepti bi l i ty to recurrent
i nfecti ons woul d be consi stent wi th l eukaemi a. A secondary pl atel et def i ci ency
(thrombocytopeni a) wi l l predi spose to spontaneous brui si ng of the ski n and bl eedi ng from the
gi ngi vae. Spontaneous gi ngi val haemorrhage, as reported by thi s l ady, i s unusual even i n
those pati ents wi th poor oral hygi ene and rei nf orces the cl i ni cal suspi ci on of l eukaemi a.
Other oral si gns to check for i ncl ude: oral purpura and petechi ae, gi ngi val swel l i ng or
i nfecti on, oral ul cerati on, mucosal swel l i ng, and evi dence of opportuni sti c i nf ecti ons, such as
candi dosi s (Tabl e 13. 4). There may al so be cervi cal l ymphadenopathy, ei ther as a resul t of a
l ymphadeni ti s secondary to oral i nfecti on or (l ess commonl y) l eukaemi c i nf i l trati on.
Thi s pati ent must be i mmedi atel y referred for ful l bl ood count. If thi s i s consi stent wi th your
cl i ni cal di agnosi s of l eukaemi a, then the pati ent must be referred to a haematol ogi st for
further

i nvesti gati on and appropri ate treatment. As an i nteri m measure thi s pati ent shoul d be gi ven
appropri ate therapy for her oral condi ti on. Thi s l ady has reported soreness of the mouth,
whi ch may be hel ped by usi ng an anal gesi c or anti septi c mouthwash, parti cul arl y i f pl aque
control i s poor. Candi dal i nf ecti on shoul d be treated by appropri ate topi cal anti f ungal
therapy. Systemi c anti bi oti cs are probabl y best avoi ded at thi s stage as these mi ght
compl i cate thi s l ady' s medi cal management. The haematol ogi st shoul d, however, be i nformed
i f there i s evi dence of orodental i nfecti on that may need treati ng bef ore def i ni ti ve therapy for
the l eukaemi a i s commenced. If any extracti ons are needed, these must be carri ed out i n a
hospi tal envi ronment because of potenti al l y seri ous compl i cati ons, such as excessi ve
postextracti on bl eedi ng.
Arrangements shoul d be made for thi s pati ent to have hi s extracti on carri ed out at l east 12
hours posthaemodi al ysi s, when potenti al bl eedi ng probl ems associ ated wi th hepari ni zati on
are l i kel y to be l ess (the hal f -l i f e of hepari n i s 46 hours). A recent ful l bl ood count shoul d
be checked to excl ude thrombocytopeni a. If there i s no establ i shed l ocal protocol f or
preoperati ve anti bi oti c prophyl axi s i n haemodi al ysed pati ents, then the pati ent' s renal uni t
shoul d be contacted for advi ce. Routi ne l ocal anaesthesi a shoul d be empl oyed for thi s
pati ent. After the extracti on i t i s advi sabl e to suture the socket and/or i nsert a haemostati c
gauze and check that there i s no postoperati ve bl eedi ng. Thi s preventi ve measure i s
i mportant as the pati ent may have a tendency to bl eed af ter the extracti on. The pati ent
Q1 What di agnosi s must be excl uded i n thi s l ady' s case? What other oral mani festati ons
of thi s condi ti on mi ght you fi nd on exami nati on?
P. 163
Q1 How woul d you manage the dental extracti on of thi s medi cal l y compromi sed pati ent?
shoul d be revi ewed and arrangements made f or regul ar dental care.
These transi ent whi te pl aques are l i kel y to represent uraemi c stomati ti s and requi re no
treatment. If the pl aques rub off they may be due to oral candi dosi s, i n whi ch case the
pati ent requi res a systemi c anti fungal agent (f or exampl e, fl uconazol e) or a bacteri al
i nfecti on. Cul ture and sensi ti vi ty of putati ve mi croorgani sms wi l l i ndi cate the therapy of
choi ce.
Project
What drugs shoul d be avoi ded f or pati ents undergoi ng haemodi al ysi s?
Q2 What i s the most l i kel y di agnosi s of the whi te plaques on the pati ent' s oral mucosa?
> Tabl e of Cont ent s > 14 - Immunodefi ci ency, hyper sensi ti vi t y, aut oimmuni t y, and or al r eacti ons t o drug
therapy
14
Immunodeficiency, hypersensitivity,
autoimmunity, and oral reactions to drug therapy
Problem cases
Case 14.1
A 30-year-ol d former nurse attends for routi ne dental care and reports that she has recentl y
been di agnosed by an al l ergi st as havi ng natural rubber l atex (NRL) al l ergy. As a resul t of
thi s she i s unabl e to conti nue nursi ng and i s now empl oyed i n an admi ni strati ve post. Thi s
l ady al so has a hi story of hay f ever and chi l dhood eczema. She i s al l ergi c to ki wi f rui t and
bananas.
Case 14.2
A 55-year-ol d l ady, wi th a hi story of stabl e angi na attends f or routi ne dental care. She i s
concernedone of her f ri ends has read i n a magazi ne arti cl e that medi cati on used to
control angi na can cause probl ems i n the mouth and may affect the teeth.
Immunodeficiency
Immunodef i ci enci es may be cl assi fi ed as pri mary or secondary. Pri mary i mmunodefi ci enci es
represent an i ntri nsi c fai l ure i n devel opment of one or more parts of the i mmune system and
are often geneti cal l y based. They can be predomi nantl y B- or T-cel l defects, a combi nati on of
both, or due to a sel ecti ve i mmunodefi ci ency (for exampl e, IgA def i ci ency). A few i ndi vi dual s
are born wi th gross pri mary i mmunodef i ci enci es. These may resul t i n l i fe-threateni ng
condi ti ons and the af fected i ndi vi dual s tend to di e young, often as a resul t of recurrent
i nfecti ons. Candi da i nfecti ons are often predomi nant i n these pati ents (see Chapter 4) who
suff er from aphthous-l i ke ul cerati on and herpes i nfecti ons. They are al so more suscepti bl e to
peri odontal di sease.
IgA defi ci ency i s rel ati vel y more common than other pri mary i mmunodefi ci enci esl evel s of
IgA are decreased i n both serum and sal i va.
Secondary i mmunodef i ci enci es are the resul t of the modi fi cati on and suppressi on of some
part of the i mmune def ence mechani sm as a resul t of an extri nsi c i nfl uence. Thi s may be the
Q1 What i s the si gni f i cance of thi s l ady' s atopi c history and her previ ous occupati on?
Q2 Why i s the reported al l ergy to f rui t of rel evance?
Q3 What speci al precauti ons need to be taken when provi di ng dental treatment f or thi s
l ady?
Q1 What i nformati on do you need to answer thi s l ady's enqui ry?
resul t of some di sease process. Endocri ne di seases such as di abetes mel l i tus and
i mmunosuppressi ve drug regi mens (for exampl e, ci cl ospori n therapy) are commonl y i nvol ved
(see Tabl e 14. 1).
Secondary i mmune suppressi on may occur i n mal i gnancy (parti cul arl y i n l eukaemi as) and i n
pati ents sufferi ng from mal nutri ti on, as i n cancrum ori s. In the l atter, i t i s suggested that an
extensi on of acute ul cerati ve gi ngi vi ti s i n suscepti bl e pati ents may occur fol l owi ng a vi ral
i nfecti on, usual l y measl es (see Chapter 4). Iatrogeni c causes of secondary i mmune
suppressi on are most commonl y associ ated wi th i mmunosuppressi ve drug regi mes,
parti cul arl y i n the preventi on of rejecti on i n transpl ant pati ents. Gross oral i nf ecti ons (often
by Candi da, but al so by other organi sms) often fol l owed the massi ve doses of steroi ds used
unti l recentl y i n the management of rejecti on. However, wi th the i ntroducti on of more
sel ecti ve drug regi mens, thi s has become somewhat l ess of a probl em. Local
i mmunosuppressi ve therapy may al so produce more restri cted effects. For exampl e, the use
of i nhal ed steroi d preparati ons for the treatment of asthma or the use of steroi d-contai ni ng
mouthwashes occasi onal l y resul t i n oral or oropharyngeal candi dosi s.
Vi ral i nf ecti ons may resul t i n secondary i mmune defi ci enci es of varyi ng degrees of
si gni f i cance. The domi nati ng i nterest i n thi s

fi el d, at the present ti me, i s i n the prof ound i mmunosuppressi on that may accompany HIV
i nfecti on, associ ated wi th the acqui red i mmune def ici ency syndrome (AIDS). The oral
mani festati ons of AIDS are di scussed i n Chapter 4.
Hypersensitivity
In contrast to the di seases caused by i mmune defi ci enci es, there are others that depend on
an overacti ve or exaggerated response of some aspect of the i mmune system. The condi ti ons
known as hypersensi ti vi ty reacti ons are of thi s type, and they depend on an enhanced
response of ei ther the humoral or the cel l -medi ated mechani sms, consequent upon a second
P. 168
Table 14.1 Examples of secondary immunodeficiencies
Disorder Example
Endocri ne di sorders Di abetes mel l i tus
Mal i gnancy Acute l eukaemi a
Vi ral i nf ecti on HIV i nfecti on
Bacteri al i nfecti on Tubercul osi s
Autoi mmune di sease Rheumatoi d arthri ti s
Nutri ti onal def i ci enci es Mal nutri ti on
Drug-i nduced (i atrogeni c) Systemi c corti costeroi d therapy
contact wi th an anti gen to whi ch the host has previousl y been sensi ti zed. These reacti ons
i ncl ude some i n whi ch a severe toxi c ef fect may be produced i n the host. The term
al l ergy i s now used to descri be adverse reacti ons that resul t f rom a pati ent bei ng
i mmunol ogi cal l y hypersensi ti ve to an exogenous agent, that i s, an al l ergen.
Hypersensi ti vi ty reacti ons were cl assi cal l y def i ned i nto four groups by Coombs and Gel (Tabl e
14. 2). It i s, however, conveni ent to consi der two mai n types: i mmedi ate and del ayed
reacti ons. Type I hypersensi ti vi ty reacti ons are i mmedi ate and medi ated by IgE anti bodi es.
Cl i ni cal mani festati ons of thi s type of reacti on range from systemi c anaphyl axi s to
angi oedema, asthma, al l ergi c rhi ni ti s, and general i zed or l ocal i zed urti cari a. Type IV
reacti ons are del ayed hypersensi ti vi ty reacti ons and medi ated by sensi ti zed T l ymphocytes.
The cl i ni cal mani f estati ons usual l y occur after 48 hours, and exampl es i ncl ude contact
dermati ti s due to ni ckel al l ergy and organ transpl ant rejecti on.
The si gni f i cance of these hypersensi ti vi ty reacti ons i n oral medi ci ne i s that the oral mucosa
may be di rectl y i nvol ved or the oral ti ssues af fected as part of a general i zed reacti on. An
al l ergi c angi oedema (type I reacti on) frequentl y presents as swel l i ng of the l i ps (see bel ow).
A l ocal i zed contact stomati ti s (type IV reacti on) can occur on the oral mucosa as a response
to a wi de range of al l ergens, i ncl udi ng toothpastes, topi cal anaestheti cs, and resi ns i n
composi te materi al s.
There are a number of condi ti ons encountered i n oral medi ci ne i n whi ch al l ergy has
been i mpl i cated but convi nci ng evi dence for these associ ati ons has yet to be produced. These
i ncl ude recurrent aphthous stomati ti s (RAS), orofaci al granul omatosi s (OFG), erythema
mul ti forme, l i chen pl anus, and pl asma cel l gi ngi vi ti s.
Angioedema
Angi oedema (soft-ti ssue swel l i ng) can be al l ergi c or non-al l ergi c i n aeti ol ogy and may occur
wi th or wi thout urti cari a (rai sed wheal s or pl aques on the ski n, as i n nettl e rash and
of ten ref erred to as hi ves). Most cases of angi oedema (and urti cari a) are non-al l ergi c
and i di opathi c.
Acute angi oedema can, however, occur as an al l ergi c response to a wi de range of sti mul ae,
i ncl udi ng f ood stuffs (for exampl e, peanuts), drugs (f or exampl e, peni ci l l i n), i nsect bi tes (for
exampl e, wasp sti ngs), and natural rubber l atex. Attacks of acute angi oedema can resul t i n
wi despread and often dangerous sof t-ti ssue swel l i ng that may aff ect the f aci al , oral , and
pharyngeal regi ons and compromi se the ai rway. Urti cari a can accompany soft-ti ssue swel l i ng.
Table 14.2 Hypersensitivity reactions
Type Description
I Anaphyl acti c type
II Cytotoxi c reacti ons
III Serum si ckness
IV Del ayed hypersensi ti vi ty
These mani festati ons are the resul t of a type I hypersensi ti vi ty reacti on that i s IgE-
medi ated. Degranul ati on of mast cel l s l eads to the l i berati on of hi stami ne and hi stami ne-
l i ke substances that cause an i ncrease i n vascul ar permeabi l i ty and resul t i n sof t-ti ssue
swel l i ngs. Respi ratory occl usi on i s l i f e-threateni ng and pati ents must be gi ven adrenal i ne
(epi nephri ne), suppl emented by steroi ds and anti hi stami nes as appropri ate (see Chapter 19,
Medi cal emergenci es i n denti stry).
Al l ergi c angi oedema affecti ng the face and neck can be l i fe-threateni ng.
Some pati ents present wi th angi oedema (wi th or wi thout urti cari a) that i s non-al l ergi c and
i ntermi ttent i n nature. Angi oedema i s a wel l documented adverse si de-eff ect of angi otensi n-
converti ng enzyme (ACE) i nhi bi tors and can occur for the f i rst ti me even after prol onged
treatment. Symptomati c epi sodes of angi oedema may al so fol l ow the i ngesti on of a
nonsteroi dal anti -i nfl ammatory drug (NSAID), such as i buprofen or aspi ri n. Pati ents wi th a
hi story suggesti ve of non-al l ergi c angi oedema shoul d be checked f or C1 esterase i nhi bi tor
def i ci ency (see bel ow). Oral anti hi stami nes are the mai nstay of treatment for non-al l ergi c
angi oedema (and urti cari a). Rel ati vel y non-sedati ng preparati ons are now avai l abl e.
Angi oedema i s a wel l documented adverse, si de-eff ect of ACE i nhi bi tors and can occur for
the fi rst ti me even after prol onged treatment.
C1 esterase inhibitor deficiency
C1 esterase i nhi bi tor defi ci ency can be heredi tary or acqui red and presents as angi oedema.
Heredi tary angi oedema i s a rare autosomal domi nant geneti c di sease. As a resul t of C1
esterase i nhi bi tor defi ci ency, the acti on of the compl ement system i s uncontrol l ed and
acti vati on of ki ni ns l eads to i ncreased capi l l ary permeabi l i ty. In i ts f ul l y devel oped form i t i s
a seri ous and l i fe-threateni ng condi ti on that may be preci pi tated by mi ni mal l ocal trauma,
such as dental treatment. C1 esterase i nhi bi tor and compl ement components C3 and C4
l evel s can be

measured. Prophyl axi s and management of heredi tary angi oedema i s by use of a syntheti c
androgen (for exampl e, stanazol e) or, i n an emergency, f resh frozen pl asma.
C1 esterase i nhi bi tor def i ci ency can be heredi tary or acqui red and presents as angi oedema.
Autoimmunity
In an autoi mmune reacti on the i mmune response i s directed agai nst the host' s own ti ssues,
whi ch, f or some reason, have become anti geni cal l y acti ve. Both the cel l -medi ated and the
humoral responses may be i nvol ved i n the process. It i s not known how the faci l i ty to
recogni ze sel f i s destroyed, and many di fferent theori es have been proposed.
A number of di seases of accepted autoi mmune ori gi n affect the oral ti ssues secondari l y.
Thus, i n perni ci ous anaemi a (Chapter 13), al though the pri mary autoi mmune process di rectl y
aff ects the gastri c pari etal cel l s, the haematol ogical changes i nduced by the resul ti ng
i nabi l i ty to absorb vi tami n B
12
may cause marked abnormal i ti es i n the oral mucosa. These
are the resul t of i nstabi l i ty of the epi thel i um i nduced by the def i ci ency. Apart from these and
si mi l ar secondary eff ects, however, a number of di seases of establ i shed or suspected
autoi mmune ori gi n aff ect the oral ti ssues di rectl y, produci ng oral l esi ons as a pri mary
symptom. These i ncl ude: i mmunobul l ous ski n di seases (Chapter 11) and Sjgren' s
syndrome (Chapter 8). The detecti on of autoanti bodies i n pati ents wi th suspected
autoi mmune di sease i s di scussed i n Chapter 2.
P. 169
Oral reactions to drug therapy
Spectrum of adverse reactions
There i s a l arge number of drugs that can cause adverse effects on the oral mucosa and
these i ncl ude oral ul cerati on (Chapter 5) and l i chenoi d drug erupti ons (Chapter 11).
Local i zed reacti ons to topi cal therapy, i ncl udi ng i rri tant substances, can al so occur (Tabl e
14. 3) and the oral ti ssues may be affected as a mani festati on of a systemi cal l y i nduced drug
reacti on (Tabl e 14. 4). One of the common adverse reacti ons of the oral mucosa to drugs i s
that of chemi cal trauma fol l owi ng contact wi th aspiri n or an aspi ri n-contai ni ng tabl et of some
ki nd. Pati ents frequentl y use thi s form of therapy for the rel i ef of toothache and the
associ ati on of a cari ous tooth causi ng toothache and an acutel y presenti ng whi te patch of the
adjoi ni ng buccal mucosa shoul d bri ng thi s possi bi l ity strongl y to mi nd (Fi g. 14. 1). The
appearance of the aff ected area may be qui te spectacul ar and onl y the hi story wi l l provi de
the di agnosi s. The condi ti on i s, qui te evi dentl y, sel f -l i mi ti ng and requi res no treatment
except for the toothache. Xerogeni c medi cati on i s di scussed f ul l y i n Chapter 8 and drugs
causi ng di scol ourati on of the oral mucosa are di scussed i n Chapter 9. Oral reacti ons to
anti bi oti cs and steroi ds are di scussed i n thi s chapter, together wi th drug-i nduced gi ngi val
overgrowth and fi xed drug erupti ons.
Table 14.3 Topical therapy: localized oral reactions
Therapy Example
Aspi ri n
Toothache sol uti ons Oi l of cl oves
Irri tant chemi cal s Hydrogen peroxi de used as root canal i rri gant
Topi cal anti bi oti cs Chl ortetracycl i ne
Topi cal steroi ds Betamethasone ri nse
Table 14.4 Systemically induced drug reactions with oral
manifestations
Drug reaction Example of drug
Li chenoi d erupti ons NSAIDs
Erythema mul ti forme (StevensJohnson syndrome) Sul fonami des
Oral reactions to antibiotics
It i s wel l known that hypersensi ti vi ty reacti ons may occur duri ng anti bi oti c therapy. These
are commonl y general i zed reacti ons i nvol vi ng the whol e metabol i sm. Thei r severi ty may vary
from a mi l d and transi ent rash to the extremel y severe reacti on of angi oneuroti c oedema, i n
whi ch oedema and swel l i ng of the ti ssues of the head and neck may extend to the tongue and
l arynx and resul t i n dangerous respi ratory obstructi on. Such a condi ti on represents an
extreme medi cal emergency and must be treated as such.
Hypersensi ti vi ty reacti ons may occur occasi onal l y fol l owi ng the repeated use of tetracycl i ne
mouthwashes. The reacti on may occur

ei ther earl y i n the i ni ti al course or after many treatments. The reacti on of ten takes the f orm
Lupus erythematosus Hydral l azi ne
Type I hypersensi ti vi ty reacti ons Peni ci l l i ns
Fi xed drug erupti ons Sal i cyl ates
Drug-i nduced bone marrow suppressi on Methotrexate
Immunosuppressi ve drugs Ci cl ospori n
Sal i vary gl and hypof uncti on Tri cyl i c anti depressants
Fig. 14.1 Aspi ri n burn of the buccal mucosa.
P. 170
of a l ocal i zed angi oneuroti c oedema wi th swel l i ng of the eyel i ds and the faci al ti ssues i n
general . If thi s reacti on occurs, i t i ndi cates that the pati ent has acqui red a hypersensi ti vi ty
to the tetracycl i ne and must be warned that future use of the drug may be dangerous. The
possi bi l i ty of thi s type of reacti on i s yet another argument agai nst the i ndi scri mi nate use of
anti bi oti c therapy. Occasi onal l y, a l ocal i zed form of hypersensi ti vi ty reacti on may be seen i n
the oral ti ssues f ol l owi ng tetracycl i ne mouthwash therapy. Thi s i s rel ati vel y l i mi ted i n nature
and l eads to mul ti pl e vesi cl e formati on. Agai n, the appearance of these symptoms shoul d
l ead to the i mmedi ate cessati on of the use of the anti bi oti c, together wi th warni ngs as to
further use. The i nci dence of hypersensi ti vi ty reacti ons due to the use of tetracycl i ne
mouthwashes i s, however, remarkabl y l ow. Treatment of hypersensi ti vi ty reacti ons depends
on the severi ty and acuteness of the symptoms. In a mi l d reacti on i t may onl y be necessary
to di sconti nue the use of the drug and to observe the pati ent careful l y. In other cases, the
use of anti hi stami nes al one wi l l be suffi ci ent to suppress the symptoms. In a f ul l y devel oped
angi oneuroti c oedema, i mmedi ate treatment wi th i ntramuscul ar adrenal i ne (epi nephri ne)
of ten combi ned wi th hydrocorti sone may be necessary (see Chapter 19).
Apart from these mani f estati ons of hypersensi ti vi ty, there are a number of mi nor reacti ons to
anti bi oti cs that may be l ocal i zed to the mouth. Bl ack hai ry tongue occasi onal l y fol l ows
treatment wi th ei ther wi de- or narrow-spectrum antibi oti cs. As the essenti al f eature of bl ack
hai ry tongue i s el ongati on of the fi l i f orm papi l l ae and thi s i s associ ated onl y wi th secondary
bacteri al changes, i t i s di f fi cul t to understand why anti bi oti c therapy shoul d i nduce thi s
parti cul ar form of reacti on. Fol l owi ng the cessati on of the therapy the tongue may return to
normal ei ther qui ckl y or, occasi onal l y, very sl owl y. Thi s form of reacti on may occur af ter
ei ther systemi c or l ocal anti bi oti c therapy, al though most cases of bl ack hai ry tongue are of
unknown aeti ol ogy.
It mi ght wel l be thought that the use of anti bi oti c mouthwashes to treat oral l esi ons woul d
l ead to symptoms ari si ng f rom the wi despread l ocal overgrowth of resi stant organi sms and,
i n parti cul ar, Candi da spp, but, i n fact, thi s i s not often so. Preci sel y as general physi ci ans
have found i t unnecessary to i ncorporate anti f ungal anti bi oti cs wi th wi de-spectrum
anti bi oti cs i n order to avoi d gastroi ntesti nal i nfestati ons by yeasts, so i t has been found
unnecessary to combi ne anti fungal anti bi oti cs wi th wi de-spectrum anti bacteri al s i n
mouthwash therapy for oral ul cerati on. Thi s does not mean, however, that such an
overgrowth i s not possi bl e and i t must al ways be remembered that any l ocal i zed use of an
anti bi oti c may l eave behi nd resi stant strai ns of organi sms, even i f no cl i ni cal overgrowth
occurs. It i s, therefore, worth rei terati ng that the use of anti bi oti c mouthwashes shoul d be
reserved for si tuati ons i n whi ch there are posi ti ve i ndi cati ons.
Oral reactions to steroids
There are now many pati ents taki ng systemi c steroi ds on a l ong-term basi s, and often thi s
treatment resul ts i n suscepti bi l i ty to i nfecti on and general l oss of ti ssue resi stance. In the
mouth, thi s takes the form of acute erythematous candi dosi s i n most cases, al though acute
or chroni c pseudomembranous candi dosi s may al so occur. Al though the pal ate i s most of ten
aff ected, the whol e of the oral and pharyngeal mucosa may become i nvol ved, as may the
mucosa of the l arynx. Secondary i nfecti on by Candi da al bi cans i s al most i nvari abl y present,
and treatment of these pati ents by l ocal anti fungal therapy i s of ten sati sfactory i n reduci ng
symptoms. In severe cases a systemi c anti fungal agent may be requi red. Thi s, however, can
be no more than a temporary sol uti on si nce these pati ents are, i n general , desti ned to
mai ntai n steroi d therapy for an i ndef i ni te peri od. It may, therefore, be necessary to
repeatedl y treat thi s candi dal i nf ecti on. In these persi stent cases of candi dal i nf ecti on the
l ong-term use of a topi cal anti f ungal preparati on i s requi red.
Steroi d mouthwashes and other topi cal preparati ons are advocated f or a number of
condi ti ons i n oral medi ci ne and may obvi ate the need f or systemi c steroi ds. Thi s f orm of
therapy mi ght be expected to be a prol i fi c source of candi dosi s but, i n f act, thi s compl i cati on
i s rel ati vel y unusual . If i t does occur, l ong-term anti fungal measures must accompany the
anti bi oti csteroi d therapy. Mi conazol e gel i s often very hel pful i n these ci rcumstances,
parti cul arl y i f the pati ent has a sore mouth and cannot tol erate nystati n pasti l l es or
amphoteri ci n l ozenges. Pati ents who regul arl y use a steroi d i nhal er f or respi ratory di sease,
such as asthma, are predi sposed to devel opi ng oral candi dosi s, parti cul arl y of the pal ate and
oropharynx. They shoul d al ways ri nse thei r mouth after use of the steroi d and, i n some
cases, a spacer devi ce may be i ndi cated (Fi g. 14. 2).
Drug therapy and the periodontal tissues
Drug-i nduced gi ngi val hyperpl asi a (overgrowth) i s a wel l recogni zed compl i cati on and the
drugs most commonl y i mpl i cated are phenytoi n, ci cl ospori n, and cal ci um-channel bl ockers
(ni fedi pi ne, verapami l , di l ti azem). Oral contracepti ves have been associ ated wi th a
hyperpl asti c oedematous gi ngi vi ti s but i t i s l i kel y that thi s response i s a secondary reacti on
to pl aque and i s therefore dependent on the standard of pl aque control .

Gingival overgrowth and sodium phenytoin (Epanutin)
Sodi um phenytoi n i s the most commonl y used drug f or the treatment of epi l epsy and, i n
approxi matel y 50 per cent of the pati ents taki ng i t, there i s a marked chroni c hyperpl asti c
gi ngi vi ti s. The hyperpl asti c reacti on i s typi cal l y papi l l ary and the i nterdental papi l l ae become
swol l en, someti mes grossl y so. Thi s i s essenti al l y a fi brobl asti c reacti on and the ti ssue i s
general l y fi rm and much l ess haemorrhagi c than i n the case of pregnancy gi ngi vi ti s. In vi ew
of the marked fal se pocketi ng that may occur, there are great di ff i cul ti es i n mai ntai ni ng oral
hygi ene and secondary i nfl ammatory changes are al most i nvari abl y present. The nature of
the reacti on to the drug i s not cl ear si nce there is evi dence that the hyperpl asi a represents
an exaggerated f orm of chroni c gi ngi vi ti s, whi ch does not occur i f i mmacul ate standards of
oral hygi ene are mai ntai ned. Al though wi thdrawal of the drug i s, i n i tsel f , suf fi ci ent to hal t
the progress of the condi ti on, the di ffi cul ti es of stabi l i zati on i n an epi l epti c pati ent may make
i t i mpracti cal to suggest i ts wi thdrawal as a treatment for the oral condi ti on. Stri ngent oral
hygi ene and regul ar scal i ng, preceded, i f need be, by gi ngi vectomy, must be carri ed out. In
severe cases, the gi ngi vectomy shoul d be performed under hospi tal condi ti ons. Al though the
prol i ferati on i s essenti al l y fi brobl asti c, the secondary i nfecti ve processes often resul t i n the
producti on of very vascul ar ti ssue, and bl ood l oss may be consi derabl e i f surgery i s
undertaken duri ng thi s phase. Hi stol ogi cal l y, none of the drug-i nduced gi ngi val changes show
Fig. 14.2 Steroi d i nhal er wi th a spacer devi ce.
P. 171
true hyperpl asi a, but there i s an i ncrease i n col l agen and ground substance.
Gingival overgrowth and ciclosporin
Ci cl ospori n i s an i mmunosuppressi ve drug that i s now wi del y prescri bed i n al l branches of
transpl ant surgery and i ncreasi ngl y used f or systemi c di seases such as rheumatoi d arthri ti s.
Ci cl ospori n-i nduced gi ngi val overgrowth (Fi g. 13. 5) i n renal pati ents was di scussed i n
Chapter 13.
Gingival overgrowth and calcium-channel blockers
These drugs are used extensi vel y for the management of cardi ovascul ar di sorders, i ncl udi ng
angi na, hypertensi on, and cardi ac arrhythmi as. Ni fedepi ne, one of the di hydropyri di nes, has
been reported as causi ng gi ngi val overgrowth i n 10 to 15 per cent of dentate pati ents. Renal
pati ents may be taki ng ci cl ospori n to prevent rejecti on of thei r transpl anted ki dney and a
cal ci um-channel bl ocker to control hypertensi on.
Drugs commonly implicated in drug-induced gingival overgrowth
Phenytoi n
Ci cl ospori n
Cal ci um-channel bl ocki ng drugs
Fixed drug eruptions
The i mmunol ogi cal mechani sm i nvol ved i n fi xed drug erupti ons has yet to be el uci dated but i t
woul d appear that these represent a type of del ayed hypersensi ti vi ty reacti on. The oral
mucosa i s rarel y affected and most l esi ons occur on the ski n. Fi xed drug reacti ons occur at
the same si te each ti me the preci pi tati ng drug i s admi ni stered. Oral l esi ons can occur on the
pal ate, l i ps, or tongue and they frequentl y start as vesi cl es or bul l ae, whi ch rapi dl y break
down to form ul cers. Drugs commonl y i mpl i cated i n fi xed drug erupti ons i ncl ude: sal i cyl ates,
dapsone, tetracycl i nes, and sul fonami des. Barbi turates have been reported as causi ng oral
l esi ons i n the absence of ski n i nvol vement.
Atopy (that i s, a hi story of asthma, eczema, hay f ever) i s a ri sk factor f or devel opi ng NRL
al l ergy. Other pati ents i denti fi ed as at ri sk i ncl ude those wi th l ong-term exposure to
NRL-contai ni ng i tems, that i s, heal th-care workers (l atex gl oves) and pati ents wi th spi na
bi f i da (uri nary catheters).
There i s a reported cross-sensi ti vi ty between NRL and certai n f rui ts (f or exampl e, bananas,
ki wi frui ts, avocados).
Thi s l ady needs to be treated i n a l atex-control l ed, dental envi ronment. Non-NRL gl oves must
be worn by al l staff di rectl y i nvol ved i n her care and i tems such as NRL dams and pol i shi ng
cups must not be used. Non-NRL dental and medi cal equi pment can be substi tuted. Local
Q1 What i s the si gni fi cance of thi s l ady' s atopi c history and her previ ous occupati on?
Q2 Why i s the reported al l ergy to frui t of rel evance?
Q3 What speci al precauti ons need to be taken when provi di ng dental treatment f or thi s
l ady?
anaestheti c cartri dges wi th syntheti c (non-NRL) bungs are avai l abl e to avoi d the potenti al
ri sk of contami nati on of the l ocal anaestheti c sol uti on. Emergency equi pment wi th non-NRL
must al so be avai l abl e (see l ocal and nati onal gui del i nes for f urther advi ce).
You wi l l obvi ousl y requi re a l i st of thi s l ady' s current medi cati on, whi ch can then be checked
agai nst a nati onal drug formul ary and manufacturers drug data sheets. Both l i st potenti al
si de-eff ects of the drugs.
Stabl e angi na usual l y resul ts from atheroscl eroti c pl aques i n the coronary arteri es. Most
angi na pati ents wi l l have a ni trate (gl yceryl tri ni trate (GTN) or i sosorbi de ni trate) for use
duri ng an acute attack, ei ther as a tabl et or a spray for subl i ngual use. GTN

i s al so avai l abl e as a fast-rel ease tabl et that i s pl aced between the upper l i p and l abi al
mucosa and l eft to di ssol ve. Many pati ents wi th angi na may requi re regul ar drug therapy and
wi l l have been prescri bed prophyl acti c aspi ri n. Mai ntenance drugs for the treatment of angi na
i ncl ude beta-bl ockers, cal ci um-channel bl ockers (for exampl e, ni fedi pi ne), and ni corandi l (a
potassi um-channel bl ocker).
The potenti al oral (dental ) si de-eff ects of these drugs i ncl ude the fol l owi ng.
Gl yceryl tri ni traterapi d rel ease buccal preparati on that contai ns l actose; thi s
causes a pronounced drop i n pH, whi ch predi sposes to dental cari es.
Beta bl ockersxerostomi a (sal i va f l ow reducedsee Chapter 8).
Cal ci um-channel bl ockers (ni f edepi ne)gi ngi val overgrowth.
Ni corandi l oral ul cerati on.
Your pati ent shoul d be reassured that the potenti al oral and dental si de-eff ects of medi cati on
for angi na are mi nor and can be managed. It i s i mportant that thi s l ady adheres to medi cal
advi ce concerni ng her medi cati on and the correct dosage. Most pati ents sufferi ng from
angi na recei ve dental treatment i n general practi ce but i t i s essenti al that appropri ate drugs
and equi pment are avai l abl e i f they devel op an angina attack. It i s al so i mportant that staf f
are adequatel y trai ned to deal wi th al l medi cal emergenci es (see Chapter 19).
Projects
Denti sts and doctors are requi red to report any adverse reacti ons that they suspect are due
to drugs that the pati ent has been prescri bed.
1. What mechani sms are i n pl ace for the reporti ng of adverse drug reacti ons?
Q1 What i nformati on do you need to answer thi s l ady's enqui ry?
P. 172
> Tabl e of Cont ents > 15 - Faci al pai n and neur ol ogi cal di st urbances
15
Facial pain and neurological disturbances
Problem cases
Case 15.1
A di stressed 60-year-ol d pati ent wi th faci al pai n presents to the oral medi ci ne cl i ni c. After
taki ng a hi story and carryi ng out an exami nati on, you excl ude a dental cause for the pai n and
make a cl i ni cal di agnosi s of tri gemi nal neural gi a. You di scuss the medi cal and surgi cal opti ons
for the management of thi s condi ti oni n the fi rst i nstance the pati ent requi res some
medi cati on.
Case 15.2
A 25-year-ol d, heal thy f emal e pati ent i s recei vi ng treatment i n your surgery. After
admi ni strati on of a l ocal anaestheti c mandi bul ar nerve bl ock (for crown preparati on of the
mandi bul ar fi rst permanent mol ar), your pati ent reports that the l ef t si de of her face feel s
strange. She i s unabl e to cl ose her l eft eye or smil e. You have prepared l ess than hal f of the
crown.
Facial pain: an overview
Pati ents wi th a compl ai nt of faci al pai n are seen dai l y i n the dental practi ti oner' s surgery.
They are often di agnosed and treated wi th rel ati ve ease, but there remai n some few pati ents
i n whom the ori gi n of the pai n symptoms remai ns obscure and who are referred to speci al i st
cl i ni cs of vari ous ki nds for di agnosi s. Apart from these pati ents wi th symptoms of pai n, very
few pati ents present wi th other neurol ogi cal di sturbances around the mouth and face. The
di agnosi s of these may be a compl ex matter and outsi de the provi nce of denti stry, but i t i s
i mportant that the dental practi ti oner i s abl e to recogni ze si gni fi cant changes and i ni ti ate
further acti on. Tabl e 15. 1 gi ves some of the condi ti ons that mi ght gi ve ri se to faci al pai n.
The matter may be further compl i cated by the di ffi cul ti es that may be met by the pati ent i n
descri bi ng hi s or her symptoms accuratel y. On the whol e, however, the terms used by pati ents
to descri be pai nstabbi ng, throbbi ng, dul l ache, and so onare
fai rl y constant and rel ated to the aeti ol ogy of the pai n.
Q1 What i s the usual drug of choi ce for thi s pati ent and what starti ng dose woul d you
prescri be?
Q2 What warni ngs and i nformati on woul d you gi ve to the pati ent?
Q3 What i nvesti gati ons do you need to carry out before starti ng on thi s drug?
Q4 If your pati ent had presented taki ng warfari n, woul d you envi sage any management
probl ems?
Q1 How woul d you manage thi s si tuati on?
The reacti on of the pati ent to pai n depends on two factors that are i ndependent of the
strength of the pai n sti mul us. These are the pai n threshol d for the pati ent (the degree of
sti mul ati on necessary for the pati ent to percei ve pai n) and the i ndi vi dual ' s sensi ti vi ty to the
pai n when percei ved. These two f actors vary greatl y from pati ent to pati ent and, i n the case
of the i ndi vi dual sensi ti vi ty, may vary from ti me to ti me i n the same pati ent, dependi ng on
general heal th and other psychol ogi cal factors. Pai n cannot be separated from an emoti onal
response.

Facial pain
Pai n i s a common and frequent occurrence experi enced by most pati ents. Pai n may be acute
or chroni c. Pai n not onl y has consequences to the pati ent and hi s or her qual i ty of l i fe, but i t
can al so have an i mpact on the pati ent' s soci al ci rcl e and on the soci ety and economy of a
nati on. Pai n i s responsi bl e for a l arge proporti on of mi ssed days of work.
The majori ty of pati ents wi th faci al pai n are suf feri ng from some easi l y detectabl e
pathol ogi cal process i n the teeth, thei r supporti ng ti ssues, or associ ated structures. Whatever
the nature of the pai n and however unusual i ts presentati on, the fi rst step must be the
el i mi nati on of the pai ns of l ocal ori gi nsuch as abnormal pul ps, buri ed teeth and roots,
peri api cal l esi ons, or acute peri odontal l esi ons. Odontogeni c pai n may be readi l y di agnosed by
cl i ni cal and radi ographi c exami nati on. Unfortunately, every dental practi ti oner knows that thi s
i s not al ways so strai ghtforward. Faci al pai n of dental ori gi n may be di ffi cul t to recogni ze and
referred or projected pai n may make l ocal i zati on diffi cul t. Cl i ni cal and radi ographi c
exami nati on may be i nconcl usi ve. Vi tal i ty testi ng of the teeth and the use of di agnosti c l ocal
anaestheti c i njecti ons may be usef ul ai ds. Pul pi ti s may be di ff i cul t to l ocal i ze, especi al l y i n a
heavi l y restored quadrantsometi mes restorati ons have to be removed and teeth dressed i n
a systemati c manner. Thi s i s frustrati ng f or both the cl i ni ci an and the pati ent. In parti cul ar,
the pai n associ ated wi th temporomandi bul ar joi nt dysf uncti on may be very puzzl i ng and may
si mul ate a number of other condi ti ons.
The di agnosi s of faci al pai n can be di ffi cul t due to the mul ti factori al nature of the probl em.
Table 15.1 Conditions that may cause facial pain
Dental pai n
Pul pi ti s, cracked tooth, peri radi cul ar pathol ogy, peri coroni ti s, dry socket
Temporomandi bul ar pai n di sorders
Neuropathi c pai n di sorders
Tri gemi nal neural gi a, gl ossopharyngeal neural gi a, postherpeti c neural gi a
Pathol ogy i n associ ated l ocal structures (i .e. sal i vary gl ands, paranasal si nuses,
eyes, cervi cal spi ne di sorders, nasopharynx, ears)
Vascul ar di sorders
Headaches: mi grai ne, cl uster headache, tensi on-type headache
Gi ant cel l arteri ti s
Psychogeni c faci al pai n
Atypi cal faci al pai n
Intracrani al l esi ons
Neopl asms
Mul ti pl e scl erosi s
Referred pai n from a remote si te
Angi na pectori s
P. 176
The nerve supply to the face
The sensory nerve suppl y to the face and oral ti ssues i s shared among a number of nerves:
the tri gemi nal nerve; the gl ossopharyngeal nerve; and the branches of the cervi cal pl exus
(see Fi g. 15.1). However, the great majori ty of pain symptoms i n the f ace are f el t i n the area
covered by the tri gemi nal nerve. There i s usual l y a cl ear-cut di sti ncti on between the zones
suppl i ed by the vari ous termi nal branches of the tri gemi nal nerve, wi th very l i ttl e overl ap, but
there i s often consi derabl e overl ap between the trigemi nal and cutaneous branches of the
cervi cal pl exus where these are adjacent. Apart from areas of overl ap there i s al so a compl ex
seri es of i nterconnecti ons between the tri gemi nal , faci al , and gl ossopharyngeal nerves and the
nerves ari si ng f rom the autonomi c nervous system, in parti cul ar, the sympatheti c fi bres
associ ated wi th bl ood vessel s servi ng the area. These sympatheti c fi bres may pl ay some part
i n the transmi ssi on of deep i mpul ses. It has been shown that such pai n can be produced by
the sti mul ati on of the superi or cervi cal sympatheti c gangl i on.
The great majori ty of pati ents compl ai ni ng of pai n i n and about the face are suf feri ng from
some form of toothache. However, there are many other possi bl e causes of such pai n (Tabl e
15. 1). The structures rel ated to the mouth that mi ght gi ve ri se to pai n symptoms are so
compl ex, and the i nnervati on of these structures so i nterrel ated, that errors i n di agnosi s are
easi l y made. The mai n sensory nerve of the area, the tri gemi nal nerve, eventual l y di vi des i nto
a l arge number of smal l termi nal branches suppl yi ng the ski n of a l arge part of the face and
scal p as wel l as the majori ty of the oral ti ssues and many deeper structures. Al though the
superfi ci al l i mi ts of the tri gemi nal nerve can be accuratel y determi ned wi th l i ttl e overl ap, the
deeper l i mi ts are much l ess wel l defi ned and understood. It i s often di ffi cul t to determi ne the
poi nt at whi ch a f aci al pai n becomes a headache (for exampl e, i n the temporal regi on) and
there may be consequent di ffi cul ti es i n communi cati on between the pati ent and the cl i ni ci an.
Another possi bl e source of confusi on when di agnosi ng orofaci al pai n i s that of projected and
referred pai n.
Projected and referred pain
If a pai n pathway i s subjected to sti mul ati on at some poi nt al ong i ts course, i t i s possi bl e f or
pai n to be fel t i n the peri pheral di stri buti on of the nerve. Thi s i s projected pai n. An exampl e
of thi s i s the faci al pai n that may occur i n pati ents wi th i ntracrani al neopl asms.
If the source of a pai n and the si te of the pai n (the l ocati on) are one and the same, the pai n
i s consi dered to be pri mary pai n. Thi s

must be di sti ngui shed from referred pai n i n whi ch the pai n i s fel t i n an area di stant f rom that
i n whi ch the causati ve pathol ogy i s l ocated. Pai n referral i s a common occurrence i n the
orofaci al regi on and cl i ni ci ans must appreci ate this phenomenonotherwi se erroneous
di agnoses and management may ensue. In referred pain the practi ti oner must be abl e to
i denti fy the true source of the pai n f rom the si te (l ocati on) of the pai n. In the dental fi el d,
perhaps the most common exampl e of referred pai n i s that i n whi ch pai n i s f el t, for i nstance,
i n the maxi l l ary teeth due to a l esi on i n a mandi bul ar tooth. In thi s case the pai n i mpul ses
ori gi nate i n the di seased tooth and, i f the pathway from thi s tooth i s bl ocked, for exampl e, by
Fig. 15.1 Sensory dermatomes of the tri gemi nal and upper cervi cal nerves.
P. 177
a l ocal anaestheti c, then the referred pai n wi l l cease. Thus, i n our dental exampl e,
anaesthesi a of the mandi bul ar branch of the tri geminal nerve wi l l rel i eve the pai n fel t i n the
area suppl i ed by the maxi l l ary branch. Thi s i s the basi s of a most usef ul test for the
i nvesti gati on of suspected referred pai n.
Al though pai n i s frequentl y referred between di fferi ng branches of the tri gemi nal nerve, pai n
referred to the face from a di stant area i s rel ati vel y uncommon. The onl y referred pai n of thi s
type seen wi th any degree of frequency i s that i n coronary i nsuffi ci ency, when pai n may
radi ate over the l eft si de of the mandi bl e. Angi na pectori s has al so been descri bed as
presenti ng as a pai n of the soft pal ate, but thi s is unusual . Thi s accompani es other wel l -
recogni zed symptoms of the condi ti on, but a very few i nstances have been reported i n whi ch
pai n over the l eft mandi bl e has been the f i rst compl ai nt of a pati ent who subsequentl y
devel oped symptoms of myocardi al i nfarcti on.
The mechani sm of projected pai n i s fai rl y cl ear, but that of referred pai n i s more contenti ous.
There i s sti l l consi derabl e debate as to the theories of pai n referral and the si te at whi ch the
cross-over or neural convergence occurs l eadi ng to the sensory mi si nterpretati on of
the i mpul ses transmi tted from the di seased si te.
Referred pain
Pai n of dental ori gi n can be ref erred to the opposing dental arch.
The evaluation of facial pain
Pai n i s extremel y subjecti ve and i n many i nstances there are few cl i ni cal si gns. Pai n needs to
be assessed on two di mensi onsas a di sease, usi ng a medi cal model , and as an i l l ness that
i nevi tabl y has an effect on the qual i ty of l i fe. The response of pati ents to pai n i s extremel y
vari abl e and i t i s thi s that makes assessment of pai n so di ffi cul t. Some pai n acts as an earl y
warni ng system and so i s cruci al for survi val , whereas other pai ns have outl i ved thei r
useful ness. Many of the cl i ni cal condi ti ons seen by the dental surgeon seem to fal l i nto a
category i n whi ch mi nor (or even qui te unrecogni zabl e) pathol ogi cal changes el i ci t a qui te
di sproporti onate response l eadi ng to great di stress i n the i ndi vi dual concerned.
The assessment and di agnosi s of orofaci al pai n requi re the cl i ni ci an to have knowl edge and
understandi ng of the anatomy, physi ol ogy, and pathol ogy of the head and neck and, i n
parti cul ar, of the orofaci al structures. The cl i ni ci an shoul d be abl e to undertake a systemati c
cl i ni cal assessment as shown i n Tabl e 15. 2. The i nvesti gati on of a pati ent compl ai ni ng of f aci al
pai n may be thought of as havi ng two stages. In the fi rst stage an assessment of the pai n i s
made rel ati ve to the dental apparatus (teeth and supporti ng ti ssues) and the cl osel y rel ated
structures, such as the maxi l l ary antra and the temporomandi bul ar joi nts. If, after ful l
exami nati on, no abnormal i ti es are found i n these areas, the i nvesti gator i s justi fi ed i n
consi deri ng other possi bi l i ti es. If the essenti al fi rst step of el i mi nati ng dental causes of the
pai n i s omi tted, conf usi on i s bound to fol l ow i n many cases. When al l these l ocal i zed causes of
pai n are el i mi nated by careful i nvesti gati on, there remai ns a number of condi ti ons of l ess
evi dent ori gi n for consi derati on i n a possi bl e di agnosi s.
History
A detai l ed pai n hi story i s cruci al for pati ents wi th faci al pai n and i n 7080 per cent of cases
a di agnosi s i s made on hi story al one. The cl i ni ci an can gai n val uabl e i nformati on by observi ng
the pati ent when he or she i s descri bi ng the features of the pai n. It i s al so i mportant that the
pati ent i s i ni ti al l y al l owed to descri be the pai n in hi s or her own wordswi thout prompti ng
from the denti st. A pai n hi story shoul d be undertaken i n a systemati c fashi on and al l the
features l i sted i n Tabl e 15. 3 shoul d be expl ored. Some f orm of more objecti ve assessment of
pai n i s i mportant i n order to measure not just i ntensi ty but the di sabi l i ty i t i s causi ng. The use
of a l i near pai n scal e and the McGi l l pai n questi onnai re may be hel pful . The hi story shoul d
i ncl ude the eff ects that the pai n has on the l i festyl e of the pati ent. Soci al , cogni ti ve,
emoti onal , and behavi oural f actors become very i mportant i n chroni c pai n and these are
di scussed further i n Chapter 17. The soci al and fami l y hi stori es are cruci al as a pati ent' s pai n
i mpacts on those wi th whom they are l i vi ng.
Examination
The exami nati on of the orofaci al ti ssues shoul d i ncl ude a gross sensory and motor eval uati on
of the crani al nerves (l i sted i n Tabl e 15. 4). Abnormal functi oni ng of the crani al nerves shoul d
i ndi cate to the cl i ni ci an that referral to a neurologi st or other appropri ate speci al i st i s
requi red. The fi fth and seventh nerve need to be exami ned wi th care and al terati ons i n
sensory functi on are anaesthesi a, paraesthesi a, dysaesthesi a, and hei ghtened sensi ti vi ty to
pai n (hyperal gesi a). Motor dysfuncti on i s evi dent from paral ysi s, gross muscl e weakness,
muscul ar atrophy, or spasti ci ty.
Neuropathic pain
Accordi ng to the Internati onal Associ ati on for the Study of Pai n, the defi ni ti on of neurogeni c
pai n (al so known as neuropathi c

pai n) i s pai n i ni ti ated or caused by a pri mary l esi on, dysfuncti on, or transi tory pertubati on i n
the peri pheral or central nervous system. Thi s defi ni ti on i s al l -i ncl usi ve and encompasses
nerve-i njury-rel ated condi ti ons.
P. 178
Table 15.2 The assessment and management of facial
pain
Hi story
Compl ai nt
Pai n hi story
Onset, peri odi ci ty, l ocati on and radi ati on, durati on, frequency, character,
and i ntensi ty of pai n
Modi f yi ng factors (i ni ti ati ng, aggravati ng, and al l evi ati ng factors)
Effect on l i festyl e
Effect on mooddepressi on, anxi ety
Outcome of previ ous i nvesti gati ons and therapi es (e. g. medi cati on)
Consi der use of a vi sual anal ogue l i near pai n scal e and a pai n questi onnai re
Dental hi story
Medi cal hi story (i ncl udi ng al cohol and drug use)
Soci al hi story

Exami nati on
Odontogeni c structures
Dental status, pul p vi tal i ty tests, stati c and dynami c occl usal rel ati onshi ps;
Rel ated structures
Muscl es of masti cati on, temporomandi bul ar joi nt, sal i vary gl ands, si nuses
Crani al nerve functi on
Gross test of sel ected or al l crani al nerves
Sensory defi ci ts
Investi gati ons
Radi ography and other i magi ng techni ques
Haematol ogy and cl i ni cal chemi stry (eg ESR, vi tami n B
12
)

Vi rol ogy
Di agnosti c occl usal appl i ancey
Bi opsy
Psychometri c questi onnai res
Nerve conducti on tests
Di agnosti c, l ocal anaestheti c nerve bl ock
Thi s may hel p defi ne the di stri buti on of pai n, and can el i mi nate or confi rm
referred pai n
Request further i nformati on
From general practi ti oner, speci al i sts, hospi tal casenotes

Management
Establ i sh pati ents' treatment goal s and l i kel y adherence to therapy
El i mi nati on of odontogeni c pai n
Investi gate or dress a restored tooth, occl usal adjustment, extracti on
Medi cal treatment
Drug therapy (carbamazepi ne, anti depressants)
Refer to appropri ate speci al i st
Further i nvesti gati ons (e.g. to neurol ogi st f or nerve conducti on tests)
Table 15.3 Pain characteristics to be evaluated when
taking a pain history
Durati on
Locati on
Radi ati on
Provoki ng factors
Associ ated factors
Peri odi ci ty
Character
Severi ty
Rel i evi ng factors
Table 15.4 The cranial nerves
Number Name Number Name
Some useful neurological terms
The term neuropathi c pai n wi l l be used i n thi s chapter. Chroni c neuropathi c pai n i ncl udes
di sorders such as tri gemi nal and gl ossopharyngeal neural gi a, postherpeti c neural gi a, di abeti c
neuropathy, and peri pheral nerve damage due to HIV i nfecti on and al cohol . Trauma or
i nf l ammatory change affecti ng the extra- or i ntracrani al course of the nerve, f or exampl e,
spi nal nerve i njury or mul ti pl e scl erosi s, can al so resul t i n neuropathi c pai n. Beni gn or
mal i gnant neopl asms ei ther compressi ng or i nfi l trati ng a nerve can gi ve ri se to neuropathi c
pai n.

Thi s chapter di scusses neuropathi c pai n that i s l i kel y to be encountered i n the oral medi ci ne
cl i ni c. It may present wi th or wi thout other symptoms of neurol ogi al di sturbances (such as
paraesthesi a, hyperal gesi a, al l odyni a, and motor nerve dysfuncti on).
Neuropathi c pai n does not usual l y respond to conventi onal anal gesi cs such as aspi ri n,
paracetamol , and nonsteroi dal anti -i nf l ammatory drugs (NSAIDs). The medi cal management of
thi s pai n i s best achi eved usi ng other pharmacol ogical agents such as anti convul sants and
someti mes anti depressants. Treatment shoul d be i ni ti ated, where possi bl e, usi ng si ngl e drug
therapy. Thi s wi l l l i mi t the si de-effects.
I Ol factory VII Faci al
II Opti c VIII Acousti c vesti bul ar (vesti bul ocochl ear)
III Ocul omotor IX Gl ossopharyngeal
IV Trochl ear X Vagus
VI Abducens XI Accessory
V Tri gemi nal XII Hypogl ossal
Al ogeni c Causi ng pai n
Al l odyni a Pai n f rom a sti mul us that does not normal l y provoke pai n, e. g. l i ght
touch
Anal gesi a Absence of a pai n response to a pai nful sti mul us
Anaesthesi a Compl ete l oss of sensati on
Dysaesthesi a Al tered unpl easant sensati on; may be spontaneous or i n response to a
sti mul us
Hyperal gesi a An i ncreased response to a sti mul us that i s normal l y pai nful
Neuropathi c
pai n
Pai n i ni ti ated or caused by a pri mary l esi on, dysfuncti on, or transi tory
probl em i n the peri pheral or central nervous system
P. 179
Neuropathi c pai n i s pai n i ni ti ated or caused by a pri mary l esi on, dysf uncti on, or transi tory
perturbati on i n the peri pheral or central nervous system.
Trigeminal neuralgia
Tri gemi nal neural gi a: sudden, usual l y uni l ateral , severe, bri ef, stabbi ng, recurrent pai ns i n
the di stri buti on of one or more branches of the fi fth crani al nerve.
Tri gemi nal neural gi a i s a di sease of such characteri sti c i ntensi ty that i t has been known and
descri bed over a l ong peri od of hi story. In spi te of thi s, the true nature of thi s extremel y
pai nful condi ti on i s not known. In some pati ents there i s a vascul ar abnormal i ty (venous or
arteri al ) that causes compressi on of the tri gemi nal rootadjacent to the pons i n the
posteri or crani al fossa. Thi s resul ts i n areas of demyel i nati on and abnormal conducti on.
El i mi nati on of thi s compressi on has l ed to l ong-term pai n rel i ef i n most pati ents. The
associ ati on between mul ti pl e scl erosi s (MS) and trigemi nal neural gi a i s wel l known and i s
di scussed l ater i n thi s chapter.
A smal l proporti on (approxi matel y 2 per cent) of pati ents wi th posteri or fossa tumours (f or
exampl e, acousti c neuromas or beni gn tumours) present wi th typi cal tri gemi nal neural gi a.
Pai nful symptoms may precede or accompany sensory or motor def i ci ts i n these cases.
The pathophysi ol ogy of tri gemi nal neural gi a has yet to be el uci dated but one theory i s that the
paroxysms of pai n i n thi s condi ti on may represent di scharges i n sel ected neurones whose
threshol d for repeti ti ve fi ri ng has been al tered, possi bl y by focal nerve i njury. Such fi ri ng may
then be i ni ti ated by i nnocuous tacti l e sti mul i (this woul d expl ai n tri gger zones). Cross-
exci tati on of nei ghbouri ng nerves coul d then resul t i n the recrui tment of suffi ci ent di schargi ng
cel l s for a noci cepti ve si gnal to be percei ved as pai n. However, none of the current theori es
ful l y expl ai n the cl i ni cal features of tri gemi nal neural gi a.
Pretrigeminal neuralgia
Thi s condi ti on i s descri bed as a dul l ache, si mi l ar to toothache, and the pati ents subsequentl y
devel op tri gemi nal neural gi a. It i s thought to occur i n up to 20 per cent of pati ents who
devel op tri gemi nal neural gi a. Therapeuti c opti ons for tri gemi nal neural gi a are effecti ve for thi s
condi ti on. The cl i ni ci an must fi rst, however, ensure that there i s no pai n of dental ori gi n
responsi bl e for these symptoms. It i s at thi s ti me that the pati ents are most often seen by the
dental surgeon for the i nvesti gati on of what may be hi ghl y perpl exi ng pai n symptoms.
Pretri gemi nal neural gi a i s a di agnosi s made i n retrospect.
Clinical presentation of trigeminal neuralgia
The i nci dence of tri gemi nal neural gi a i s around 150 per 1 mi l l i on and more pati ents are f emal e
(f emal e:mal e rati o approxi matel y 2:1). The preval ence of the condi ti on i s 1 i n 1000. The peak
onset i s i n the fi fth to seventh decades and symptoms usual l y occur after the fourth decade.
The pai n di stri buti on i s al most al ways uni l ateral in the fi rst i nstance, wi th onl y 3 per cent of
cases subsequentl y devel opi ng bi l ateral i nvol vement. Pai n may aff ect one, two, or even three
di vi si ons. The f i rst di vi si on on i ts own i s onl y rarel y affected. The pai n i s of great i ntensi ty, i s
descri bed as sharp and stabbi ng i n nature (l anci nati ng), and l asts for onl y a few seconds. Thi s
transi ent attack may be repeated i n a matter of mi nutes or hours. There may be no apparent
preci pi tati ng factor, but many pati ents have a tri gger zone, a poi nt on the face or i n
the mouth (often outsi de the area of pai n di stri buti on) where the l i ghtest contact may i ni ti ate
an attack. Sti mul i may i ncl ude touchi ng, washi ng, draughts of col d ai r, toothbrushi ng, eati ng,
or smi l i ng. Between the paroxysms of pai n there may be a dul l background ache i n the area or
no pai n at al l . These attacks rarel y occur at ni ght. The so-cal l ed frozen face i s an
attempt by the pati ent to defer attacks by l i mi ti ng movement, hence avoi di ng the tri ggeri ng of
the pai n. Spontaneous remi ssi on for a matter of weeks or months may occur, but thi s i s rarel y
permanent. Pati ents i n severe pai n l ose wei ght, are unabl e to soci al i ze, and often become
depressed.
Descri ptors of pai n i n tri gemi nal neural gi a:
sharp
stabbi ng
el ectri c shock-l i ke
Investigation of patients with trigeminal neuralgia
Tri gemi nal neural gi a i s di agnosed pri nci pal l y from the pai n hi story. There i s no speci fi c
di agnosti c test, but a ful l cl i ni cal exami nati on, incl udi ng assessment of the crani al nerves,
shoul d be carri ed out to excl ude other causes of pai n. Very few pati ents have subtl e sensory
changes, and tri gger poi nts can usual l y be el i ci ted. The dental surgeon has a pi votal rol e i n
the di agnosi s of pati ents presenti ng wi th tri gemi nal neural gi a. He/she must al ways excl ude a
dental cause for the pati ent' s pai nful symptoms.
Pati ents wi th tri gemi nal neural gi a may ascri be the pai n to toothache i n what may appear to be
an enti rel y sound tooth. Fol l owi ng the extracti on of thi s tooth the pai n may then be descri bed
as comi ng from a nearby tooth and extracti on of this may al so be demanded by the pati ent. It
i s not rare to fi nd pati ents made vi rtual l y uni l ateral l y edentul ous i n thi s way. Many eventual
di ffi cul ti es can be avoi ded i f i t i s recogni zed that any pati ent presenti ng wi th possi bl e
tri gemi nal neural gi a symptoms

shoul d be consi dered as worthy of ful l i nvesti gati on before further extracti ons are consi dered.
Trigeminal neuralgiakey features
Bri ef paroxysms of sharp, stabbi ng pai n
Tri gger zone(s)
Provoki ng factors i ncl ude eati ng, tal ki ng, shavi ng, washi ng face
Pai n usual l y confi ned to one di vi si on of tri gemi nal nerve
Usual l y uni l ateral
Usual l y pai n-free duri ng sl eep
Drug therapy wi th carbamazepi ne i s the medi cal treatment of choi ce
Peri pheral or central surgi cal treatments are management opti ons
May have extended remi ssi on wi th compl etel y pai n-free peri ods
The dental surgeon has a pi votal rol e i n the di agnosi s of pati ents presenti ng wi th tri gemi nal
neural gi aa dental cause for the pati ent' s pai nful symptoms must al ways be excl uded.
Conversel y, the si tuati on may ari se i n whi ch pai n cl osel y resembl i ng tri gemi nal neural gi a may
ari se from some qui te ordi nary dental pathol ogy such as a retai ned root or a cracked tooth or
even from unsati sfactory dentures. If there i s any abnormal i ty of thi s ki nd present, i t shoul d
be treated as the essenti al fi rst step i n the management of the pati ent.
Younger pati ents (< 50 years of age) presenti ng wi th tri gemi nal neural gi a, parti cul arl y i f
there i s bi l ateral i nvol vement, shoul d be ref erred to a neurol ogi st for appropri ate di agnosti c
testi ng to excl ude mul ti pl e scl erosi s.
P. 180
As has been poi nted out, tri gemi nal neural gi a tends to occur i n l ater l i fe. The onset of
symptoms of tri gemi nal neural gi a i n a pati ent under the age of 40 years shoul d be treated
wi th some suspi ci on as a possi bl e fi rst warni ng of the onset of mul ti pl e scl erosi s (see next
secti on). In thi s condi ti on the neural gi a-l i ke pai ns are often the f i rst symptoms. It has been
suggested that pati ents of thi s age group shoul d possi bl y be ref erred for a speci al i st
neurol ogi cal assessment. When assessi ng the need for a neurol ogi cal opi ni on on a pati ent wi th
suspected tri gemi nal neural gi a, i t shoul d al ways be remembered that there are no di agnosti c
i ndi cators other than the pai n i tsel f. Any other associ ated neurol ogi cal f eatures, such as
muscl e weakness, bl urri ng of vi si on, di zzi ness, paraesthesi a, or the possi bl e i nvol vement of
other crani al nerves, shoul d be taken as absol ute indi cati ons for a ful l neurol ogi cal
assessment and cerebral i magi ng.
Central nervous system i magi ng shoul d be undertaken i f there i s sensory l oss.
There are a number of condi ti ons to be consi dered i n the di f ferenti al di agnosi s of tri gemi nal
neural gi a (Tabl e 15. 5) but i n many of these the pai n symptoms reported by the pati ent are
not characteri sti c of tri gemi nal neural gi a (that i s, bri ef, shooti ng, paroxysmal pai n).
Tri gemi nal neural gi a may be the fi rst mani festati on i n a smal l number of pati ents wi th MS.
These are younger than the tri gemi nal neural gi a popul ati on as a whol e and thei r neural gi a i s
often bi l ateral . Conversel y, tri gemi nal neural gi a is di agnosed i n 15 per cent of pati ents
wi th MS.
Medical management of trigeminal neuralgia
Pharmacol ogi cal therapy i s sti l l consi dered the most appropri ate management for most
pati ents wi th tri gemi nal neural gi a, but recent resul ts from l arge neurosurgi cal seri es woul d
justi f y surgi cal i nterventi on earl y on, parti cul arly i n the younger pati ent. Al l pati ents shoul d
be gi ven a di spassi onate account of the medi cal and surgi cal opti ons avai l abl e.
The medi cal treatment of tri gemi nal neural gi a i s pri mari l y wi th the use of
anti convul sants
Carbamazepi ne i s currentl y the drug of choi ce
The i ni ti al dose of carbamazepi ne i s 100 mg, twi ce dai l y; thi s i s i ncreased gradual l y, by
100 mg each day, unti l the pai n i s control l ed
Table 15.5 The differential diagnosis of trigeminal
neuralgia
Dental pai n, especi al l y cracked tooth syndrome
Si nus pai n
Postherpeti c neural gi a
Tri gemi nal neural gi a secondary to nerve i njury, tumour, or other pathol ogi cal
condi ti on
Cl uster headache
Temporomandi bul ar pai n dysfuncti on syndrome
A maxi mum dai l y dose of 8001000 mg shoul d not be exceeded
Pharmacol ogi cal treatment of tri gemi nal neural gi a depends l argel y upon the use of
anti convul sants and the drug of choi ce has tradi ti onal l y been carbamazepi ne (Tegratol ).
Response to carbamazepi ne i s reportedl y di agnosti c for tri gemi nal neural gi a; however, i t i s
worth menti oni ng that carbamazepi ne can occasi onal ly reduce (or abol i sh) pai n of odontogeni c
ori gi n. Dental causes of faci al pai n shoul d, therefore, have been el i mi nated pri or to the
commencement of drug therapy. Other anti convul sant drugs are occasi onal l y used, but
carbamazepi ne remai ns the gol d standard therapeuti c agent for tri gemi nal neural gi a
and has been eval uated i n randomi zed pl acebo-control l ed tri al s. The i ni ti al dosage of
carbamazepi ne i s kept l ow and started at 100 mg, twi ce dai l y, and gradual l y i ncreased i n dai l y
i ncrements of 100 mg unti l control of pai n i s attained. A maxi mum dose of 8001000 mg
dai l y shoul d not be exceeded. The medi cati on shoul d be taken about

30 mi nutes before meal s, thus maxi mi zi ng pai n control for masti cati on. About 70 per cent of
pati ents wi th tri gemi nal neural gi a are esti mated to benefi t f rom carbamazepi ne. Al l pati ents
wi l l have mi nor si de-effects, whi ch vary and are dose-dependent. Some pati ents experi ence
drowsi ness or di zzi ness, whereas some report gastroi ntesti nal reacti ons. In a very few
pati ents, however, the much more seri ous si de-effect of agranul ocytosi s may occur. The
el derl y are parti cul arl y suscepti bl e to si de-effects. The whi te cel l count may be moni tored by
repeated bl ood tests, but i t woul d appear that, when thi s reacti on occurs, i t may do so
suddenl y and wi thout pri or warni ng. Other, l ess seri ous, haematol ogi cal effects have been
recorded i ncl udi ng anaemi a, neutropeni a, thrombocytopeni a, and hyponatraemi a. For these
reasons i t i s general l y recommended that haematol ogy and bl ood chemi stry testi ng i s carri ed
out at the commencement of treatment, and at 1 month and 3 months, and thereafter
dependi ng on ci rcumstances. It i s i mportant to rei terate, however, that a sudden, potenti al l y
fatal , fal l i n the whi te cel l count may occur as a very rare event, and may be enti rel y mi ssed
by l ong-term bl ood-testi ng regi mes. It i s al so general l y recommended that l i ver functi on tests
shoul d be part of the drug moni tori ng programme, as carbamazepi ne i s acti ve i n l i ver enzyme
i nducti on. Hyponatraemi a (a reduced serum l evel of sodi um) has al so been reported wi th
carbamazepi ne. When prescri bi ng carbamazepi ne the cl i ni ci an shoul d al ways be aware of
possi bl e drug i nteracti onssome drugs may become i neffecti ve because therapeuti c serum
l evel s are not achi eved or mai ntai ned due to the i ncreased rate of metabol i sm. Warfari n i s one
such drug.
Monitoring for patients on carbamazepine therapy
Ful l bl ood count, l i ver functi on tests, and el ectrol ytes shoul d be moni tored:
pri or to therapy
at 1 month
at 3 months
A further si de-effect of carbamazepi ne, whi ch occurs i n approxi matel y 7 per cent of pati ents,
i s the occurrence of a severe rash. Just as i n the case of agranul ocytosi s, thi s may occur qui te
unexpectedl y after a l ong peri od of treatment wi th the drug.
As a response to the probl ems associ ated wi th carbamazepi ne therapy, a rel ated drug,
oxcarbazepi ne, was i ntroduced. Thi s has essenti al l y the same properti es as carbamazepi ne but
i s reputed to be better tol erated. Thi s i s not, however, al ways the case. Oxcarbazepi ne has yet
to establ i sh i ts pl ace as a substi tute for carbamazepi ne as up to a quarter of pati ents
experi ence cross-sensi ti vi ty.
There are other drugs that may be used i n the treatment of tri gemi nal neural gi a for pati ents
who cannot tol erate carbamazepi ne. These i ncl ude phenytoi n, l amotrogi ne, bacl ofen, and
P. 181
cl onazepam. None of these drugs are as effecti ve as carbamazepi ne i n the control of
tri gemi nal neural gi a, al though phenytoi n i n conjuncti on wi th carbamazepi ne may be val uabl e
i n some pati ents unresponsi ve to carbamazepi ne al one. The starti ng dose for phenytoi n i s
usual l y 300 mg/day (i n di vi ded doses) al though thi s drug al one i s rarel y successful i n
control l i ng pai n. Phenytoi n has a number of si de-effects i ncl udi ng a severe rash. Consi deri ng
the morbi di ty and preval ence of tri gemi nal neural gia, very few control l ed studi es have been
carri ed out on drugs avai l abl e for treatment. A recentl y i ntroduced drug, gabapenti n, has been
eval uated for use i n postherpeti c neural gi a and di abeti c neuropathy and may be useful f or
tri gemi nal neural gi a. It appears to be comparati vel y free of seri ous si de-effects and can be
used i n combi nati on wi th carbamazepi ne.
Side-effects of carbamazepine therapy
Drowsi ness, di zzi ness, confusi on
Verti go
Nausea
Ski n reacti onrash
Leukopeni a (anaemi a, neutropeni a, thrombocytopeni a, and apl asti c anaemi a occur
rarel y)
Hepati c mi crosomal enzyme i nducti on (and consequent drug i nteracti ons)
Hyponatraemi a
Surgical treatment of trigeminal neuralgia
A mi nori ty of pati ents are unsui tabl e for drug therapy, ei ther because they areor have
becomeunresponsi ve to i t or cannot tol erate the si de-effects. Others are keen to expl ore
the surgi cal opti ons i n the hope of a permanent cure. There a number of di fferent surgi cal
approaches avai l abl e for the treatment of tri gemi nal neural gi a and these vary i n compl exi ty
from rel ati vel y si mpl e peri pheral procedures (for exampl e, cryotherapy) to hi gh-frequency
sel ecti ve coagul ati on of the nerve fi bres i n the regi on of the gangl i on and open i ntracrani al
procedures i n the posteri or fossa for mi crovascul ar decompressi on of the tri gemi nal nerve
(Tabl e 15. 6). Ful l detai l s of these procedures are, however, outsi de the scope of thi s book
(see Project 3 at the end of thi s chapter). When consi deri ng a surgi cal approach to treatment,
the fol l owi ng f actors must be consi dered: postoperati ve pai n rel i ef; recurrence rates; general
heal th; ri sks; and adverse effects.
Mi crovascul ar decompressi on of the tri gemi nal nerve i s now consi dered the surgi cal treatment
of choi ce by a number of centres, provi ded that the pati ent i s i n reasonabl e heal th and
evi dence of vascul ar decompressi on has been i denti fi ed, as i t i s the onl y procedure that i s
non-destructi ve. A speci f i c i magi ng techni que, magneti c resonance tomographi c angi ography
(MRTA), i s

used to opti mal l y i mage the tri gemi nal nerve and bl ood vessel s i n i ts vi ci ni ty.
P. 182
Table 15.6 Surgical treatments in current use for
trigeminal neuralgia
Surgical therapies Procedure Morbidity Recurrence
after
surgery
(average)
Peri pheral
Cryotherapy,
neurectomy,
al cohol i njecti ons
The peri pheral nerve
branch i s ei ther
di rectl y exposed or
i njected under l ocal
anaesthesi a
Low and l ocal ,
mai nl y mi l d
sensory l oss
Up to a
year
Gasseri an gangl i on
Radi ofrequency,
thermocoagul ati on
Short-acti ng,
i ntermi ttent general
anaesthesi a used to
penetrate foramen
oval e. The Gasseri an
gangl i on i s subjected
to cycl es of thermal
l esi oni ng
(temperature varyi ng
from 60 to 80C)
Sensory
i mpai rment to the
tri gemi nal nerve,
anaesthesi a
dol orosa (rare),
corneal
anaesthesi a (wi th
subsequent ri sk of
kerati ti s).
Mortal i ty rate l ow.
35 years
Gl ycerol i njecti on Local anaesthesi a
( sedati on) can be
used. Meckel ' s cave
i s f i l l ed wi th gl ycerol
Dysaesthesi a
wi thi n the
tri gemi nal area
Up to 3
years
Mi crocompressi on
(bal l oon
compressi on)
Ful l general
anaesthesi a used.
The Gasseri an
gangl i on i s
compressed by a
bal l oon for a few
seconds
Dysaesthesi a can
be probl emati c.
Motor component
often i nvol ved
35 years
Posteri or fossa
Mi crovascul ar
decompressi on
Ful l general
anaesthesi a used.
Any vessel s l yi ng on
the tri gemi nal nerve
at the poi nt of entry
to the crani um are
moved asi de or
removed
8th crani al nerve
damage may
occur. Mortal i ty
rates of up to
0. 7% reported.
Seri ous morbi di ty,
10%
8 years
Surgi cal treatment of tri gemi nal neural gi afactors to be consi dered:
pai n rel i ef
recurrence rates and thei r management
compl i cati onsmorbi di ty and mortal i ty
Glossopharyngeal neuralgia
Gl ossopharyngeal neural gi a i s si mi l ar to neural gi a affecti ng the tri gemi nal nerve, but much
l ess common. The pai n i s of the same nature as that of tri gemi nal neural gi a and i s uni l ateral l y
fel t i n the oropharynx, someti mes wi th pai n referred to the i psi l ateral ear. Al though the mai n
component of the pai n i s descri bed (as i n tri gemi nal neural gi a) as stabbi ng or shooti ng i n
nature, there may al so be an appreci abl e resi dual ache that may l ast f or some ti me after the
paroxysmal attack. It i s l ess severe than tri gemi nal neural gi a. The pai n i s often preci pi tated
by swal l owi ng, chewi ng, or coughi ng, and there may be a tri gger zone. Cardi ac arrhythmi as
and syncope have been descri bed duri ng attacks. Gl ossopharyngeal neural gi a may be
secondary to a tumour of the throat, posteri or crani al fossa, or jugul ar foramen.
Gl ossopharyngeal neural gi a i s a rare condi ti on characteri zed by a paroxysmal , sharp,
l anci nati ng pai n that affects one si de of the throat and base of the tongue. It i s usual l y
tri ggered by swal l owi ng.
Treatment i s the same as for tri gemi nal neural gi a. Spontaneous remi ssi on of thi s condi ti on i s
possi bl e but fi rst-l i ne

treatment i s usual l y wi th an anti convul sant and carbamazepi ne i s successful i n most cases. A
posi ti ve response to thi s hel ps confi rm the di agnosi s. A surgi cal approach may be requi red i f
drug therapy becomes i neffecti ve or probl emati cal .
Postherpetic neuralgia
Herpes zoster (shi ngl es) i s the reacti vati on of a latent i nfecti on wi th the vari cel l a zoster vi rus
(see Chapter 4). Thi s vi rus i s usual l y reacti vated once i n a l i feti me, wi th fewer than 5 per cent
Stereotacti c
radi osurgery
Local anaesthesi a
( sedati on) can be
used. Usi ng
stereotacti c
techni ques the
posteri or fossa i s
i denti fi ed and a
gamma kni fe i s
di rected at the
proxi mal tri gemi nal
root near the pons
Long-term effects
of radi ati on
unknown. Sensory
l oss of sl ow onset
Mean 15
months
*Adapted wi th permi ssi on from T. J. Nurmi kko, Pai n Research Insti tute, Uni versi ty of
Li verpool , and P. R. El dri ge, The Wal ton Centre f or Neurol ogy and Neurosurgery NHS
Trust, Li verpool , UK.
P. 183
of pati ents havi ng a second attack. Herpes zoster i s more l i kel y to occur i n l ater l i fe and i n
pati ents who have T-cel l i mmunosuppressi on. Pai n may occur bef ore, duri ng, or after an
attack of f aci al herpes zoster. The fi rst warni ng of such an attack may be a severe burni ng
pai n occurri ng i n the area of the erupti on, up to 2 days bef ore the vesi cl es appear. The pai n i n
herpes zoster i s much more severe than that i n herpes si mpl ex, i n whi ch the symptoms are
l argel y due to the secondary i nf ecti on of ruptured vesi cl es (Chapter 4). The pai n associ ated
wi th herpes zoster usual l y subsi des wi thi n weeks. In some pati ents a neural gi a-l i ke pai n
persi sts f or months or even years. The pai n has an i ntense burni ng component and may be
accompani ed by stabbi ng sensati onsthere i s typi cal l y hyperal gesi a or paraesthesi a and
al l odyni a. The di stri buti on of pai n fol l ows the di stri buti on of the ori gi nal i nfecti on. Thi s
neural gi a tends to be a persi stent as opposed to a paroxysmal pai n. In addi ti on, the pai n has
a more vari abl e character and severi ty than tri geminal neural gi a. The i nci dence of tri gemi nal
postherpeti c neural gi a i s uncertai n, but ol der patients wi th herpes zoster are parti cul arl y
suscepti bl e to postherpeti c neural gi a60 per cent of pati ents over 60 are affected by thi s
di stressi ng condi ti on. It i s rel ati vel y uncommon i n those under the age of 50 years. The ri sk of
postherpeti c neural gi a i s al so thought to be greater i f pati ents have severe pai n or rash duri ng
the acute phase and al so i f the pati ents experi ence pai n pri or to the appearance of the rash.
Treatment of establ i shed postherpeti c neural gi a can be very di ffi cul ti t i s often resi stant to
treatment. It does not appear to respond to carbamazepi ne and anal gesi cs may al so be
i neffecti ve. Steroi ds have been advocated but there i s no confi rmati on of thei r val ue i n
preventi ng postherpeti c neural gi a. Tri cycl i c anti depressants have been associ ated wi th pai n
rel i ef i n some pati ents and gabapenti n has been shown, i n cl i ni cal tri al s, to be ef fecti ve for
postherpeti c neural gi a. Anti vi ral treatment combi ned wi th anti depressants i n the el derl y i n the
acute phase has been found to reduce the durati on and preval ence of postherpeti c neural gi a,
by as much as 50 per cent. The use of transcutaneous el ectri cal sti mul ati on i s thought to be
effecti ve for some pati ents.
To summari ze, prophyl axi s offers the best hope of preventi ng the di stressi ng pai n of
postherpeti c neural gi a affecti ng the tri gemi nal nerve. Al l pati ents wi th herpes zoster shoul d be
vi gorousl y treated wi th systemi c anti vi ral agents such as aci cl ovi r. These shoul d be
commenced as soon as possi bl e af ter the onset of the rash. Mul ti centre cl i ni cal tri al s have
establ i shed that successful treatment of the pri mary i nfecti on consi derabl y reduces the ri sk of
the subsequent devel opment of neural gi a.
Pai n may precede, accompany, and persi st after the acute vesi cul ar phase of herpes
zoster
Pai n persi sti ng af ter herpes zoster i s cal l ed postherpeti c neural gi a
The mai n ri sk factor i s i ncreasi ng age
Neuropathic pain secondary to other conditions
Faci al pai n and headache may be symptoms of a wi de vari ety of i ntra- and extracrani al l esi ons
such as neopl asms or vascul ar abnormal i ti es. Faci al pai n may ari se through i nvol vement ei ther
by pressure or by mal i gnant i nfi l trati on of the tri gemi nal gangl i on of the peri pheral branches
of the nerve. Pri mary and secondary i ntracrani al neopl asms, nasopharyngeal tumours,
aneurysms, and cerebral epi dermoi d cysts are among the most commonl y recorded l esi ons of
thi s type causi ng faci al pai n. Si mi l arl y, the l esi ons l eft fol l owi ng brai n damage of any ki nd
may be the source of faci al pai n. In cases of thi s type, faci al pai n i s onl y rarel y the onl y
symptom, al though i t may wel l be the earl i est. Invol vement of other crani al nerves and the
presence of unusual symptoms such as anaesthesi a or paraesthesi a shoul d at once rai se the
suspi ci on of the possi bi l i ty of a l esi on of thi s ki nd.
Another cause of faci al pai n ari si ng from compression of the tri gemi nal nerve occurs i n Paget' s
di sease aff ecti ng the base of the skul l (see Chapter 18). Some degree of cl osure of the
forami na i s common, and the consequent stri cture of the nerve may l ead to a vari ety of pai n
symptoms. A further cause of trauma to the tri gemi nal nerve i n Paget' s di sease may be the
deformati on of the skul l as a whol e, causi ng compressi on of the sensory root of the nerve
where i t crosses the petrous bone. The pai n often si mul ates tri gemi nal neural gi a, al though
achi ng pai ns may someti mes be reported. A si mi l ar compressi on of the audi tory nerve may
gi ve ri se to deafness, and changes i n the cervi cal spi ne may al so l ead to pai n i n the area
served by the sensory nerves of the cervi cal pl exus.
Migraine
There are many vari ants of mi grai ne and a ful l descri pti on of the di f ferent types i s outsi de the
scope of oral medi ci ne. The denti st shoul d, however, be fami l i ar wi th the symptoms of
mi grai ne. Pati ents wi th mi grai ne and cl uster headache (mi grai nous neural gi a) occasi onal l y
present i n the oral medi ci ne cl i ni c. Al though mi grai ne i s essenti al l y a headache, i t may al so
have a f aci al component, usual l y over the maxi l l a, and thi s may cause confusi on. In most
cases, however, the pati ents compl ai n of an i ntense headache, i ntermi ttent i n nature and
usual l y (but not i nvari abl y) uni l ateral hence the term hemi crani a. Each attack of
headache may l ast for several hours or even a

number of days, and i s often associ ated wi th vari ous symptoms such as nausea and vi sual
di sturbance. The systemi c upset i s consi derabl e and many pati ents need to take bed rest i n a
qui et dark room unti l the attack subsi des. In cl assi c mi grai ne pati ents have an aura where the
headache i s preceded by neurol ogi cal symptoms and defi ci tsvi sual , sensory, speech, or
motor di sturbances may occur and usual l y l ast around 15 mi nutes. Mi grai ne wi thout aura i s,
however, wel l recogni zed. The hi story of the condi ti on wi th i ts associ ated di sturbances often
gi ves a strong cl ue to i ts nature, as does the frequent presence of a fami l y hi story. Some
pati ents recogni ze a preci pi tati ng factorsometi mes of stress, someti mes of a foodstuf f. The
onset of mi grai ne may occur at any age, but i s most common duri ng adol escence. In mi grai ne
there are no symptoms between attacks.
The cause of mi grai ne has general l y been attri buted to i nstabi l i ty of the crani al arteri es. The
response of mi grai ne to drug therapy i s vari abl e. Si mpl e anal gesi cs, anti -emeti cs, propranol ol ,
and ergotami ne can be successful . Fi rst-l i ne drug treatment for the acute phase shoul d be a
si mpl e anal gesi c such as paracetamol , aspi ri n, or a NSAID. An anti -emeti c may be hel pful i n
some pati ents when vomi ti ng i s a probl emthe admini strati on of drugs by supposi tory i s
benefi ci al for such pati ents. 5-hydroxytryptami ne (5-HT
1
; serotoni n) agoni sts (for exampl e,
sumatri ptan or newer anal gesi cs) may be gi ven by the oral , i ntranasal , or subcutaneous route.
Hi stori cal l y, the most wi del y used group of drugs have been deri ved f rom ergot, these are now
best avoi ded i n vi ew of thei r powerful si de-effects.
Mi grai ne prophyl axi s may be val uabl e f or pati ents who have two or more severe attacks each
month. Beta-bl ockers, such as propranol ol , and 5-HT
1
antagoni sts (Pi zoti fen) may be
benefi ci al . Methysergi de i s used onl y occasi onal l y because of the ri sk of unwanted si de-
effects, i ncl udi ng the very seri ous one of retroperi toneal fi brosi s. Methysergi de shoul d onl y be
admi ni stered under hospi tal supervi si on. Sodi um valproate and tri cycl i c anti depressants may
be consi dered. Cl oni di ne, a drug that has been used i n l arger doses for the treatment of
hypertensi on and whi ch has a vascul ar stabi l i zi ng eff ect, has been used for the control of
mi grai nous attacks. Thi s drug has consi derabl e si de-effects, i t may aggravate depressi on and
produce i nsomni a, and i s not now recommended routi nel y for prophyl axi s of mi grai ne.
The di agnosi s of mi grai ne i s made on basi s of the cl i ni cal hi story, but a physi cal exami nati on
of the pati ent and a caref ul assessment of the hi story i s requi red to excl ude other causes of
headache. There i s no evi dence that occl usal probl ems cause mi grai ne. The use of a nocturnal
acryl i c occl usal spl i nt, whi ch si gni fi cantl y i ncreases the verti cal occl usal di mensi on, has been
advocated by some to hel p pati ents who wake up i n the morni ng wi th mi grai ne or devel op
mi grai ne shortl y after waki ng but the evi dence i s not based on randomi zed control l ed tri al s.
P. 184
Pai n of a mi grai nous type may al so be associ ated with Mel kersson Rosenthal syndrome (see
Chapter 12).
Cluster headache (periodic migrainous
neuralgia/migrainous neuralgia)
Thi s condi ti on, cl osel y associ ated wi th mi grai ne, may al so appear as a di agnosti c probl em i n
the oral medi ci ne cl i ni c or i n the dental surgery. The aeti ol ogy of thi s condi ti on i s thought to
be vascul ar changes at the base of the skul l . Whi l st many features of the pai n may be si mi l ar
to those of cl assi cal mi grai ne, there are di ff erences (Tabl e 15. 7). The pati ents are much more
commonl y young mal es and they may experi ence 18 attacks per day. Al cohol and
vasodi l ators may preci pi tate attacks, but they al so occur spontaneousl y. Cl uster headache i s
l ess common than mi grai ne. Duri ng an attack there are repeated epi sodes of pai n, usual l y of
about 30 mi nutes i n durati on, wi th a vari abl e i nterval between. The pai n i s usual l y l ocated
behi nd and around the eye, wi th extensi ons to the maxi l l a and to the temporal area. The pai n
l asts for 30120 mi nutes and i s usual l y accompanied by vascul ar changes. Autonomi c
symptoms are often present, such as weepi ng of the eye on the affected si de and congesti on
of the nasal mucous membranes. The attacks often occur at regul ar ti mes at ni ght and di sturb
sl eephence the pseudonym al arm cl ock pai n. Spontaneous remi ssi on can occur
after several months of attacks. The characteri sti c groupi ng

of attacks of pai n over a short peri od fol l owed by l ong peri ods of remi ssi on has l ed to the
term cl uster headache. Some pati ents do not have these epi sodi c occurrences and
they are then di agnosed as havi ng chroni c cl uster headaches.
P. 185
Table 15.7 Comparative features of migraine and cluster
headaches
Migraine Cluster headaches
Age and gender
Sl i ghtl y more common i n femal es, can
occur at any age, mai nl y
Mai nl y young mal es, > 20 years
2nd3rd decades
Si te
Usual l y uni l ateral temporal , occupi tal , or
frontal muscl es
Uni l ateral , orbi tal regi on
Nature of pai n and i ntensi ty durati on
Severe throbbi ng, pul sati ng conti nuous,
hoursdays (472 h) usual l y day
ti me. Up to 12 attacks per month
Severe bori ng epi sodi c 30180 mi n,
often at ni ght. Can be several attacks
per day.
The pai n often responds to anti mi grai ne drugs i n the same way as mi grai ne i tsel f but
subcutaneous i njecti on of sumatri ptan i s thought to be the treatment of choi ce duri ng an
attack. There are very cl ear di agnosti c cri teri a for thi s condi ti on, whi ch has l ed to hi gh-qual i ty
mul ti centre randomi zed control l ed tri al s of therapy.
Characteristics of cluster headaches
Mal e to f emal e rati o, 9:1
Predi l ecti on for young mal es
Epi sodi c severe pai n of 30 mi n to 3 hour durati on that can wake a pati ent f rom sl eep
Centred over orbi tal regi on
Accompani ed by l acri mati on and nasal congesti on
Al cohol may provoke pai n
Tension-type headache
Tensi on-type headache i s now the term recommended to descri be a vari ety of poorl y defi ned
condi ti ons, such as tensi on headache, stress headache, and muscl e
contracti on headache, that are very common. Tensi on-type headaches are characteri zed
by pai n that i s mi l d to moderate i n severi ty, and the di stri buti on i s bi l ateral . The compl ai nt i s
one of a feel i ng of ti ghtness, constri cti on, or pressure. The pai n may affect the frontal ,
occi pi tal , and temporal muscl es. The headache may be epi sodi c or chroni c and i s not
Preci pi tati ng f actors
Stress, foodstuffs for some pati ents Al cohol for some pati ents
Prodromal symptoms
Aura-phase. Common neurol ogi cal
symptoms (e.g. vi sual di sturbances,
speech probl ems, numbness)
Aura phase i s uncommon
Associ ated features
Fami l y hi story common. May be sensi ti ve
to l i ght, smel l s, noi se. vomi ti ng i s
common
Fami l y hi story uncommon. Vascul ar
changes on aff ected si de
(l acri mati on/nasal congesti on)
Treatment
Avoi dance of preci pi tati ng f actors Avoi dance of preci pi tati ng factors.
NSAIDs, 5HT
1
agoni sts.
Sumatri ptan, NSAIDs
accompani ed by systemi c di sturbance or neurol ogi cal si gns.
The causes of tensi on-type headache are poorl y understood, but muscul ar tensi on and stress
are thought to be i mportant. Overwork, fati gue, an emoti onal cri si s, and menstruati on are
common preci pi tati ng f actors. It i s worth noti ng that tensi on-type headaches do not usual l y
wake the pati ent from sl eep. Si mi l ar symptoms may be experi enced by pati ents who have
drug-rel ated headaches, hypertensi on, or hyperthyroi di sm. A mi nori ty of pati ents may suffer
from both tensi on-type headache and mi grai ne.
Treatment of tensi on-type headaches i nvol ves avoi dance of tri gger factors such as al cohol ,
i mprovi ng sl eep patterns, taki ng more exerci se, or usi ng rel axati on techni ques or other
stress-copi ng strategi es. Drug treatments consi st of the i ntermi ttent use of si mpl e anal gesi cs
such as aspi ri n, paracetamol , or NSAIDs. Caffei ne-contai ni ng drugs shoul d be avoi ded. Most
pati ents wi th epi sodi c tensi on-type headaches have short-l asti ng attacks and respond wel l to
si mpl e anal gesi cs. However, around 23 per cent of sufferers have chroni c tensi on-type
headaches wi th i ntractabl e symptoms that are resi stant to si mpl e anal gesi cs. Some of these
are potenti ated by overuse of anal gesi cs. The use of tri cycl i c anti depressants may be hel pful
but, i deal l y, shoul d be used i n conjuncti on wi th cogni ti ve and behavi oural approaches.
Facial pain: miscellaneous conditions
Two condi ti ons that shoul d be menti oned because they may very occasi onal l y cause faci al pai n
are temporal arteri ti s and auri cul otemporal syndrome (Frey' s syndrome). It i s i mportant that
the denti st i s aware of the former l esi on because earl y di agnosi s can prevent seri ous
compl i cati ons.
Giant cell arteritis (temporal arteritis/cranial arteritis)
Gi ant cel l arteri ti s i s an i nfl ammatory condi ti on that typi cal l y af fects the wal l s of medi um-
si zed arteri es. The l umen of the vessel becomes compromi sed and, as a consequence,
i schaemi a of the ti ssues suppl i ed by the artery may occur. Gi ant cel l arteri ti s can affect many
parts of the body (pol ymyal gi a rheumati ca), but the head i s often i nvol ved. When the
temporal artery i s i nvol ved i t i s cal l ed temporal or crani al arteri ti s.
Temporal arteritis
In temporal arteri ti s the pai n i s l ocal i zed to the temporal and frontal regi onsthi s
corresponds to the area suppl i ed by the superfi ci al temporal artery. The pai n i s descri bed as a
severe throbbi ng ache, but paroxysmal pai n i s al so occasi onal l y descri bed. In between attacks
of pai n the affected area may be very tender to the touch and the temporal artery may be
tortuous, pronounced. The pai n may be i ni ti ated by eati ng and, as a consequence, wei ght l oss
may ensue. The masti catory pai n i s due to i schaemi a of the muscl es and shoul d be
di fferenti ated from temporomandi bul ar pai n di sorders. The pati ents, most of whom are i n
ol der age groups and predomi nantl y femal e, al so compl ai n of general mal ai se and di ffuse
muscul ar and joi nt pai ns. There may al so be degenerati on of vi si on because of i nvol vement of
the reti nal artery and subsequent i schaemi a of the opti c nerve. The essenti al pathol ogy of thi s
condi ti on i s general i zed i nfl ammati on wi th an i nfi ltrate of gi ant cel l s. Thi s condi ti on can aff ect
other vessel s i n the head and neck (for exampl e, l ingual arteri ti s).
It i s i mportant that thi s condi ti on i s recogni zed and treated as earl y as possi bl e, because i t
may otherwi se progress to cause i rreversi bl e damage to the opti c nerve. Pati ents shoul d
therefore be assessed on an emergency basi s. Di agnosi s i s made on cl i ni cal grounds, l argel y
dependent on the evi dentl y enl arged and pai nful artery and on el evated erythrocyte
sedi mentati on rate (ESR). The ESR i s markedl y rai sed (over 100 mm i n 1 hour) and C-reacti ve
protei n (CRP) l evel s may al so be el evated. A normal ESR vi rtual l y rul es out temporal arteri ti s.
Absol ute di agnosi s depends on the fi ndi ng of a typical gi ant-cel l i nfl ammatory process i n a
bi opsy of the affected vessel , whi ch shoul d onl y be done i n pati ents who have a rai sed ESR.
Treatment i s wi th hi gh doses of systemi c steroi ds, of the order of 60 mg of predni sol one dai l y,
unti l

symptoms di sappear. Pati ents are mai ntai ned on a l ower dose of steroi ds for up to 2 years.
Characteristics of temporal (giant cell) arteritis
A vascul ar di sease pri mari l y affecti ng el derl y peopl emean age 70 years
Sudden-onset pai n or headache that may be i ni ti ated or exacerbated by eati ng
Bl i ndness i s a recogni zed compl i cati oni t i s therefore i mportant to promptl y di agnose
and treat thi s condi ti on
The ESR i s abnormal l y hi gh; arteri al bi opsy may demonstrate granul omatous arteri ti s
Di f ferenti al di agnoses: temporomandi bul ar joi nt di sorders: tri gemi nal neural gi a
Treat wi th hi gh-dose systemi c steroi ds
Auriculotemporal syndrome (Frey's syndrome)
The auri cul otemporal syndrome i s caused by damage to the auri cul otemporal nerve as i t
passes through the substance of the paroti d gl and i n the earl y part of i ts course. It i s a rare
condi ti on that may be the resul t of a wi de range of pathol ogi cal processes wi thi n the gl and,
both i nfl ammatory and neopl asti c, or of surgi cal trauma (postgustatory neural gi a). The
symptoms are of erythema and sweati ng i n the cutaneous di stri buti on of the auri cul otemporal
nerve. The symptoms are usual l y i n response to gustatory sti mul i . In approxi matel y 10 per
cent of cases the condi ti on i s accompani ed by pai n i n the di stri buti on of the auri cul otemporal
nerve. The pai n i s of ten descri bed as burni ng i n nature. Between attacks of pai n there
may be anaesthesi a or paraesthesi a of the ski n i n the affected area. The term gustatory
neural gi a has been proposed for thi s pai n. No effecti ve treatment has been i denti f i ed f or
thi s condi ti on.
Frey' s syndrome i s a recogni zed compl i cati on of surgi cal trauma (for exampl e, paroti d gl and
surgery and temporomandi bul ar joi nt surgery), but i t can al so be due to acci dental trauma,
l ocal i nfecti on, sympatheti c dysfuncti on, and paroti d pathol ogy. It i s evi dent, therefore, that,
once the condi ti on i s recogni zed, conf i rmati on of the di agnosi s and subsequent treatment
depend on a ful l i nvesti gati on of the i nvol ved paroti d gl and. The topi cal appl i cati on of an
anti perspi rant may be hel pf ul i n control l i ng the sweati ng.
Neurological disturbances
Facial nerve deficits
Def i ci ts of the seventh crani al nerve (faci al nerve) may resul t from a central or peri pheral
cause and i t i s essenti al to di sti ngui sh between these two types of neurol ogi cal l esi on.
Characteristics of Frey's syndrome
Fl ushi ng and sweati ng i n the cutaneous di stri buti on of the auri cul otemporal nerve
fol l owi ng a gustatory sti mul us
Pai n i s uncommon (l ess than 10 per cent of cases)
Trauma, l ocal i nfecti on, l ocal surgery, sympatheti c dysfuncti on, and central nervous
system di sorders are possi bl e aeti ol ogi cal factors
1. Upper motor neurone l esi ons are central l esi ons that affect the nerve fi bres responsi bl e
for movement i n the l ower faci al muscl es. In such a l esi on, the muscl es over the maxi l l a
and mandi bl e are weakened, but those of the forehead are unaffected. Thi s i s because
P. 186
the motor nerves to the muscl es of the forehead are regul ated by both cerebral
hemi spheres, wi th free crossi ng of nerve fi bres. In contrast, the muscl es of the l ower
part of the face are i nnervated onl y by nerves control l ed i n the opposi te hemi sphere. In
these l esi ons the emoti onal movements of the whol e of the face may be unaffected si nce
these are i ni ti ated by upper motor neurones separated from the pyrami dal motor fi bres
that serve the faci al muscl es f or normal functi on.
2. Lower motor neurone l esi ons affect both the upper and l ower f aci al muscl es. The
forehead and f ace are equal l y affected. In these cases the pathol ogi cal process i s
peri pheral to the faci al motor nucl eus i n the pons. The most common cause of such a
paral ysi s i s Bel l ' s pal sy (di scussed bel ow) but a wi de vari ety of l esi ons af fecti ng the
faci al nerve bel ow the base of the skul l (i ncl udi ng some i n the paroti d gl and) may be
responsi bl e for symptoms. Emoti onal movements are al so l ost i n the area si nce the
l esi on responsi bl e affects the common path of al l the peri pheral fi bres.
Peri pheral paral ysi s i s subdi vi ded i nto i ntrapetrosal and extrapetrosal l esi ons. When the l esi on
affects the faci al nerve i n the i ntrapetrosal tract, the chorda tympani and stapedi us nerve are
al so i nvol ved. There may, therefore, be a heari ng def i ci t (hyperacusi a) and l oss of taste on
the anteri or two-thi rds of the tongue on the affected si de. Si nce the chorda tympani i s gi ven
off wi thi n the f aci al canal at the base of the skull , l esi ons occurri ng bel ow thi s wi l l not affect
the sense of taste.
The nerves responsi bl e for motor functi on to the face are shown i n Tabl e 15. 8.
Anaesthesia and paraesthesia
The onset of anaesthesi a or paraesthesi a, ei ther slowl y or suddenl y, i n the di stri buti on of a
nerve may have l ocal i zed or general i zed si gni fi cance; Tabl e 15. 9 l i sts possi bl e condi ti ons that
may cause orofaci al anaesthesi a or paraesthesi a.
Local causes of anaesthesi a i n branches of the tri gemi nal nerve i ncl ude di rect trauma to the
nerve (as i n fractures, traumati c

extracti ons, sagi tal spl i t osteotomi es), pressure on the nerve by a forei gn body (such as a
tooth root or root canal medi cament), i nfl ammatory changes i n rel ati on to the nerve
(especi al l y osteomyel i ti s), and neopl asms encroaching on the nerve. More general i zed
neurol ogi cal condi ti ons that may affect the tri geminal nerve i n thi s way i ncl ude mul ti pl e
scl erosi s and the neuropathy of mi xed connecti ve tissue di sease (Chapter 11). Si mi l ar changes
may occur as the resul t of a vari ety of i ntracrani al space-fi l l i ng l esi ons such as an acousti c
neuroma (Fi g. 15. 2)
P. 187
Table 15.8 The nerves responsible for motor control of
the head and neck
Number Cranial nerve Muscles
V Tri gemi nal Masti catory muscl es
VII Faci al Muscl es of f aci al expressi on
IX Gl ossopharyngeal Uvul a, soft pal ate, base of tongue, pharynx
Characteristics of upper motor neurone disease
A central l esi on that causes parti al paral ysi s of the faci al muscl es
The l ower f aci al muscl es, contral ateral to the l esion, are paral ysed
The orbi cul ari s ocul i and frontal i s muscl es, whi ch recei ve bi l ateral corti cal f i bres, have
l i mi ted functi on
The bl i nk refl ex and movement of f orehead muscl es unaff ected
Characteristics of lower motor neurone disease
A l esi on affecti ng the faci al nerve al ong i ts i ntra- or extrapetrosal course
There i s paral ysi s of the faci al muscl es around the eye, forehead, and mouth, the bl i nk
refl ex i s l ost, and drool i ng i s common
There may be taste and heari ng defi ci ts i f the l esion i s si tuated wi thi n the i ntrapetrosal
tract
Causes of facial palsy
XI Accessory Trapezi us
XII Hypogl ossal Tongue
Intracrani al causes Extracrani al causes (i ncl udi ng i ntrapetrosal l esi ons)
Cerebrovascul ar acci dents Incorrect pl acement of l ocal anaestheti c
Cerebral tumours Iatrogeni c (fol l owi ng resecti on of tumour)
Neurol ogi cal di seases Paroti d tumour
Mul ti pl e scl erosi s Heerfordt' s syndrome
Head i njuri es Mel kerssonRosenthal syndrome (Chapter 12)
Ramsey Hunt syndrome (Chapter 4)
Table 15.9 Conditions that may cause orofacial
anaesthesia or paraesthesia
Fol l owi ng a cerebrovascul ar acci dent there may be a range of neurol ogi cal abnormal i ti es
affecti ng the face and mouth. Among the more di ffi cul t to deal wi th are the changes i n
sensati on, propri ocepti on, and muscul ar control , whi ch may make denture-weari ng very
di ffi cul t for the affected pati ent.
Fol l owi ng a cerebrovascul ar acci dent there may be a range of neurol ogi cal abnormal i ti es
affecti ng the face and mouth.
Bell's palsy
Bel l ' s pal sy i s the name gi ven to an acute paral ysis of the faci al nerve. It i s practi cal l y al ways
uni l ateral and no obvi ous cause i s found i n the vast majori ty of cases. It i s thought that some
cases

may have a vi ral aeti ol ogy; possi bl y herpes si mpl ex vi rus. Bel l ' s pal sy i s the most common
l ower motor neurone l esi on and, dependi ng upon the severi ty of the condi ti on, some or al l of
the muscl es of expressi on on the affected si de may be weakened or paral ysed. The extent of
the paral ysi s i s easi l y seen i f the pati ent i s asked to smi l e, to cl ose the eyes, and to furrow
the brow. There may or may not be l oss of taste sensati on on the anteri or l ateral part of the
tongue on the affected si de, dependi ng on whether or not the chorda tympani nerve i s
i nvol ved. Approxi matel y hal f the pati ents compl ai n of severe pai n i n the area of the paroti d
gl and or the earspreadi ng down the mandi bl ealthough thi s general l y precedes the
paral ysi s or occurs i n the earl y stages of the condi ti on. The faci al appearance i s characteri sti c.
The i mpai red f uncti on of the seventh crani al nerve can be demonstrated by aski ng the pati ent
to smi l e, cl ose thei r eyes, and attempt to purse thei r l i ps. The af fected si de wi l l be unabl e to
perform these acti ons.
Local Systemic
Local anaestheti c Mul ti pl e scl erosi s
Trauma Intracrani al l esi ons
Resorpti on of the al veol ar ri dge exposi ng mental
foramen
Hyperventi l ati on/tetany
Medi cati on
Psychogeni c
Infecti on (osteomyel i ti s, herpes zoster)
Mal i gnanci es
P. 188
Prompt di agnosi s and treatment of Bel l ' s pal sy may prevent f uncti onal and aestheti c
i mpai rment.
One currentl y recommended treatment regi me for Bel l' s pal sy i s a combi nati on of systemi c
steroi ds and aci cl ovi r. The anti vi ral i s advocated because of the possi bl e i nvol vement of the
herpes si mpl ex vi rus. Aci cl ovi r i s gi ven at a dose of 400 mg, four ti mes dai l y. Steroi ds are
admi ni stered i n hi gh dosage60 mg predni sol one dai l y for 5 days, then reduci ng the dose
over the same peri od of ti me, i s a reasonabl e regi me. The rati onal e behi nd thi s approach i s
unproven, but i t i s thought that steroi ds may be advantageous by reduci ng oedema around the
faci al nerve. Some workers have suggested that i t needs to be i nsti tuted wi thi n the fi rst
2472 hours of onset of the Bel l ' s pal sy. Others suggest that i t shoul d be adopted onl y i f
spontaneous resol uti on does not take pl ace. There i s no concl usi ve evi dence from randomi zed
control l ed tri al s that steroi ds or anti vi ral agents, gi ven al one or i n combi nati on, provi de any
l ong-term benefi t for pati ents wi th Bel l ' s pal sy. Nonethel ess, i t i s the authors vi ew that,
unl ess there are si gni fi cant contrai ndi cati ons to steroi d therapy, i t shoul d be started wi th a
systemi c anti vi ral agent at the earl i est opportuni ty after di agnosi s. Thi s i s i n an attempt to
avoi d the possi bi l i ty of l ong-term cosmeti c deformity that accompani es an unresol ved faci al
pal sy. It i s i mportant to protect an eye that remains parti al l y open, ei ther by an eye pad or
shade.
Management of Bell' s palsy
Ini ti al hi gh-dose predni sol one, reduci ng after 57 days
Systemi c anti vi ral therapy
Fig. 15.2 Magneti c resonance scan showi ng a mass i n the cerebel l oponti ne angl e.
(Pati ent presented wi th faci al numbness)
Ensure adequate eye protecti on
Most pati ents wi th Bel l ' s pal sy recover wi thi n a few weeks, but f or the chroni c cases
supporti ve or correcti ve treatment may be necessary. A si mpl e spl i nt or a modi f i cati on to an
exi sti ng denture may hel p support the soft ti ssues and i mprove the faci al prof i l e of the
affected si de. Referral to physi otherapy departments for el ectri cal sti mul ati on of the paral ysed
faci al muscl es may al so be hel pful . Gal vani c sti mul ati on i s consi dered to be worthwhi l e
because i t sti mul ates muscl e contracti on, possi bl y promotes motor end-pl ate functi on, and i s
of psychol ogi cal benefi t. Hand-hel d, pati ent-operated smal l nerve sti mul ators are now
avai l abl e for thi s purpose. Surgi cal treatment may be consi dered i n some pati ents for cosmeti c
reasons, but there i s l i ttl e evi dence that thi s approach i s successful . Faci al pal sy as a
mani festati on of the Mel kerssonRosenthal syndrome i s ful l y di scussed i n Chapter 12.
Multiple sclerosis
Mul ti pl e scl erosi s (di ssemi nated scl erosi s) i s a disease featuri ng progressi ve demyel i nati on of
nervous ti ssue wi th epi sodes of rel apse and remi ssi on. Thi s resul ts i n permanent and
i ncreasi ng di sabi l i ty. The condi ti on affects mai nl y young adul ts, i t i s sl i ghtl y more preval ent i n
femal es, and the usual age of onset i s 2040 years. The di sease can present wi th a wi de
range of symptoms (Tabl e 15. 10) dependi ng upon the si tes of l esi ons i n the brai n. Di agnosi s
of the condi ti on depends upon cl i ni cal features, magneti c resonance i magi ng (to demonstrate
the central areas of demyel i nati on), and excl usi on of other neurol ogi cal defi ci ts.
Pati ents wi th mul ti pl e scl erosi s (MS) may present wi th pai n resembl i ng tri gemi nal neural gi a.
Thi s can occur at any stage of the di sease and i t can be a presenti ng feature. If tri gemi nal
neural gi a i s the fi rst mani festati on of MS, the pati ents are often younger than the tri gemi nal
neural gi a popul ati on as a whol e and thei r neural gi a i s often bi l ateral .
The cl i ni ci an shoul d, however, consi der the possi bil i ty of mul ti pl e scl erosi s when a pati ent
younger than 50 years ol d presents wi th symptoms suggesti ve of tri gemi nal neural gi a. It i s
i mportant, however, that thi s suspi ci on i s not voi ced to the pati ent unti l they have been ful l y
assessed by a neurol ogi st. Mul ti pl e scl erosi s does not necessari l y gi ve ri se to symptoms that
mi mi c cl assi cal tri gemi nal neural gi ai t may gi ve ri se to persi stent pai n, wi th no i denti fi abl e
tri gger areas. Paraesthesi a and al l odyni a may al so be features of the condi ti on. Mul ti pl e
neurol ogi cal defi ci ts are usual l y present such as muscl e weakness, vi sual di sturbances,

and sensory l oss. The management for the faci al pain i s si mi l ar to that for tri gemi nal
neural gi a.
P. 189
Table 15.10 Common symptoms of multiple sclerosis
Type of symptom Description
Vi sual Loss of vi sual acui ty and col our vi si on and eye pain (due to
opti c neuri ti s); di pl opi a
Weakness of the
l i mbs
Ti ngl i ng or paraesthesi a may be present
Verti go
Mul ti pl e scl erosi s (MS) i s di agnosed i n 24 per cent of pati ents wi th tri gemi nal neural gi a.
Extrapyramidal syndromes
Extrapyrami dal syndromes are neurol ogi cal di sorders that affect pathways other than the
pri nci pal ones concerned wi th vol untary movement. They are characteri zed by abnormal i ty of
muscul ar acti on wi th tremors. Parki nsoni sm i s the best known condi ti on of thi s ki nd.
It has recentl y become evi dent, however, that symptoms of a si mi l ar ki nd may occur fol l owi ng
the use of certai n drugs, especi al l y i n el derl y pati ents. In parti cul ar, sedati ves and
tranqui l l i zers of the phenothi azi ne group have been i mpl i cated. These pati ents may
occasi onal l y come to the dental surgeon for di agnosi s si nce the dyski neti c symptoms are often
most evi dent i n the muscl es of the masti catory apparatus. Thi s can make dental treatment
di ffi cul t. Repeti ti ve movements or tremor of the tongue or mandi bl e, whi ch may be of a
bi zarre nature, shoul d be suspected as havi ng a drug-i nduced ori gi n i n pati ents of thi s ol der
age group. Treatment i s by wi thdrawal of the offendi ng drug, al though resol uti on may take
some ti me.
Extrapyrami dal syndromes are neurol ogi cal di sorders that aff ect pathways other than the
pri nci pal ones concerned wi th vol untary movement.
Case 15.1
Carbamazepi ne i s the fi rst drug of choi ce. A reasonabl e regi me woul d be to commence the
pati ent on 100 mg twi ce dai l y, i ncreasi ng by 100 mg every two to three days unti l the
paroxysms of pai n are control l ed. Cauti on i s requi red i n el derl y pati ents as they may be very
suscepti bl e to the si de-effects. A pai n di ary i s useful to moni tor the effects of the drug.
The pati ent shoul d be warned about the fol l owi ng possi bl e si de-effects: ti redness; di zzi ness;
ataxi a; doubl e vi si on; and i nabi l i ty to concentratepati ents report that they f eel spaced
out or l i ke a zombi e.
Ataxi a
Q1 What i s the usual drug of choi ce for thi s pati ent and what starti ng dose woul d you
prescri be?
Q2 What warni ngs and i nformati on woul d you gi ve to the pati ent?
They shoul d be i nstructed:
not to dri ve whi l st experi enci ng the si de-effects li sted;
to be cauti ous when dri nki ng al cohol ;
to contact the hospi tal or medi cal practi ti oner i f a rash devel ops.
The pati ent shoul d be tol d that the drug i s an anti convul sant and not a conventi onal anal gesi c.
It shoul d, therefore, be taken regul arl y, not on demand l i ke other anal gesi cs and,
preferabl y, 30 mi nutes before a meal . The pati ent shoul d be tol d that they need to be
revi ewed regul arl y, that the dose of anti convul sant may need to be i ncreased, and that bl ood
tests wi l l be requi red. Pai n rel i ef shoul d occur, for the majori ty of pati ents, wi thi n 24 hours,
al though the f ul l therapeuti c benefi t may not be attai ned for 23 weeks.
The pati ent shoul d have bl ood taken so that some basel i ne i ndi cators can be obtai ned (ful l
bl ood count, l i ver functi on tests, and el ectrol ytes; see text).
Carbamazepi ne i s a hepati c mi crosomal enzyme i nducer. Therefore, i f taken concurrentl y wi th
any other drug that i s metabol i zed by the l i ver, a pharmacoki neti c drug i nteracti on wi l l occur.
In thi s case warfari n wi l l be metabol i zed more qui ckl y and, consequentl y, the serum warfari n
l evel s wi l l fal l . Thi s wi l l i nvari abl y reduce the effi cacy of warfari n and the pati ent' s
prothrombi n ti me wi l l be al teredthe i nternati onal normal i zed rati o (INR) wi l l drop. Thi s
coul d i ncrease a pati ent' s suscepti bi l i ty to thromboembol i c sequel ae. The dose of warfari n wi l l ,
therefore, probabl y need to be i ncreased and moni tored whi l st the pati ent i s taki ng
carbamazepi ne and for a short ti me after cessati on of the anti convul sant. Pati ents on warf ari n
carry a warni ng card and thi s wi l l gi ve i nformati on about thei r dose, INR, and a contact
number f or thei r anti coagul ati on cl i ni c. Thi s cl i nic wi l l adjust the pati ent' s warfari n dose i f
necessary.
Case 15.2
The pati ent i s exhi bi ti ng symptoms of a l ower motor neurone pal sy. You need to check that
the si gns are uni l ateral and that the pati ent has not recentl y been experi enci ng faci al
weakness or symptoms of a neurol ogi cal defi ci t, such as paraesthesi a. The l eft-si ded faci al
muscul ature shoul d be checked for functi on to ensure that thi s i s a l ower motor neurone
probl em and that the pati ent i s not suf feri ng from an upper motor neurone l esi on such as a
cerebrovascul ar acci dent; al though thi s woul d be unl i kel y i n thi s parti cul ar case. The most
l i kel y di agnosi s i s a transi ent faci al nerve pal sy resul ti ng from the deposi ti on of l ocal
anaestheti c around the seventh crani al nerve (usuall y i nto or near the paroti d gl and). Faci al
nerve paral ysi s i s evi dent wi thi n mi nutes of the i njecti on and the durati on of faci al weakness
i s usual l y l ess than 12 hours. If a l onger-acti ng local anaestheti c sol uti on such as bupi vacai ne
has been used then resol uti on wi l l be del ayed. It is, however, unl i kel y that bupi vacai ne woul d
be routi nel y used for restorati ve procedures.

The f ol l owi ng acti ons are advi sed.
Reassure the pati ent; expl ai n that i t i s an uncommon but wel l recogni zed compl i cati on of
Q3 What i nvesti gati ons do you need to carry out before starti ng on thi s drug?
Q4 If your pati ent had presented taki ng warfari n, woul d you envi sage any management
probl ems?
Q1 How woul d you manage thi s si tuati on?
P. 190
the anaestheti c techni que that was used.
Use an eye patch over the affected eye to prevent corneal damage (the pati ent shoul d
be weari ng protecti ve gl asses duri ng the treatment).
Assess the general condi ti on of the pati ent and conti nue treatment i f she i s happy to do
so. If the pati ent appears di stressed, the tooth shoul d be tempori zed (further
preparati on may be requi red f or thi s) to ensure that the pati ent wi l l not experi ence any
postoperati ve di scomfort or pai n from the mol ar tooth.
Revi ew next day to ensure that the paral ysi s has resol ved. A tel ephone cal l , i f possi bl e,
l ater that day i s good practi ce.
Wri te the detai l s of the i nci dent cl earl y i n the case notes stati ng what i nformati on was
gi ven to the pati ent and the detai l s f or pati ent fol l ow up.
Projects
1. Descri be how you woul d assess the functi on of the crani al nerves.
2. Descri be the i ntracrani al course of the tri gemi nal and faci al nerves.
3. What i nformati on i s avai l abl e f or pati ents wi th tri gemi nal neural gi a: (a) i n the form of
l eaf l ets; (b) on the i nternet?
4. Acute si nusi ti s can be mi staken for pai n of dental ori gi n; what i s the evi dence for the
effi cacy of anti mi crobi al therapy i n thi s condi ti on?
> Tabl e of Cont ents > 16 - Temporomandi bul ar di sorder s
16
Temporomandibular disorders
Problem cases
Case 16.1
A 20-year-ol d femal e pati ent attends your surgery feel i ng f everi sh and compl ai ni ng of a pai n
over the l eft si de of her jaw. She i s very worri ed because she cannot open her mouth ful l y.
These symptoms have devel oped over the past 24 hours. Exami nati on shows a maxi mum
openi ng of approxi matel y 12 mm.
Case 16.2
A 70-year-ol d edentul ous pati ent presents to your practi ce wi th bi l ateral pai n l ocated over the
temporomandi bul ar joi nt and i n the pre-auri cul ar regi on. There i s no hi story of l ocki ng. He has
been weari ng hi s compl ete dentures sati sfactori l y for 30 years. The pati ent reports that the
pai n has been getti ng gradual l y worse over the past 3 years and thi nks that i t may be due to
arthri ti s. He i s now getti ng a bi t f ed-up wi th the pai n. Hi s daughter wonders i f
he mi ght need new dentures. She comments, hi s mouth seems to have caved i n and he
doesnt l ook as i f he has any teethhe i s al so fi ndi ng i t di f fi cul t to eat. Bi l ateral
crepi tus i s audi bl e over the temporomandi bul ar joi nts and there i s tenderness of the masseter
and temporal i s muscl es on pal pati on. Maxi mum openi ng i s i n excess of 40 mm. The pati ent i s
grossl y overcl osed due to excessi ve occl usal wear on the dentures.
Introduction
Pati ents wi th symptoms from the temporomandi bul ar joi nt (TMJ) are frequentl y seen and
managed by dental practi ti oners. Some pati ents are, however, referred for speci al i st care,
ei ther to oral medi ci ne, maxi l l ofaci al , or restorati ve denti stry departments or to
mul ti di sci pl i nary cl i ni cs that are dedi cated to di sorders of the TMJ. The topi c of TMJ probl ems
has generated a pl ethora of l i terature, but a great deal of confusi on sti l l exi sts as to the
termi nol ogy, aeti ol ogy, and management of TMJ conditi ons. Unfortunatel y, there i s very l i ttl e
objecti ve sci ence to substanti ate most of the publ icati ons.
When anal ysi ng symptoms ari si ng from the TMJ i t shoul d be borne i n mi nd that there are two
di sti nct sources of such symptoms. The fi rst i s from the muscl es, joi nt structures, and other
associ ated ti ssues as a resul t of abnormal physi cal acti vi ty wi thi n the joi nt (dysfuncti on). The
Q1 What are the l i kel y causes for thi s pati ent' s tri smus?
Q2 Li st the possi bl e aeti ol ogi es for acute and chroni c l i mi tati on of mouth openi ng.
Q1 What condi ti ons may be responsi bl e for thi s pati ent' s pai n and what coul d you do, i n
the fi rst i nstance, to hel p you reach a defi ni ti ve di agnosi s?
Q2 There are several causes of pai n that may present i n or adjacent to the
temporomandi bul ar joi nts. Make a l i st of the possi bl e di fferenti al di agnoses (you wi l l
need to use i nformati on f rom other chapters to answer thi s ful l y).
second i s from pathol ogi cal changes i n the joi nt i tsel f and, i n such ci rcumstances, the joi nt
damage may be associ ated wi th a systemi c abnormal i tyrheumatoi d arthri ti s i s an exampl e
of thi s si tuati on. Di fferenti ati on between these may be very di ff i cul t when the pati ent fi rst
presents. The majori ty of cases of pai n and other symptoms ari si ng from the TMJ resul t from a
dysfuncti on syndrome rather than any pri mary pathol ogy of the joi nt.
Temporomandi bul ar joi nt probl ems may be due to:
Investigation of the stomatognathic system
History
A hi story shoul d be taken as previ ousl y descri bed (Chapter 15). Thi s must i ncl ude the
character, i ntensi ty, f requency, durati on,

si te, and radi ati on of any pai n. Exacerbati ng, rel i evi ng, and associ ated f actors shoul d al so be
i denti fi ed. In di sorders of the TMJ the f ol l owi ng four features are very i mportant i n hel pi ng the
cl i ni ci an arri ve at a di agnosi s.
1. Pai n hi story. Pai n may ori gi nate pri mari l y wi thi n the TMJ or from the muscl es of
masti cati onboth arthral gi c and myogeni c pai n may be present. Pai n may be fel t as a
dul l ache over the area of the joi nt, the ear, over the temporal f ossa, or over the
maxi l l a. The pai n may be bi l ateral or uni l ateral and i s usual l y descri bed as bei ng
constant, but wi th acute exacerbati ons. It i s duri ng these acute exacerbati ons that the
radi ati on of the pai n f rom the joi nt often occurs. In some i nstances associ ated pai n i n
the neck, upper arm, occi pi tal area, or al ong the li ngual nerve may be reported. The
severe attacks of pai n occur predomi nantl y i n the earl y morni ng i n some pati ents,
whereas i n other pati ents they are more common at the end of the day. Acute epi sodes
may al so be preci pi tated after a meal , at the wi de openi ng of the mouth, or duri ng the
ni ght when l yi ng heavi l y on one si de of the face.
Muscul ar pai n associ ated wi th temporomandi bul ar pain may cause a headache. It i s
possi bl e that some practi ti oners may confuse the diagnosi s wi th mi grai ne. Si mi l arl y,
pai n ari si ng wi thi n the joi nts themsel ves may be attri buted to earache. Many pati ents
found to have pai nful joi nts wi l l have had an ear exami nati on wi th negati ve resul ts.
2. Joi nt sounds. The pati ent most commonl y compl ai ns of a cl i ck. Thi s cl i ck represents the
movement of one component of the joi nt over the others, and can mean that the di sc i s
sl i ppi ng out of pl ace, sti cki ng, or mal f uncti oni ng. Joi nt sounds are qui te common and are
not al ways si gni fi cant. The presence of a cl i ck does not, on i ts own, i ndi cate that
treatment i s requi red. Cl i cks may be qui te l oud and readi l y audi bl e, and thi s may cause
soci al embarrassment when eati ng. In other cases, however, a stethoscope i s requi red to
hear the sound. Al though an acute epi sode of pai n and cl i cki ng may be preci pi tated by
movement of the TMJ, pati ents may fi nd that pai n i s associ ated wi th peri ods i n whi ch
(1) a dysfuncti on syndrome
(2) pathol ogi cal changes i ncl udi ng:
i nternal derangement
apl asi a, hypopl asi a, hyperpl asi a
arthri ti c condi ti onstraumati c arthri ti s, i nfecti ve arthri ti s, rheumatoi d arthri ti s,
osteoarthrosi s, ankyl osi ng spondyl i ti s, crystal -i nduced arthri ti s (gout), psori ati c
arthropathy
maxi l l ofaci al traumamandi bul ar condyl ar fractures
di sl ocati on
ankyl osi s
neopl asi a
P. 194
cl i cki ng i s mi ni mal . There may be a cl i ck on both openi ng and cl osi ngthi s i s referred
to as a reci procal cl i ck. Apart from the si ngl e l oud cl i ck, other sounds may be heard i n
the joi nt on stethoscopi c exami nati on (auscul tati on). Crepi tus (a grati ng or gravel -l i ke
sound) i s most commonl y heard i n osteoarthrosi s. Care must be taken i n the use of the
stethoscope to el i mi nate the crackl i ng sound produced by l ayi ng the bel l of the
i nstrument over hai r. The resul ti ng crackl i ng sound can easi l y be mi staken for joi nt
crepi tati on.
3. Restri cti on of openi ng. The pati ent may report di ffi cul ty on wi de openi ng, often
associ ated wi th the i mmi nent onset of a l oud cl i ck. In other i nstances the di ffi cul ty may
be i n appl yi ng pressure on cl osi ng the mouth. The inabi l i ty to open the mouth wi de, due
to refl ex muscul ar spasm (contracti on) of the masti catory muscl es, i s cal l ed tri smus and
i s usual l y a temporary rather than permanent condi ti on. Pati ents wi th TMJ probl ems
often compl ai n that thei r jaw l ocks. Thi s may mean that the jaw cannot open normal l y.
It sti cks or l ocks and thi s restri cts openi ng. Thi s i s thought to be due to the di sc
bei ng squashed and bunched up anteri orl y, preventi ng further openi ng. Thi s i s
usual l y termed a cl osed l ock si tuati on and i s mai nl y due to anteri or di sc
di spl acement wi thout reducti on and may fol l ow di sc l axi ty and stretchi ng or possi bl y
teari ng or perforati on i n the posteri or bi l ami nar zone area of the di sc. Al ternati vel y, the
term l ocki ng refers to the mandi bl e temporari l y becomi ng stuck i n an
open posi ti onthe pati ent i s not abl e to cl ose or open the jaw further. Thi s compl ai nt
i s not the same as di sl ocati on. Di sl ocati on refers to the di spl acement of the head of the
condyl e out of the gl enoi d f ossa to a posi ti on anteri or to the arti cul ar emi nence.
4. Swel l i ng. Pati ents wi th TMJ di sturbances occasi onal l y compl ai n of swel l i ng over the
maxi l l a, but there i s no cl ear reason for thi s. A sl i ght degree of soft-ti ssue swel l i ng may
be occasi onal l y noted on exami nati on. In a few other i nstances pati ents may compl ai n of
tenderness and swel l i ng i n the area of the paroti d, presumabl y an ef fect brought about
by the cl ose proxi mi ty of part of thi s gl and to the TMJ. In these cases i t may be a matter
of some di ffi cul ty to di sti ngui sh between paroti d i nvol vement i n joi nt di sturbance or
joi nt i nvol vement i n a paroti d pathol ogy.
Tri smus i s the i nabi l i ty to open the mouth wi de due to refl ex muscul ar spasm
(contracti on) of the masti catory muscl es and i s usual l y a temporary rather than
permanent condi ti on.
Di sl ocati on of the TMJ ref ers to the di spl acement of the head of the condyl e out of the
gl enoi d fossa to a posi ti on anteri or to the arti cular emi nence.
Thorough dental , medi cal , and soci al hi stori es are requi red. Any hi story of trauma to the TMJ
shoul d be expl ored and the outcomes of any previ ous treatments, i f any, shoul d be
ascertai ned. It i s al so i mportant to i denti fy i f the pati ent suffers from other pai n syndromes,
and i f they have hi gh l evel s of emoti onal stress. (Chapter 17 descri bes the assessment of
psychol ogi cal factors i n some detai l . )
Examination
The cl i ni ci an shoul d have a sound knowl edge of the f uncti onal anatomy of the TMJ and
associ ated structures pri or to undertaki ng an exami nati on of the pati ent. Exami nati on of the
TMJ and masti catory muscl es shoul d begi n by observi ng the degree of symmetry of the
mandi bl e and f ace, and by observi ng the path of excursi on of the mandi bl e on openi ng and
cl osi ng. It i s hel pful to focus on a speci fi c l andmark (such as the mesi al i nci sal edge of a

mandi bul ar central i nci sor) whi l st aski ng the pati ent to open and cl ose thei r mouthi n thi s
P. 195
way any l ateral devi ati on wi l l be noted. The amount of mandi bul ar openi ng shoul d be
recorded. An i nter-i nci sal openi ng of approxi matel y 3545 mm i s wi thi n the normal range.
Loud joi nt sounds may be heard duri ng mandi bul ar functi on. In order to exami ne the joi nt by
pal pati on the exami ner shoul d be i n front of the pati ent so that movement of the mandi bl e
may be rel ated to those pal pated i n the condyl ar heads. A si ngl e fi nger i s pl aced over each
condyl ar head whi l e the mandi bul ar movements are carri ed out. Abnormal tenderness
associ ated wi th the l ateral aspect of the joi nts i s detected by l i ght pressure over the condyl e
and the i mmedi ate pre-auri cul ar regi on. Exami nati on of the posteri or joi nt can be undertaken
by i ntra-auri cul ar (i ntra-meatal ) pal pati onthe li ttl e fi ngers are posi ti oned i n the external
audi tory meatus fol l owed by the gentl e appl i cati on of forward pressure. Fai nt joi nt sounds
may be heard by usi ng a stethoscope pl aced over the condyl e head whi l e mandi bul ar
movements are performed.
The cl i ni ci an shoul d record the amount of mandi bul ar openi ng. An i nter-i nci sal openi ng of
approxi matel y 3545 mm i s wi thi n the normal range.
Muscul ar tenderness associ ated wi th joi nt di sturbance may be detected by pal pati on of the
masseter and temporal i s muscl es. The reader must be cogni zant of the ori gi n and i nserti ons of
the masti catory muscl es. Cl i ni cal exami nati on of the masseters i s carri ed out by aski ng the
pati ent to cl ench the teeth f i rml y together and palpati ng a muscl e manual l y. When the
masseters are contracted, the exami ni ng fi nger i s run up the anteri or muscl e border
i ntraoral l y, counterpressure bei ng exerted from the external surface. When the exami ni ng
fi nger reaches the zygomati c ori gi n of the masseter, tenderness becomes evi dent and i s shown
by the pati ent' s reacti on. A si mi l ar test shoul d be carri ed out on the opposi te si de. The
temporal ori gi n of the temporal i s can be assessed extraoral l y when the pati ent i s cl enchi ng,
but the i nserti on of the tendon i s fel t i ntraoral l y by runni ng the l i ttl e fi nger up the anteri or
border of the mandi bul ar ascendi ng ramus. It i s not practi cal to di rectl y pal pate the medi al
and l ateral pterygoi d muscl es. The l ateral pterygoid muscl es can, however, be assessed by
appl yi ng l ateral pressure to the mandi bl e and aski ng the pati ent to resi st the force appl i ed.
Spasm of thi s muscl e wi l l el i ci t pai n or tenderness i n the pre-auri cul ar regi on.
Muscul ar tenderness associ ated wi th joi nt di sturbance may be detected by pal pati on of the
masseter and temporal i s muscl es.
Dentition
Fol l owi ng the exami nati on of the TMJ and associ ated muscl es, a careful dental exami nati on
shoul d be undertaken. The cl i ni ci an shoul d i denti fy and treat orodental pathol ogy that may be
a source of pai n. The occl usal rel ati onshi p of the teeth, both stati c and dynami c, i s recorded.
Centri c occl usi on (CO) and centri c rel ati on (CR) shoul d be i denti fi ed and thei r rel ati onshi p
noted. In the majori ty of pati ents these posi ti ons are not coi nci dent. There shoul d be a smal l
(1 mm) anteri or sl i de from CR to CO. Large sl i des shoul d be noted, parti cul arl y i f the sl i de i s
i n a l ateral di recti on. Occl usal i nterferences shoul d be i denti fi ed duri ng mandi bul ar excursi ons
and the presence of faceti ng, fractured restorati ons, and tooth wear noted. Soft-ti ssue
evi dence suggesti ve of bruxi sm i s ri dgi ng of the buccal mucosa and l ateral borders of the
tongue. Study casts shoul d be taken as a basel i ne record.
Features suggestive of bruxism
Tooth-wear facets that match i n mandi bul ar border (parafuncti onal ) movements
Crenated (pi e-crust) l ateral border of the tongue
Ri dgi ng of the buccal mucosa
Masseteri c hypertrophy
Hi story of repeated fracture of restorati ons
Imaging
The compl ex anatomy of the TMJ has l ed to the devel opment of many radi ographi c vi ews. The
mai n val ue of pl ai n fi l m radi ography i s to i denti f y gross anatomi cal or functi onal changes that
woul d i ndi cate an underl yi ng organi c cause. Earl y bone pathol ogy i s not usual l y detected and
erosi ons must be qui te advanced to be seen on pl ai n fi l ms. A standard panorami c radi ograph
may be hel pful to excl ude dental di sease but thi s is not a good vi ew for showi ng the arti cul ar
surf aces of the TMJ. An open mouth dental pantomograph wi l l show the condyl ar necks
and l ateral vi ew of the condyl ar heads (Fi g. 16.1). The rel ati on of the condyl e heads to the
di sc and fossae are not, however, wel l di spl ayed. Transpharyngeal vi ews al so gi ve the bony
outl i ne of the condyl e and the l ateral aspect of the arti cul ar surface. Transcrani al vi ews, wi th
the mouth open and cl osed, demonstrate condyl ar anatomy and the range of movement i n the
joi nt, i ncl udi ng the si ze of the joi nt space (Fi g. 16.2).

Fig. 16.1 Panorami c vi ew wi th mouth open showi ng condyl e heads and neck.
Fig. 16.2 Transcrani al vi ews of temporomandi bul ar joi nt wi th mouth open, teeth i n
occl usi on, and at rest.
P. 196
Computeri zed tomography (CT) i s useful for i magi ng the hard-ti ssue detai l . Magneti c
resonance i magi ng (MRI) vi sual i zes both soft-ti ssue detai l and bony outl i ne (Fi g. 16.3) as wel l
as bei ng usef ul i n determi ni ng the posi ti on, function, and form of the di sc when the mouth i s
open and cl osed. Thi s i s requi red i n the preoperative assessment pri or to di sc surgery.
Arthrography i nvol ves the i njecti on of a radi o-opaque contrast agent i nto the joi nt space
(usual l y the l ower one) to del i neate arti cul ar surfaces and the di sc. Thi s techni que remai ns
the onl y trul y dynami c study of the joi nt and the most sensi ti ve exami nati on f or i denti fyi ng
di sc perf orati ons. Arthrography i s i ndi cated i n chroni c temporomandi bul ar pai n dysfuncti on
syndrome that has not responded to i ni ti al treatment modal i ti es and al so for persi stent l ocki ng
and l i mi ted mouth openi ng of unknown aeti ol ogy. The techni que i s not, however, al ways wel l
tol erated by pati ents and carri es a rel ati vel y hi gh radi ati on dose. It has now l argel y been
repl aced by MRI.
An open mouth dental pantomograph wi l l show the condyl ar necks and l ateral
vi ew of the condyl ar heads, but the rel ati on of the condyl e heads to the di sc and fossae
are not wel l di spl ayed.
Transpharyngeal vi ews gi ve the bony outl i ne of the condyl e and the l ateral aspect of the
arti cul ar surface.
Transcrani al vi ews, wi th the mouth open and cl osed, demonstrate condyl ar anatomy and
the range of movement i n the joi nt, i ncl udi ng the si ze of the joi nt space.
Arthroscopy
Mi ni -arthroscopy may be an i nvesti gati ve or therapeuti c procedure. Thi s mi ni mal l y i nvasi ve
techni que al l ows for the di rect exami nati on of the upper joi nt space. Lower joi nt space
arthroscopy i s not usual l y performed due to the smal l si ze of the space between the di sc and
the condyl ar head i n compari son to the si ze of the i nstruments used for mi ni -arthroscopy.
Fig. 16.3 Sagi tal magneti c resonance scan showi ng dento-al veol ar compl ex, i ncl udi ng
the temporomandi bul ar joi nt.
Lysi s of the upper joi nt space, l avage, capsul ar distensi on, removal of i ntraarti cul ar adhesi ons
and l oose bodi es, di sc rel ease, and the pl acement of corti costeroi d preparati ons may be
performed wi th arthroscopi cal surgery. On rare occasi ons more extensi ve i ntraarti cul ar
surgery may be undertaken, such as bi opsy and di sc reposi ti oni ng. TMJ arthrocentesi s maybe
carri ed out as an i sol ated procedure, to provi de arti cul ar l ysi s and l avage, but wi thout the
necessi ty of usi ng expensi ve arthroscopi cal equi pment.
Temporomandibular pain dysfunction syndrome (TMPDS)
There are many synonyms used to descri be thi s conditi onmyof aci al pai n dysfuncti on, faci al
arthromyal gi a, faci al pai n dysfuncti on, masti catory muscl e di sorder. The term
temporomandi bul ar pai n dysfuncti on syndrome (TMPDS) wi l l be used i n thi s text. Thi s
condi ti on i s the most preval ent di sorder of the TMJ and aff ects predomi nantl y femal e pati ents.
Epi demi ol ogi cal data suggests that 4080 per cent of pati ents wi th di sorders of

the TMJ are f emal e. In TMPDS the predomi nant compl ai nt i s of pai n, whi ch may take any of
the forms previ ousl y descri bed. Thi s pai n may be associ ated wi th l i mi tati on of openi ng or wi th
joi nt sounds, al so as previ ousl y outl i ned. The pati ents qui te often admi t to a hi story of
psychol ogi cal stress, al though overt psychi atri c abnormal i ty i s unusual .
There may be a hi story of previ ous joi nt cl i cki ng, l i mi tati on of openi ng, trauma, or recurrent
di sl ocati on. Questi oni ng may el i ci t the presence of a habi t that al ters mandi bul ar posi ti oni ng
or acti on. Some pati ents may hol d the mandi bl e i n a parti cul ar posi ti on, usual l y a protrusi on
or l ateral posi ti on, when engaged i n some mental acti vi ty. There may be a hi story of bi ti ng on
some forei gn body, such as a pen or penci l . The pati ent may be aware of gri ndi ng thei r teeth,
but a hi story of bruxi sm i s often suppl i ed by some other member of the pati ent' s fami l y or a
partner. Exami nati on may reveal one or several of the fol l owi ng: l i mi tati on of openi ng of the
mouth; devi ati on of the mandi bl e on openi ng; cl i cking heard or fel t i n the joi nt; gross
mal occl usi on l eadi ng to abnormal joi nt movements, or mi nor degrees of mal occl usi on wi th
abnormal cuspal gui dance of cl osure; gross occl usal attri ti on; occl usal i nterferences;
unsati sfactory dentures; tenderness of the muscl es of masti cati on.
Signs and symptoms associated with TMPDS
Pre-auri cul ar pai n that may radi ate to other si tes
Tenderness of the joi nt on pal pati on
Li mi ted jaw movement or devi ati on of mandi bl e on openi ng and cl osi ng
Tenderness of masti catory muscl es on pal pati on
Joi nt sounds (cl i cks, crepi tus)
Headache
Cl i ni cal l y, there i s tenderness or pai n of the TMJ and muscl es of masti cati onthi s may be bi -
or uni l ateral . Li mi tati on of mandi bul ar openi ng and joi nt sounds are of ten present. In most
pati ents wi th chroni c symptoms, radi ographs of the joi nts reveal no abnormal i ty of structure,
al though l i mi tati on or i ncrease i n joi nt movement may be seen.
The aeti ol ogy of TMPDS i s uncl ear. Many theori es have been postul ated, i ncl udi ng skel etal jaw
rel ati onshi ps, occl usal di sharmoni es, l ack of posteri or teeth, and uni l ateral tooth l oss, but
there i s no cl ear evi dence to support these. Interesti ngl y, rel ati vel y few pati ents wi th
compl ete dentures appear to present wi th TMPDS. Parafuncti onal cl enchi ng and gri ndi ng and
abnormal posturi ng of the jaw have been i mpl i cated as i ni ti ati ng or perpetuati ng factors i n
some pati entsi t i s hypothesi zed that repeti ti ous adverse l oadi ng causes mi crotrauma of the
masti catory system. Psychosoci al factors such as anxi ety and depressi on may predi spose
certai n pati ents to temporomandi bul ar di sorders and may al so serve to perpetuate the
symptoms. In some pati ents there i s a hi story of trauma. Both yawni ng and dental treatment
P. 197
have al so been i mpl i cated. Care must be taken i n differenti ati ng the pai n due to
temporomandi bul ar di sturbances from that ari si ng from dental causes or from faci al pai n of
the types descri bed i n Chapters 15 and 17. In particul ar, the di fferenti al di agnosi s between
faci al pai n of psychogeni c ori gi n and that caused by chroni c temporomandi bul ar dysf uncti on i n
pati ents undergoi ng emoti onal stress i s di ffi cul t. In fact, the two condi ti ons occasi onal l y seem
to merge i n a pati ent wi th physi cal si gns of TMJ dysfuncti on, but wi th the demonstrati ve,
anxi ous, obsessi ve outl ook typi cal of the pati ent converti ng hi dden anxi eti es i nto faci al pai n
symptoms. Some pati ents wi th TMPDS have a hi story of general pai n di sorders. Needl ess to
say, i n such cases, the i ni ti al presumpti ve di agnosi s shoul d be of joi nt dysfuncti on. Onl y when
al l physi cal si gns of TMPDS have been addressed by treatment, shoul d the di agnosi s of
psychogeni c pai n be more fi rml y entertai ned. It i s i n observi ng the reacti on to treatment that
the di fferenti al di agnosi s i s perhaps best made. Irrespecti ve of the aeti ol ogy of chroni c
temporomandi bul ar pai n, the psychol ogi cal response to pai n cannot be i gnored i n the
management of thi s condi ti onChapter 17 expl ores thi s further.
Di sc di spl acement has been i mpl i cated as bei ng i mportant i n the aeti ol ogy of TMPDS.
However, abnormal i ti es i n the l ocati on of the di sc have been noted i n many pati ents wi th and
wi thout TMPDS.
Management
A ful l di scussi on of the management of temporomandibul ar di sorders i s outsi de the scope of
thi s book and thi s i s onl y a bri ef overvi ew. In vi ew of the fact that the aeti ol ogy i s poorl y
understood, i t i s not surpri si ng that the management of thi s condi ti on i s contenti ous. The
range of management opti ons used for TMPDS i s l i sted i n Tabl e 16. 1. Many pati ents wi th
TMPDS are successf ul l y managed i n practi ce by off eri ng an expl anati on for the symptoms and
reassurance, together wi th occl usal appl i ances i f indi cated. Ini ti al management shoul d al ways
be conservati ve, and preferabl y i t shoul d be non-i nvasi ve and reversi bl e. These requi rements
are i mportant for the management of TMJ di sorders because a successful outcome cannot be
ensured. The val ue of reassurance and counsel l i ng cannot be underesti mated. Pati ents shoul d
be tol d about the nature and prognosi s of thi s condi ti on. An i nformati on l eafl et i s hel pful to
rei nforce thi s. A mi nori ty of pati ents wi l l be worri ed that they have a seri ous, even l i fe-
threateni ng, condi ti on and i nf ormati on can al l ay such fears. There may al so be a strong
pl acebo response to the di fferent treatment modal i ti es advocated for TMPDS.
The successf ul management of TMPDS cannot be guaranteed. Therefore the i ni ti al treatment
of TMPDS shoul d be:
non-i nvasi ve
reversi bl e
Many pati ents respond to reassurance ( a removable occl usal appl i ance)
Table 16.1 Spectrum of management strategies for
temporomandibular disorders
Initial (conservative) management Further (specialist) management
Reassurance Psychol ogi cal i nterventi on

The use of occl usal appl i ances, or spl i nts as they are commonl y cal l ed, i s frequentl y hel pful
for some pati ents, and studi es have demonstrated that there i s a si gni fi cant pl acebo eff ect.
Occl usal appl i ances shoul d be removabl e and gi ve ful l occl usal coverage. There i s no pl ace for
the routi ne use of spl i nts that onl y gi ve parti al occl usal coveragesuch spl i nts may cause
unwanted tooth movement. Tabl e 16. 2 hi ghl i ghts some i mportant poi nts concerni ng the
provi si on of occl usal appl i ances, whi l st Tabl e 16. 3 gi ves detai l s of the three spl i nts most
commonl y used for TMJ di sorders.
Jaw exerci ses and vari ous forms of physi otherapy may al l pl ay a part i n treatment. Exerci ses
to correct faul ty patterns of acti vi tysuch as devi ati on on openi ng and cl osuremay be
hel pful . Therapy wi th anal gesi c or rel axant drugs may have a l i mi ted use, but onl y over a
restri cted peri od of ti me. Nonsteroi dal anti -i nf l ammatory drugs (NSAIDs) are the anal gesi cs of
choi ce, provi ded that there are no contrai ndi cati ons to thei r use. The use of benzodi azepi nes
for thei r muscl e rel axant properti es may be hel pful i n acute cases where there i s tri smus. In
addi ti on, thei r hypnoti c acti on can be benefi ci al in ensuri ng sl eep. It i s i mperati ve that onl y
short courses of benzodi azepi nes are used, i deal l y 2 weeks or l ess, because of the potenti al
ri sk of dependence. Benzodi azepi nes are, therefore, onl y of val ue duri ng the acute phase of
TMPDS.
Educati on Occl usal adjustment
Habi t management (e. g. jaw
exerci ses, awareness of dayti me jaw
cl enchi ng)
Occl usal rehabi l i tati on (e.g. restorati ve,
orthodonti c, orthognathi c surgery)
Modi fi cati on of functi on (e. g. chewi ng,
yawni ng, si ngi ng)
Anti depressants
Psychotherapy
Rest Intraarti cul ar steroi ds
Anti -i nf l ammatory agents and
anal gesi cs (l ocal and systemi c)
Mani pul ati on under general anaesthesi a
Muscl e rel axants (e. g. di azepam) Surgery (e. g. arthrocentesi s,
arthroscopy, or open arti cul ar surgery at
arthrotomy)
Occl usal spl i nts (removabl e) Psychi atri c l i ai son cl i ni c/pai n cl i ni c
Physi otherapy
P. 198
Table 16.2 Points to remember when providing removable
occlusal appliances in the management of
temporomandibular disorders
There i s no evi dence that permanent occl usal rehabil i tati on i s of val ue for TMJ di sorders and
such treatment shoul d onl y be undertaken wi th special i st advi ce. In a smal l number of cases
orthodonti c treatment or a combi ned approach usi ng orthognathi c surgery i s i ndi cated. Most
maxi l l ofaci al surgeons woul d onl y perform orthognathi c surgery for a pati ent wi th a
temporomandi bul ar di sorder i f, i n addi ti on to the arti cul ar si gns and symptoms, the pati ent
was havi ng probl ems from the mal occl usi on and underl yi ng skel etal rel ati onshi ps due to
i nadequate functi on or for aestheti c reasons. In a few cases of chroni c TMJ di sorders,
anti depressant drugs, wi th or wi thout psychotherapy, may hel p. TMJ surgery shoul d be
reserved for pati ents wi th a cl earl y i denti fi abl e joi nt abnormal i ty, such as a di spl aced or
damaged di sc. There are other prerequi si tes for surgery. As a general rul e the condi ti on tends
to be sel f-l i mi ti ng and i s not thought to progress to a degenerati ve joi nt condi ti on i n the
majori ty of cases. TMJ surgery shoul d not be carri ed out unti l the fol l owi ng cri teri a have been
met.
1. A mechani cal i ntraarti cul ar joi nt probl em shoul d have been proven by the use of
speci al i zed i magi ng, such as MRI.
2. Al l exhausti ve nonsurgi cal reversi bl e treatment measures have fai l ed to control the
presenti ng si gns and symptoms.
3. The pati ents' symptoms del eteri ousl y affect thei r day to day acti vi ty to the extent that
they have a poor qual i ty of l i fe.
Chroni c TMPDS that i s resi stant to conservati ve management may requi re the modal i ti es
used to treat chroni c pai n of psychogeni c ori gi n.
Internal derangement
Internal derangement i s a common di sorder and i s due to the permanent
di spl acement of the arti cul ar di sc, whi ch has an abnormal rel ati onshi p to
both the gl enoi d fossa and arti cul ar emi nence.
Internal derangement i s a common di sorder and i s due to an abnormal rel ati onshi p of the di sc
to the condyl e, gl enoi d fossa, and arti cul ar emi nence. Thi s condi ti on i s di fferent from TMPDS
because the arti cul ar di sc i s permanentl y, not i ntermi ttentl y di spl aced, as i n TMPDS. The cl i ck
i n true arti cul ar derangement i s conti nuousnot intermi ttent. Pai n i s not al ways a feature,
especi al l y

i n the earl y stages of thi s condi ti on. Di sc di spl acement may be di vi ded i nto two
Pri or to constructi on of occl usal spl i nts:
Undertake a pretreatment anal ysi s of the stomatognathi c system
Make comprehensi ve wri tten records
Archi ve study model s wi th an occl usal record prior to spl i nt therapy
Al l occl usal appl i ances:
have a pl acebo effect
al ter occl usal contacts
decrease occl usal forces transmi tted to teeth
Do not use parti al coverage spl i nts (ri sk of uncontrol l ed tooth movement)
Rei nforce the need for good oral and appl i ance hygiene
P. 199
presentati onseach has a di fferent treatment modal i ty.
Disc displacement with reduction
Reproduci bl e reci procal cl i cki ng
Di sc di spl acement shown by i magi ng and absence of degenerati ve bone di sease
In addi ti on, there may be pai n, devi ati on of jaw movements, no l i mi tati on of openi ng.
Disc displacement without reduction
Table 16.3 Characteristics of commonly used occlusal
appliances in disorders of the TMJ
Soft vacuum-formed spl i nts
Usef ul mai nl y for muscul ar (myof aci al ) si gns and symptoms
Soft ful l coverage spl i nts, better tol erated i n the l ower arch
Qui ck to makei mpressi on requi red of onl y one arch
Do not conform to a speci fi c occl usal prescri pti on, but can be made i n di fferent
thi cknesses
Not amenabl e to occl usal adjustment
Worn at ni ght ti me
Good for acute TMPDS; can make bruxi sm worse i n some pati ents
Last approxi matel y 6 months
Stabi l i zati on spl i nt
Hard acryl i c, ful l coverage spl i nt
Techni cal l y demandi ng and ti me-consumi ng to make. Requi res i mpressi ons of both
arches and a record of centri c rel ati on. A f ace bow i s requi red i n di ffi cul t occl usal
cases.
Maxi l l ary or mandi bul ar appl i ance but maxi l l ary i s often easi er to adjust
Worn at ni ght ti me, for l ong-term use
Desi gned to provi de an i deal occl usi on at rest and i n functi on (i . e. centri c
occl usi onmaxi mum si mul taneous occl usal contactsi deal l y thi s shoul d equate to
centri c rel ati on. There shoul d be i nci sal and cani ne gui dance wi th no posteri or
i nterferences)
Anteri or reposi ti oni ng spl i nt
Mandi bul ar or maxi l l ary hard acryl i c, ful l occl usal coverage
Requi re i mpressi ons of both arches and an occl usal record wi th the mandi bl e
protruded
Indi cated for di sc di spl acement wi th reducti on (i .e. cl i ck di sappears when pati ent i s
asked to open and cl ose from a protrusi ve mandi bul ar posi ti on)
For functi onal use, as much as possi bl e; i deal l y worn 24 h/day for about 12 weeks
Avoi d i n adol escents to prevent i t acti ng as a functi onal orthodonti c appl i ance
Objecti ve i s to al l ow the di sc to reposi ti on
Persi stent l i mi tati on of mouth openi ng ( 35 mm) wi th hi story of sudden onset
Di sc di spl acement shown by i magi ng and absence of degenerati ve bone di sease
In addi ti on there may be pai n and cl i cki ng.
Disc displacement with reduction
In thi s condi ti on the di sc i s di spl aced duri ng openi ng and cl osi ng. Duri ng functi on the
mal al i gned di sc reduces or i mproves i ts structural rel ati onshi p wi th the condyl ar head.
In di sc di spl acement wi th reducti on there i s a reciprocal cl i ck, noted duri ng openi ng and
cl osi ng, that i s not al ways pai nf ul , and jaw devi ati on on openi ng and cl osi ng i s common. The
reci procal cl i ck i s usual l y el i mi nated when the pati ent opens and cl oses from a protruded
mandi bul ar posi ti on, often wi th the i nci sors i n an edge to edge posi ti on confi rmi ng the
presence of anteri or di sc di spl acement wi th reduction. Thi s condi ti on does not al ways meri t
treatment, especi al l y i f the onl y presenti ng probl em i s that of a cl i cki ng joi nt. The
management of thi s condi ti on may range from counsell i ng to the provi si on of a stabi l i zati on
spl i nt and physi otherapy. Muscl e rel axants are occasi onal l y used and, i n a mi nori ty of cases,
surgery may be consi dered. The condi ti on may deteri orate wi th the di sc becomi ng
progressi vel y more di spl aced and possi bl y i nterfering wi th openi ng of the mandi bl e, l eadi ng to
di sc di spl acement wi thout reducti on and a cl osed l ock si tuati on.
Disc displacement without reduction
In thi s condi ti on the di sc i s di spl aced duri ng openi ng and cl osi ng. The mal al i gned di sc does not
i mprove i ts structural

rel ati onshi p wi th the condyl ar head duri ng functi onthe di sc i s permanentl y di spl aced,
usual l y i n an anteri or or anteromedi al di recti on. There i s a hi story of the jaw l ocki ng, because
the mi sal i gned di sc mechani cal l y obstructs the condyl e duri ng jaw openi ng. There i s usual l y no
evi dence of any arti cul ar cl i cki ng of a reci procal nature, al though a previ ous hi story of such
cl i cki ngfol l owed by l ocki ngmay be gi ven. The di sc di spl acement may, on occasi on, be
reduced from a protruded mandi bul ar posi ti on, al though thi s i s not al ways the case. The
i mpl i cati on i s that the protruded mandi bl e has pl aced the condyl e i n a posi ti on where i t i s i n a
normal functi onal rel ati onshi p wi th the di scas opposed to compressi ng the posteri or part of
the di sc (bi l ami nar zone). In other cases, the cl osed l ock i s not reduci bl e wi th anteri or
mandi bul ar reposi ti oni ng (protrusi on) and, consequentl y, the di sc di spl acement i s permanent.
Counsel l i ng, physi otherapy, and muscl e rel axants can be of val ue i n these cases and an
anteri or reposi ti oni ng spl i nt may al so be i ndi cated. Surgery may be sui tabl e for a mi nori ty of
pati ents.
Di sc di spl acement can someti mes be reduced from a protruded mandi bul ar
posi ti on.
Rheumatoid arthritis
Rheumatoi d arthri ti s i s a common mul ti system, autoimmune, i nfl ammatory di sease. It i s now
accepted that the TMJ i s i nvol ved to some extent i n a l arge proporti on of pati ents wi th
general i zed rheumatoi d di sease. It i s, however, unusual for the pati ent to present,
undi agnosed, wi th pri mary symptoms for the TMJ. When pati ents wi th rheumatoi d arthri ti s do
seek treatment for TMJ probl ems the major compl ai nt i s of l i mi tati on of openi ng and crepi tus,
sti ffness, and pai n fol l owed by achi ng. Si gni fi cant pai n as a symptom i s rare. Crepi tus i s the
most common cl i ni cal si gn on exami nati onjoi nt tenderness and functi onal abnormal i ti es
may al so be seen. Symptoms are usual l y bi l ateral . Radi ographi c evi dence of changes has been
found i n a hi gh proporti on of these pati ents, mani festi ng as erosi ons, prol i f erati ons, and
fl atteni ng of the condyl ar head. As i n other joi nts, the di sease process may occur i n a phasi c
P. 200
manner, acute exacerbati ons bei ng fol l owed by ei ther a heal i ng phase or a secondary chroni c
phase. Ankyl osi s of the TMJ can occur. Rarel y, i t is possi bl e f or the pati ent to devel op an
anteri or open bi te deformi ty caused by destructi on of the condyl es and the l oss of condyl ar
and posteri or and occl usal face hei ght.
Immunol ogi cal tests for rheumatoi d arthri ti s were outl i ned i n Chapter 2. A rai sed erythrocyte
sedi mentati on rate (ESR) and hypergammagl obul i naemia are usual l y present, together wi th an
el evated ti tre of rheumatoi d factor and anti nucl ear and other anti bodi es. These tests may
prove posi ti ve before systemi c changes have been noti ced. It i s al so worth rememberi ng that
a si gni fi cant proporti on of pati ents wi th rheumatoid arthri ti s have Sj gren' s syndrome. These
i ndi vi dual s may devel op sal i vary gl and pathol ogy that presents as faci al pai n. Sjgren' s
syndrome i s descri bed i n ful l i n Chapter 8.
Nonsteroi dal anti -i nf l ammatory anal gesi cs are the mai nstay of treatment for rheumatoi d
arthri ti s. However, more severe cases are treated by di sease-modi fyi ng drugs such as
methotrexate or azathi opri ne. Surgery of the TMJ may be i ndi cated f or ankyl osi s and cases of
severe condyl ar destructi on. In some pati ents prostheti c joi nt repl acement or autogenous
(costochondral ) grafti ng may be requi red to decrease pai n, i mprove appearance, or restore
functi on.
Osteoarthrosis (osteoarthritis)
Changes may take pl ace i n the TMJ as part of a general i zed, degenerati ve arthri ti c condi ti on.
Osteoarthrosi s i s a metabol i c defect of arti cul ar carti l age and i s usual l y asymptomati c i n the
TMJs and i s seen as a chance fi ndi ng on radi ographs. Radi ographi c changes may not al ways be
evi dent but, when present, they are vari abl e and i ncl ude a reducti on i n joi nt space and
erosi ons of the arti cul ati ng surfaces of the condyle and of the fossa. Osteophytes may
occasi onal l y be seen at the anteri or edge of the condyl e.
Most pati ents wi th osteoarthrosi s are femal e and over the age of 50 years. Pai n i s rare and
the hi story i s usual l y of joi nt sounds and gradual ly i ncreasi ng sti f fness. The joi nt may be
tender to pressure and crepi tati ons are often heard and fel t when the joi nt i s moved.
Movement may al so be restri cted. When pai n i s present i t tends to be l ocal i zed to the pre-
auri cul ar regi oni n contrast to the myofaci al pai n di stri buti on of TMPDS. The rheumatoi d
factor i s negati ve and the ESR normal i n these pati ents. Symptomati c anti -i nf l ammatory
anal gesi cs are usual l y effecti ve for osteoarthrosi s and muscl e rel axants are occasi onal l y
i ndi cated. Surgery i s rarel y appropri ate.
Masseteric hypertrophy
Uni l ateral enl argement of the muscl es of masti cati on, and of the masseter i n parti cul ar,
occasi onal l y occurs as a response to seri ous derangement of the occl usi on l eadi ng to
uni l ateral masti cati on. It may al so, however, occur wi th l i ttl e or no occl usal di sharmony and,
i n fact, may be bi l ateral rather than uni l ateral . In the affected pati ents the compl ai nt i s
usual l y onl y of i ncreasi ng faci al asymmetry. On exami nati on the masseter (or masseters) i s
found to be enl arged as a whol e, of ten wi th a marked i ncrease overl yi ng the mandi bul ar
i nserti on. When the masseter i s defi ned for exami nati on by aski ng the pati ent to cl ench the
teeth, the muscl e i s easi l y pal pated, the l ower part often standi ng out and resembl i ng a soft-
ti ssue mass. Pl ai n radi ography (posteri oranteri or mandi bl e) or a dental panorami c vi ew
may show a marked concavi ty at the i nserti on of the masseter, at the peri phery of whi ch there
i s l i ppi ng of the bone. If el ectromyographi c f aci l iti es are avai l abl e, i t i s may be possi bl e to
demonstrate atypi cal muscl e acti vi ty i n al l the muscl es of masti cati on. Magneti c resonance
i magi ng i s useful i n defi ni ng normal anatomy and hypertrophy of the muscl e and i n i denti fyi ng
any other pathol ogy (Fi g. 16.4).

Management of masseteri c hypertrophy wi th i njecti on of botul i num toxi n has been reported,
but there i s no convi nci ng evi dence of i ts l ong-term benefi t.
P. 201
Fig. 16.4 Axi al magneti c resonance scan showi ng pterygoi d and masseter muscl es.
Tumours
Pseudotumours, such as synovi al chondromatosi s, and true tumours of the TMJ are rare.
Osteomas (Fi g. 16.5) and chondromas form the majority of these and are beni gn and restri ct
mandi bul ar openi ng. They may al so resul t i n occl usal probl ems wi th occasi onal skel etal and
jaw deformi ti es. Mal i gnant osteosarcomas are excepti onal l y rare i n the TMJ, al though
mal i gnant tumours of nearby structures, such as the paroti d gl and, may i nvol ve the TMJ area
by secondary i nvasi on.
Tri smus i s the reduced abi l i ty to open the mandi bl e due to refl ex muscul ar spasm
(contracti on) of the masti catory muscl es. The

term i s, however, often used more broadl y to ref er to an i nabi l i ty to open the mouth ful l y, for
exampl e, due to ankyl osi s of the TMJ fol l owi ng trauma or fi brosi s of the masti catory muscl es
fol l owi ng exposure to external beam therapeuti c radi ati on.
The most l i kel y cause of thi s 20 year ol d' s recent hi story of pai n and tri smus i s a dental
i nf ecti on. At her age thi s may be associ ated wi th an i mpacted mandi bul ar thi rd mol ar due to
peri coroni ti s. Questi oni ng the pati ent about her dental hi story wi l l be hel pful . If the condi ti on
i s peri coroni ti s, i t i s possi bl e that the pati ent i s aware that she has an erupti ng tooth and may
have experi enced si mi l ar symptoms i n the past. A radi ograph i s essenti al to confi rm the
posi ti on of a parti al l y erupted tooth. The pati ent may have had an i nferi or dental nerve bl ock
for recent treatment and thi s occasi onal l y resul ts i n damage to the muscl es due to
i nf l ammati on or haematoma formati on.
The most common causes of tri smus are i nfecti on (f or exampl e, peri coroni ti s, si al adeni ti s),
acute TMPDS, and trauma. The trauma may be i atrogeni c (fol l owi ng removal of mandi bul ar
thi rd mol ars or i nferi or dental nerve bl ocks) or a resul t of vi ol ence or an acci dent (faci al and
condyl ar fractures, damage to the muscl es of masti cati on). The causes of tri smus are l i sted i n
Tabl e 16. 4.
Thi s edentul ous 70-year-ol d pati ent may be suf feri ng from TMPDS, despi te the fact that i t i s
l ess common i n edentul ous and el derl y pati ents. An ol der pati ent wi th crepi tus may be
sufferi ng from osteoarthrosi s. The pai n hi story i s di f ferent for these two condi ti ons. A pl ai n
fi l m of the TMJs, such as an open jaw dental pantomograph may show i rregul ari ti es of the
condyl ar surface such as erosi ons or osteophytes i n osteoarthrosi s.
Fig. 16.5 An osteoma of the neck of the condyl e.
Q1 What are the l i kel y causes for thi s pati ent' s tri smus?
P. 202
Q2 Li st the possi bl e aeti ol ogi es for acute and chroni c l i mi tati on of mouth openi ng.
Q1 What condi ti ons may be responsi bl e for thi s pati ent' s pai n and what coul d you do, i n
the fi rst i nstance, to hel p you reach a defi ni ti ve di agnosi s?
Thi s pati ent has two probl ems, pai n and overcl osure wi th hi s denturesthese may or may
not be rel ated. In the fi rst i nstance, one of the dentures coul d be fi tted wi th a soft occl usal
spl i nt and the pati ent revi ewed over a 6-week peri od to see i f the pai n has reduced. The spl i nt
wi l l i ncrease the occl usal verti cal di mensi on and thi s may be benef i ci al i n reduci ng the pai n.
Thi s i s a reversi bl e procedure and a hel pful one pri or to constructi ng new dentures for thi s
pati ent. NSAIDs may be prescri bed (provi di ng there are no contrai ndi cati ons). If symptoms do
not resol ve after the constructi on of new dentures, physi otherapy may be consi dered. Thi s
pati ent may need referral to a speci al i st cl i ni c i f a radi ographi c assessment has not been
undertaken or i f the pai n fai l s to respond to the management outl i ned above.
Table 16.4 Causes of trismus
Extra-articular Intra-articular
Infecti on/i nfl ammati on i n rel ated
structures (e.g. peri coroni ti s, si al adeni ti s)
Haematoma, i nfl ammati on, or i nfecti on
fol l owi ng mandi bul ar
Trauma (post-operati ve oedema, faci al
trauma)
TMPDS
Tetany
Tetanus
Fi brosi s due to burns or radi ati on
Systemi c scl erosi s and submucous fi brosi s
Neopl asm
Infecti ve arthri ti s
Osteoarthrosi s
Rheumatoi d arthri ti s
Ankyl osi s of TMJ
Intra-capsul ar condyl ar
fracture bl ock i njecti ons
Di sl ocati on
Table 16.5 Causes of pain in or adjacent to the
temporomandibular joints
TMPDS
Internal derangement (di sc di spl acement)
Arthri ti s
Osteoarthrosi s
Odontogeni c i nfecti on
Traumafractures
Iatrogeni ci nf l ammati on f ol l owi ng mandi bul ar bl ock i njecti on or removal of
mandi bul ar thi rd mol ars
Sal i vary gl and di sease (e.g. mumps, autoi mmune si aladeni ti s, mal i gnanci es)
Gi ant cel l arteri ti s (i schaemi c pai n)
Mal i gnanci es
Ear probl ems
Q2 There are several causes of pai n that may present i n or adjacent to the
temporomandi bul ar joi nts. Make a l i st of the possi bl e di fferenti al di agnoses.
Tabl e 16. 5 l i sts causes of pai n i n or adjacent to the temporomandi bul ar joi nts.
Projects
1. Descri be the features of the di fferent anatomi cal zones of the arti cul ar di sc and l i st the
ori gi n and i nserti on of al l the muscl es of masti cati on. Expl ai n wi th the ai d of di agrams
the di fferences between di sc di spl acement wi th reducti on and di sc di spl acement wi thout
reducti on.
2. What are the surgi cal treatments avai l abl e for TMPDS?
3. What rol e does the physi otherapi st pl ay i n the management of TMPDS?
> Tabl e of Cont ents > 17 - Psychogenic or ofaci al probl ems
17
Psychogenic orofacial problems
Problem cases
Case 17.1
A 55-year-ol d femal e pati ent presents wi th a 3-month hi story of a burni ng and ti ngl i ng
sensati on on the ti p of the tongue. The pati ent rel ates the onset of symptoms to the
provi si on of new repl acement compl ete dentures.
Case 17.2
A new pati ent presents to your practi ce and gi ves a 3-year hi story of toothache. On
exami nati on the pati ent has a heavi l y restored denti ti on.
Case 17.3
A 60-year-ol d edentul ous f emal e i s seen by a col l eague i n your dental practi ce. Thi s l ady has
a 3-month hi story of faci al pai n, whi ch i s i ncreasi ng i n i ntensi ty. The exami ni ng denti st thi nks
that the pati ent has ei ther atypi cal faci al pai n or tri gemi nal neural gi a. Radi ographs do not
show any si gni fi cant abnormal i ty and the dentures are sati sfactory. You are asked to gi ve your
opi ni on.
Introduction
A substanti al number of orof aci al probl ems may occur as a mani festati on of psychosomati c
di sease. These i ncl ude atypi cal faci al pai n, tensi on headaches (see Chapter 15), oral
dysaesthesi a (i ncl udi ng burni ng mouth syndrome), and di sturbances i n taste and sal i vati on.
Psychol ogi cal symptoms may aggravate or i ni ti ate di sease. Many condi ti ons that have organi c
causes can have si gni fi cant psychosomati c components, such as asthma and mi grai ne. It has
been esti mated that approxi matel y one-thi rd of al l consul tati ons between pati ents and thei r
medi cal practi ti oner are essenti al l y about psychol ogi cal probl ems. Di agnosi s of a
psychosomati c symptom does not necessari l y i mpl y that the pati ent has an underl yi ng
psychi atri c i l l ness. A transi ent emoti onal di sorder, such as an anxi ety state or stressful l i fe
events, can frequentl y gi ve ri se to orofaci al symptoms. Pati ents who are emoti onal l y di sturbed
may present wi th physi cal symptoms and there i s an unfortunate tendency to di smi ss these as
bei ng i magi nary. It i s i mportant to appreci ate that emoti onal di sturbances can have
an effect on the hormonal , vascul ar, and muscul ar systems, and may produce physi cal
symptoms such as xerostomi a or faci al pai n. The denti st, however, must al ways be very
Q1 How woul d you i nvesti gate thi s l ady' s symptoms and what are the possi bl e causes?
Q1 Di scuss how you mi ght di fferenti ate between pai n of dental ori gi n and atypi cal
odontal gi a i n thi s pati ent.
Q1 What i nformati on from the pai n hi story and cl i ni cal exami nati on woul d you use to
di fferenti ate between these two condi ti ons? (You wil l al so need to read Chapter 15 to
answer thi s questi on. )
caref ul to el i mi nate any organi c cause for pati ents' symptoms before di agnosi ng them as bei ng
due to an underl yi ng emoti onal or psychol ogi cal probl em.
Emoti onal di sturbances (anxi ety, depressi on, and stress) can exacerbate or cause physi cal
symptoms.
It i s al so i mportant to bear i n mi nd that pati ents wi th orof aci al pai n of psychogeni c ori gi n may
sti l l devel op pai n of dental ori gi n. It can be al l too easy for a cl i ni ci an to be l ess
di scri mi natory i n assessi ng new pai ns. A thorough pai n hi story and dental exami nati on
are al ways requi red when the pai n hi story al ters. Pati ents wi th a hi story of psychol ogi cal
probl ems frequentl y feel that they have been l abel l ed and are prejudged by the
medi cal and dental professi on. The cl i ni ci an needs to keep an open mi nd. The pati ent
shoul d be abl e to recogni ze that hi s or her compl aint i s bei ng taken seri ousl y. It i s al ways
worth rememberi ng that a pati ent may have an undi agnosed, l i fe-threateni ng condi ti on.
Examples of psychosomatic diseases
Oral dysaesthesi a
Temporomandi bul ar dysf uncti on
Di srupti ve gaggi ng
Dry mouth
Anorexi a nervosa
Tensi on headaches
Chroni c fati gue syndrome
Pani c attacks

The busy dental surgery i s not the i deal pl ace to el i ci t a soci al hi story and pati ents may be
rel uctant to di scuss personal probl ems i n such an envi ronment. However, many pati ents bui l d
up a rel ati onshi p of trust wi th thei r denti st, parti cul arl y i f they have been under hi s or her
care for a whi l e. Taki ng a soci al and psychol ogi cal hi story i s often ti me-consumi ng but can be
i nval uabl e i f i t provi des an i nsi ght i nto orofaci al symptoms wi th no demonstrabl e organi c
cause. A pati ent may appear to be copi ng bravel y with a condi ti on unti l questi oned about
l i festyl e and emoti onal wel l -bei ng. Tabl e 17. 1 suggests several questi ons that may be usef ul
i n assessi ng how a pati ent i s copi ng wi th a chroni c condi ti on such as atypi cal faci al pai n or
burni ng mouth syndrome. The use of psychometri c questi onnai res can be hel pful . However,
many requi re ski l l ful i nterpretati on. The Hospi tal Anxi ety and Depressi on (HAD) scal e i s a
rel ati vel y strai ghtforward i ndi cator of a pati ent' s anxi ety and depressi on. It can assi st the
cl i ni ci an i n i denti fyi ng emoti onal probl ems i n patients. The scal e was devi sed for use i n non-
psychi atri c hospi tal departments and can be fi l l ed i n by the pati ent over a few mi nutes, and
qui ckl y scored by the cl i ni ci an.
Pati ent confi denti al i ty i s of the utmost i mportance and must be mai ntai ned i n al l aspects of
cl i ni cal denti stry. Thi s poi nt must be stressed to al l members of the dental team.
The successful management of chroni c pai n condi ti ons requi res that psychol ogi cal i ssues are
addressed.
Termi nol ogy can someti mes be confusi ng to the uni niti ated. Somati zati on i s a term used to
descri be the process whereby an i ndi vi dual ' s psychol ogi cal and soci al di stress are mani fested
as bodi l y symptoms that cannot be whol l y attri buted to organi c pathol ogy. Somatoform
di sorders are theref ore psychogeni c condi ti ons. However, there are stri ct cri teri a that shoul d
P. 206
be ful fi l l ed before a di agnosi s of somatof orm di sorder i s made.
Somati zati on i s a term used to descri be the process whereby an i ndi vi dual ' s
psychol ogi cal and soci al di stress are mani fested as bodi l y symptoms that
cannot be whol l y attri buted to organi c pathol ogy.
Chronic orofacial pain
Atypical facial pain
Clinical features
Thi s i s a rel ati vel y common cause of non-odontogenic faci al pai n, whi ch i s encountered
parti cul arl y i n mi ddl e-aged women. Atypi cal faci al pai n i s characteri zed by a conti nuous dul l
ache. If the pai n i s not conti nuous, i t i s present for most of the ti me. The descri ptors of the
pai n can be vari abl e and are often emoti ve. The pai n f requentl y affects the maxi l l a and can be
bi l ateral or uni l ateral . However, i t i s not unusual for i t to be poorl y l ocal i zed. The pai n i s not
usual l y provoked by faci al movements, and general l y fai l s to respond to si mpl e anal gesi cs.
Characteri sti cal l y, i t affects the non-muscul ar si tes of the f ace and an epi sode may l ast for
hours or days, al though i t can be i ntermi ttent. The pai n i s frequentl y aggravated by fati gue,
worry, or emoti onal upset.
Atypi cal faci al pai n i s characteri zed by a conti nuous dul l ache that can be bi l ateral or
uni l ateral but frequentl y affects the maxi l l a.
Several speci al i st opi ni ons may have been sought and numerous i nvesti gati ons undertaken
pri or to the pati ent presenti ng to the oral medi ci ne cl i ni c. Typi cal l y, pati ents have been seen
by (or al ready have appoi ntments for) ENT speci al i sts, physi ci ans, maxi l l ofaci al surgeons, and
neurol ogi sts. Many have sought treatment from practi ti oners of al ternati ve medi ci ne. Tabl e
17. 2 summari zes the features of atypi cal faci al pai n.
A number of studi es have suggested that atypi cal faci al pai n i s often provoked by some form
of dental treatment, such as extracti ons or restorati ve procedures, but i t may al so be i ni ti ated
or exacerbated by stressful l i fe events such as bereavement, di vorce, or i l l ness i n the fami l y.
The pati ents frequentl y compl ai n of pai n el sewhere i n the body, whi ch may have been
Table 17.1 Useful questions when taking a history of pain
with suspected psychogenic origin
How are you sl eepi ng?
Has your appeti te been affected by thi s condi ti on?
Does the compl ai nt stop you enjoyi ng yoursel f (e. g. soci al i zi ng wi th fri ends)?
How do your fami l y/partner/fri ends react to your condi ti on?
What do you thi nk (or do you have any i dea what) has caused the pai n?
Do you do anythi ng to hel p you cope wi th your pai n /di scomfort?
Does anythi ng take your mi nd off the pai n (e. g. exerci se, rel axati on, dri nki ng
al cohol )?
di agnosed as i rri tabl e bowel syndrome, tensi on headaches, or dysmenorrhoea

(among many other possi bi l i ti es), so that there i s f requentl y a compl ex (and perhaps
i naccurate) hi story of chroni c i l l heal th. It has been esti mated that up to 80 per cent of
pati ents wi th psychogeni c faci al pai n have other chroni c pai n condi ti ons (see Tabl e 17. 3).
There i s al so consi derabl e overl ap of symptoms between atypi cal f aci al pai n and chroni c
temporomandi bul ar pai n dysfuncti on. Some authori ti es bel i eve that the two groups are
i ndi sti ngui shabl e. It i s certai nl y the case that some pati ents wi th atypi cal faci al pai n may gi ve
a hi story of i ntermi ttent temporomandi bul ar joi nt (TMJ) dysf uncti on, and others report
symptoms of oral dysaesthesi a.
Atypical odontalgia
Atypi cal odontol agi a (i di opathi c odontal gi a) i s a vari ant i n whi ch the pai n i s l ocal i zed to one
tooth (or a number of teeth) that appears to be cl ini cal l y and radi ol ogi cal l y sound. Thi s
si tuati on i s very di ffi cul t to di agnose. The symptoms can resembl e those of pul pi ti s and
P. 207
Table 17.2 Features of atypical facial pain
Dul l , naggi ng nature but pai n descri ptors may not be consi stent
Emoti ve adjecti ves may be used to descri be the pai n
Pai n i ntensi ty may vary
Uni l ateral or bi l ateral di stri buti on of pai n but l ocati on may change wi th ti me
Pai n i s not usual l y rel ated to anatomi cal di stri buti on of a nerve
Si mpl e anal gesi cs are usual l y i neffecti ve
Pai n exacerbated by stress and/or dental treatment
Hi story of other chroni c pai n di sorders i s common
No obvi ous underl yi ng organi c si gns
Hi story of extensi ve restorati ve and/or surgi cal treatment to resol ve the pai n i s
common
Frequentl y pati ent has been seen by several speci al i sts for thi s condi ti on
Table 17.3 Chronic medical conditions frequently
associated with atypical facial pain
Irri tabl e bowel syndrome
Dyspepsi a
Headaches
Dysmenorrhoea
Neck and/or back pai n
Fi bromyal gi a
Chroni c fati gue syndrome
peri odonti ti s and repeated repl acement restorati ons f ai l to resol ve the pai n. Operati ve
i nterventi on can often aggravate the condi ti on. Cl assi cal l y, the offendi ng tooth i s root-fi l l ed i n
an attempt to el i mi nate pai n of pul pal ori gi n. Periradi cul ar surgery may then be carri ed out.
Ul ti matel y, the tooth i s extracted. The pai n i s then frequentl y transf erred to an adjacent tooth
or teeth. On occasi on, thi s can resul t i n an aggri eved pati ent bl ami ng the denti st for taki ng
the wrong tooth out. In the absence of teeth the pai n may then persi st i n the al veol us.
Expl oratory surgery and ri dge-smoothi ng procedures may be tri ed before a di agnosi s of
atypi cal f aci al pai n i s made.
A provi si onal di agnosi s of atypi cal faci al pai n can often be made after l i steni ng to the pati ent' s
hi story, but i t i s essenti al to el i mi nate al l other demonstrabl e causes of faci al pai n,
parti cul arl y those due to dental condi ti ons. Pati ents who gi ve a hi story suggesti ve of sensory
di sturbance, or have evi dence of a crani al nerve defi ci t, must be referred for speci al i st
eval uati on and i magi ng, to el i mi nate the possi bi l i ty of a l atent neopl asm or general i zed
neurol ogi cal di sorder.
Pati ents wi th unexpl ai ned sensory defi ci ts of crani al nerves shoul d be referred for further
i nvesti gati on to excl ude space-occupyi ng l esi ons and undi agnosed neurol ogi cal condi ti ons.
The resul ts of one study have shown that about hal f the pati ents presenti ng wi th symptoms of
atypi cal f aci al pai n were suff eri ng from an underl yi ng psychi atri c di sturbance, most commonl y
a depressi ve i l l ness or neurosi s. Adverse l i fe events are frequentl y reveal ed on cl oser
questi oni ng of pati ents wi th atypi cal f aci al pai n, and i ncl ude mari tal di ffi cul ti es and chroni c i l l
heal th i n the fami l y. Chroni c anxi ety and bouts of depressi on are al so i mportant factors i n thi s
type of faci al pai n. Unfortunatel y, some pati ents wi th atypi cal faci al pai n do not readi l y accept
the i mportance of underl yi ng psychol ogi cal factors and such i ndi vi dual s are frequentl y di ffi cul t
to manage. They of ten prefer to pursue numerous further i nvesti gati ons or expl oratory
surgery, whi ch, as menti oned previ ousl y, may exacerbate the si tuati on. It i s essenti al not to
i mpl y to the pati ent that there i s anythi ng i magi nary i nvol ved i n thei r pai n as thi s
may be met wi th di sbel i ef and, i n some cases, anger. Despi te goi ng to great l engths to avoi d
pati ents mi si nterpreti ng your di agnosi s, they are frequentl y of the opi ni on that you thi nk thei r
pai n or condi ti on i s al l i n thei r mi nd. Thi s bel i ef may be so fi rml y hel d that the pati ent
wi l l refute your di agnosi s and decl i ne your advi ce. The authors fi nd i t hel pful , whi l e di scussi ng
the si tuati on wi th the pati ent, to compare the pai n to that of a stress-i nduced
headache, whi ch may occur as a resul t of cramped muscl es or bl ood vessel s. The concept of a
paral l el wi th a stress headache i s general l y wel l accepted by pati ents, who are abl e to
appreci ate that thi s i s not general l y due to any underl yi ng pathol ogy.
Pati ents wi th atypi cal faci al pai n often want a physi cal sol uti on to thei r condi ti on.
There are several opti ons avai l abl e to the cl i ni ci an i n the management of faci al pai n.
Counsel l i ng and reassurance may be al l that i s requi red by some pati ents, but others may
requi re psychotropi c medi cati on, wi th or wi thout psychotherapy. These opti ons are outl i ned at
the end of thi s secti on.
Oral dysaesthesia
Oral dysaesthesi a i s a term used to denote di sturbances of oral sensati on. It i ncl udes
condi ti ons such as burni ng mouth syndrome, but al so encompasses symptoms of abnormal
taste (dysguesi a) or xerostomi a i n cases where there are no cl i ni cal si gns or di scerni bl e
cause. A number of di fferi ng features of oral dysaesthesi a may occur i n the same pati ent.
Burning mouth syndrome
Burni ng mouth syndrome (BMS) (stomatodyni a i n an ol der termi nol ogy) i s a condi ti on i n whi ch
the pati ent presents wi th a compl ai nt of a general ized soreness or burni ng sensati on i n the
mouth. The tongue i s frequentl y aff ected (gl ossopyrosi s or gl ossodyni a) but the burni ng
sensati on can affect al l parts of the oral mucosa and i n some i nstances i s l ocal i zed to a smal l
di screte area. Some pati ents wi th these symptoms may have, on exami nati on, some readi l y
recogni zabl e abnormal i ty, such as geographi c

tongue that i s responsi bl e for the symptoms. Others have a compl etel y normal appearance of
the oral mucosa and i t i s thi s group who are i ncl uded i n the di agnosti c category of BMS. A ful l
assessment of these pati ents i s essenti al to el i mi nate any i denti fi abl e cause, such as systemi c
di sease or cl i ni cal l y undi agnosed oral candi dosi s. Screeni ng tests shoul d be undertaken
routi nel y to excl ude haemati ni c defi ci enci es or endocri ne probl ems such as di abetes (see
Tabl es 17. 4 and 17. 5). A number of pati ents who compl ai n of a burni ng mouth al so have a dry
mouth and shoul d be i nvesti gated to el i mi nate conditi ons such as Sj gren' s syndrome
(Chapter 8).
Denture-i nduced probl ems shoul d be el i mi nated as far as possi bl e, but i t must be appreci ated
that some i ndi vi dual s cannot tol erate dentures, however wel l constructed. A very smal l
proporti on of pati ents wi th BMS wi l l have had thei r probl em ascri bed to an al l ergy to denture
materi al s, and i t may be extremel y di ffi cul t to di ssuade pati ents from thi s attracti ve, but
unl i kel y cause. Al l ergy to denture materi al s can occur but i s very uncommon. Irri tati on due to
l eachi ng out of excess acryl i c monomer has al so been reported, but shoul d not occur i f the
dentures have been processed correctl y. In one survey of pati ents presenti ng wi th a burni ng
mouth, the pati ents were predomi nantl y (but not excl usi vel y) femal e wi th a mean age of
around 60 years and weari ng compl ete dentures. The majori ty compl ai ned of a burni ng
sensati on i n the tongue and upper denture-beari ng area. The next most common si te was the
mucosa of the l i ps and, then, other si tes of the oral mucosa. From the resul ts of thi s survey i t
woul d appear that three groups of pati ents emerged: those wi th a demonstrabl e source of
l ocal i rri tati on (50 per cent); those wi th an i denti fi abl e systemi c abnormal i ty (30 per cent);
and those wi th a psychogeni c background (20 per cent). Systemi c factors i ncl uded
haematol ogi cal defi ci enci es and undi agnosed di abetes, but other femal e endocri ne
abnormal i ti es were not promi nent.
Features of burning mouth syndrome
Predomi nantl y mi ddl e-aged femal es (7 femal es:1 mal e)
General i zed or l ocal i zed burni ng of the oral mucosa
Oral mucosa l ooks normal
P. 208
The resul t of one study has suggested that si ngl e or combi ned defi ci enci es of vi tami ns B
1
, B
2
,
or B
6
may be present i n a number of pati ents wi th BMS, and that vi tami n therapy may benefi t
such i ndi vi dual s. However, other studi es have fai l ed to show any response to these vi tami n B
suppl ements. Serum l evel s of zi nc have been i nvestigated i n pati ents wi th BMS, but there i s
no convi nci ng evi dence that zi nc defi ci ency i s i nvol ved i n the pathogenesi s of thi s condi ti on.
A number of pati ents di agnosed as sufferi ng from BMS have no i denti f i abl e underl yi ng cause
for thei r compl ai nt. These i di opathi c cases frequentl y have an underl yi ng psychogeni c cause,
such as chroni c anxi ety or depressi on. Many have a cancer phobi a and know of someone who
has suffered from oral cancer. It i s therefore hel pful to expl ore the pati ents' bel i efs about
thei r condi ti on. Thi s may i nvol ve aski ng them di rectl y i f they thi nk they mi ght have cancer.
Some pati ents wi th thi s condi ti on respond wel l to reassurance, suppl emented by an
i nf ormati on l eafl et about BMS. However, others may requi re psychi atri c i nterventi on, i n the
Table 17.4 Burning mouth syndrome: underlying
conditions
Di abetes
Haemati ni c defi ci enci esvi tami n B
12
, i ron, f ol ate*
Sal i vary gl and hypofuncti on.
Candi dosi s
Parafuncti onal habi ts (chroni c trauma)
Gastro-oesophageal refl ux di sease (GORD)
Depressi on
Al l ergy to restorati ve or denture materi al s
*Vi tami n B
1
, B
2
, B
6
defi ci enci es have al so been i mpl i cated but there i s l i ttl e sci enti fi c
evi dence to support thi s associ ati on.
Table 17.5 Burning mouth syndrome: investigations
Detai l ed cl i ni cal hi story
Ful l cl i ni cal exami nati on
Bl ood tests
Ful l bl ood count
Serum B
12
+ fol ate
Red bl ood cel l fol ate
Serum ferri ti n
Bl ood gl ucose
Mi crobi ol ogyquanti tati ve assessment of carri age of oral Candi da*
Assessment of sal i vary gl and functi on
*Isol ati on of Candi da speci es i s not i ndi cati ve of a candi dal i nfecti on. Therefore, a
routi ne swab i s of l i mi ted use i f there are no cl i ni cal si gns. Quanti tati ve assessments
are onl y undertaken i n speci al i st centres.
form of anti depressant medi cati on wi th or wi thout psychotherapy (see next secti on). An
overvi ew of management opti ons for BMS i s gi ven i n Tabl e 17. 6.
Management of chronic orofacial pain
The management of chroni c orofaci al pai n i s usual l y undertaken i n a hospi tal envi ronment
where the pati ent can be ful l y i nvesti gated

and the response to medi cati on and psychotherapy moni tored. On recei pt of ref erral f rom a
denti st i t i s often advi sabl e to wri te to the pati ent' s medi cal practi ti oner to enqui re i f there i s
any rel evant medi cal , psychol ogi cal , and psychi atri c hi story. Thi s al l ows i nval uabl e
i nf ormati on concerni ng previ ous therapi es and treatment to be col l ated and assessed.
Si mi l arl y, i t i s i mportant f or the oral physi ci an to keep the medi cal practi ti oner i nformed about
pati ent management.
A number of dental school s i n the UK and el sewhere now have psychi atri c l i ai son cl i ni cs that
can provi de an i deal envi ronment for the pati ent to be i ntroduced to psychi atri c advi ce.
Pati ents can fi nd the di agnosti c l abel l i ng of a condi ti on very reassuri ng. A smal l proporti on of
pati ents are extremel y anxi ous or agi tated because they bel i eve that they have a seri ous or
l i fe-threateni ng condi ti on such as cancer. Reassurance may be al l that i s needed for some
pati ents. Some pati ents can demonstrate remarkabl e i nsi ght i nto thei r condi ti on, and
counsel l i ng or a psychol ogi cal approach to therapy may be hel pful .
Antidepressant medication
Tri cycl i c anti depressants, such as dosul epi n hydrochl ori de (dothi epi n) or nortryptyl i ne, have
been used successful l y i n other forms of chroni c pai n, such as back pai n, and thei r anal gesi c
acti on appears to be i ndependent of the anti depressant acti on. These drugs have been shown
to be effecti ve i n atypi cal faci al pai n and BMS but they do have some si de-effects, such as
drowsi ness and dry mouth. It i s hel pf ul to make i t cl ear to the pati ent that the anti depressant
i s bei ng gi ven for i ts pai n control properti es and that i t i s not l i kel y to l ead to any form of
addi cti on. Pati ents wi l l often appreci ate the pai n-rel i evi ng properti es of anti depressants i f
they are made aware of thei r use i n other chroni c pai n condi ti ons such as arthri ti s, chroni c
back pai n, and postherpeti c neural gi a. It i s al so hel pful to i nform pati ents that chroni c pai n
often causes a reacti onary depressi on. Drowsi ness has al ready been menti oned as a si de-
effect of tri cycl i c anti depressants and thi s may be probl emati cal . An al ternati ve woul d be to
use one of the nonsedati ng sel ecti ve serotoni n reuptake i nhi bi tors (SSRIs) such as sertral i ne.
P. 209
Table 17.6 Management of burning mouth syndrome
El i mi nate
Systemi c di sease
Local causes (e. g. candi dosi s)
Counsel l i ng
Cogni ti ve behavi oural therapyrefer to cl i ni cal psychol ogi st for assessment
Anti depressant therapy
However, i f a pati ent wi th chroni c orofaci al pai n is havi ng di ffi cul ty sl eepi ng, the sedati ng
properti es of tri cycl i cs may be hel pful . Xerogeni c si de-effects l i mi t thei r use i n oral
dysaesthesi a.
Benefits from therapy with tricyclic antidepressants
Anal gesi c eff ects
Improved sl eep
Mood el evati on
Cognitive behavioural therapy
There are several types of psychol ogi cal approach to the management of psychogeni c
probl ems i ncl udi ng chroni c pai n. Cogni ti ve behavi oural therapy (CBT) i s one techni que that
may be used by cl i ni cal psychol ogi sts. In essence, CBT expl ores an i ndi vi dual ' s emoti ons,
thoughts, atti tudes, and bel i efs. The consequences that these have on behavi our are al so
assessed. Frequentl y the therapi st wi l l di scover that a person' s assumpti ons and bel i ef s may
be unhel pful and i rrati onal . Thi s i s of ten the case i n pati ents who have cancer phobi a.
Thei r bel i efs aggravate thei r anxi ety. The goal of CBT i s to repl ace dysfuncti onal cogni ti ve
structures wi th more real i sti c functi onal onescogni ti ve restructuri ng. Tabl e 17. 7 shows the
stages i nvol ved i n CBT. A case hi story of one pati ent wi th BMS i s gi ven i n the text box.
Side-effects of antidepressants
Tricyclic antidepressants
These commonl y have the fol l owi ng si de-effects:
sedati on;
dry mouth;
consti pati on;
bl urred vi si on;
uri nary retenti on.
These eff ects are due to the anti chol i nergi c (more speci fi cal l y, anti muscari ni c) acti vi ty of
tri cycl i c anti depressants. The i ndi vi dual drugs wi thi n the tri cycl i cs do vary i n thei r propensi ty
to cause si de-effects (for exampl e, there are tri cycl i cs that have i ncreased sedati ve
properti es). Some members have addi ti onal si de-effects. Tol erance to si de-effects does occur
i n many pati ents. The si de-effects are reduced by commenci ng therapy on l ow doses and then
i ncreasi ng the dose gradual l y. Therapy for el derl y pati ents shoul d al ways be i ni ti ated at l ow
doses because the hypotensi ve properti es of tri cycli cs predi spose to di zzi ness and possi bl e
syncope.
Selective serotonin re-uptake inhibitors (SSRIs)
These have fewer anti muscari ni c si de-effects than tri cycl i cs. The typi cal si de-effects are:
gastroi ntesti nal di sturbances such as nausea, dyspepsi a, vomi ti ng, abdomi nal pai n,
di arrhoea, and consti pati on;
headache;
sexual dysf uncti on.
Abrupt cessati on of therapy wi th SSRIs i s not recommended as i t can be associ ated wi th
headaches, paraesthesi a, di zzi ness, and anxi ety.
Hyponatraemi a i s a possi bl e si de-effect of al l anti depressants i n the el derl y and may be
mani fest as drowsi ness, confusi on, or convul si ons.
Compl ete pai n rel i ef i s not al ways feasi bl e and pati ents shoul d be made aware that CBT can
hel p the pati ent understand thei r condi ti on and symptoms and hopeful l y turn down the

pai n vol ume. Pai n di ari es have proved to be a useful tool i n hel pi ng pati ents understand that
thei r envi ronment can i nfl uence pai n.
P. 210
Table 17.7 Major components of cognitive behaviour
therapy with chronic pain patients*
Step 1 Assessment
Pai n l evel s
Intensi ty
Frequency
Durati on
Qual i ty
Si gnal of i mpendi ng pai n epi sode or attenuati on
Emoti onal reacti ons to pai n
Worry
Anxi ety
Depressi on
Anger and hosti l i ty
Cogni ti ons and bel i efs
Sel f -effi cacy (i . e. Can I cope wi th the pai n? or Can I do what the
cl i ni ci an i s expecti ng me to do?)
Locus of control
Expectati ons of pai n (over a ti me course, or i n certai n si tuati ons)
Behavi our
Medi cati on (prescri bed and non-prescri bed)
Acti vi ty l evel s
Avoi dance of pai nful area (when eati ng, posi ti oni ng tongue)
Encouragi ng sympathy and support from others
Step 2 Deri ve formul ati on (see Fi g. 17.1)
Step 3 Cogni ti ve restructuri ng
Rel axati on
Di stracti on: used i ntermi ttentl y duri ng cri ti cal moments (e. g. peak l evel s of pai n
i ntensi ty)
Bel i efs: change negati ve and catastrophi c thi nki ng (e.g. Thi s conti nuous pai n
means I must have cancer)
Step 4 Behavi oural changes
Acti vi ty: i ncrease physi cal and soci al behavi our
Attenti on: decrease attenti on from others i n response to pai n behavi our
Avoi dance: reduce the degree of avoi di ng areas of mouth wi th i ncreased sensi ti vi ty
Medi cati on: revi ew the use of excessi ve medi cati on and rati onal i ze to eff ecti ve l evel s
* Adapted, wi th permi ssi on, from Humphri s, G. M. , Longman, L. P., and Fi el d, E. A.
(1996). Cogni ti ve-behavi oural therapy for i di opathic burni ng mouth syndrome: a
report of two cases. Bri ti sh Dental Journal 181, 2048.
Disturbances in taste and salivation
Pati ents who compl ai n of an unpl easant taste i n whom no abnormal i ty of any ki nd i s detected
are very di ffi cul t to manage. Pati ents commonl y report a sour, metal l i c, or bi tter taste and are
typi cal l y mi ddl e-aged femal es. These i ndi vi dual s often become qui te obsessi onal about thei r
condi ti on and are frequentl y concerned that they have hal i tosi s as wel l as a bad taste. Others
compl ai n of a dry mouth, despi te the fact that the oral mucosa appears moi st and sal i vary
fl ow i s normal . Symptoms of BMS someti mes coexi st and some descri be del usi onal symptoms
such as sand or gri t i n the mouth or an excessi ve di scharge of mucus. The compl ai nt can be
associ ated wi th si gni fi cant detri mental changes i n di et and l i festyl e.
Cognitive behavioural therapy: a case history
An exampl e of thi s behavi oural approach i s i l l ustrated i n Fi g. 17.1. Thi s fl ow di agram
represents a hypothesi s or formul ati on for a pati ent wi th BMS who presented to the
authors' cl i ni c. It summari zes the i mportant features of hi s symptoms and the factors that
may i nfl uence the condi ti on. Thi s 38-year-ol d mal e noti ced hi s oral dysaesthesi a shortl y after
major l i fe eventsredundancy, mari tal breakdown, restri cted access to hi s chi l dren, and
movi ng back to resi de i n hi s parents' home. The pati ent had no i denti fi abl e physi cal cause for
hi s BMS and was referred for psychol ogi cal assessment. The pati ent attended three 50-mi nute
sessi ons, whi ch al l owed the cl i ni cal psychol ogi st to construct the formul ati on i n Fi g. 17.1. The
psychol ogi st hypothesi zed that the major l i fe events produced anxi ety. The pati ent presented
wi th symptoms of severe pai n, anxi ety, pani c attacks, and depressi on. Hi s anxi ety was
exacerbated by the bel i ef that hi s condi ti on was due to cancer. The pati ent was soci al l y
i sol ated and had no formal structure to hi s day. The pati ent was amenabl e to psychol ogi cal
i nterventi on and attended three further sessi ons for therapy. The pati ent was encouraged to
devel op a more structured l i festyl e (regul ar sl eep, eati ng properl y), soci al acti vi ti es were
i ncreased, and the bel i ef that he had oral cancer was refuted. Rel axati on techni ques were
demonstrated to the pati ent and methods to hel p the pati ent cope wi th the pai ncopi ng
strategi eswere suggested. The pati ent was pai n-free at a 6-month revi ew appoi ntment.
Thi s case hi story i s adapted, wi th permi ssi on, from Humphri s, G.M., Longman, L. P. , and Fi el d,
E. A. (1996). Cogni ti ve-behavi oural therapy for i di opathi c burni ng mouth syndrome: a report
of two cases. Bri ti sh Dental Journal 181, 2048.
There are several condi ti ons that may cause an al terati on i n taste and hal i tosi s (see Chapter
6) and these shoul d be excl uded or treated i n the fi rst i nstance. Many pati ents have a
combi nati on of symptoms descri bed above, whi ch can be mani festati ons of an underl yi ng
cogni ti ve di sorder. Fi nanci al worri es, bereavement, and cancer phobi a are frequentl y reveal ed.
Pati ents may respond to reassurance but a number wil l requi re psychol ogi cal management.

Delusional symptoms
A smal l number of pati ents have del usi onal symptoms i nvol vi ng the oral cavi ty. A del usi on i s
an abnormal bel i ef, from whi ch the i ndi vi dual cannot be di ssuaded and whi ch i s not i n keepi ng
wi th hi s or her cul tural background. These pati ents may have a hi story of psychi atri c i l l ness so
i t i s prudent to l i ai se wi th the medi cal practi ti oner. One group of pati ents that may be
encountered by the dental surgeon are those who suffer from del usi onal hal i tosi s. Despi te
i ntensi ve oral hygi ene regi mens and numerous

i nvesti gati ons, these pati ents i nsi st that thei r breath smel l s when i t does not. Such pati ents
Fig. 17.1 Psychol ogi cal formul ati on for a pati ent wi th burning mouth syndrome. The
major factors are shown wi th hypothesi zed associ ati ons (arrowheads i ndi cate the
di recti on of i nfl uence). (Thi s case hi story i s adapted, wi th permi ssi on, from Humphri s,
G. M. , Longman, L.P., and Fi el d, E. A. (1996). Cogni ti ve-behavi oural therapy for i di opathi c
burni ng mouth syndrome: a report of two cases. Bri ti sh Dental Journal 181, 2048. )
P. 211
P. 212
are very di ffi cul t to manage and frequentl y have no other mani f estati on of a psychi atri c
di sorder. Del usi onal hal i tosi s i s a f orm of a monosymptomati c hypochondri acal psychosi s.
Another presentati on of thi s condi ti on i s the phantom bi te syndrome i n whi ch pati ents
i nsi st that they have an abnormal bi te, whi l e others have a fal se bel i ef that there are l umps or
seeds under the oral mucosa. It i s i mportant that the denti st i denti fi es the pati ent who i s
del uded about thei r occl usi on. Irreversi bl e occl usal rehabi l i tati on i n these pati ents i s to be
avoi ded. Pati ents wi th del usi onal symptoms do not readi l y accept psychi atri c assi stance or a
psychol ogi cal l y based expl anati on of thei r symptoms. It i s advi sabl e for the cl i ni ci an to i nform
the general medi cal practi ti oner about hi s or her concerns. Organi c causes of dental and faci al
pai n shoul d be excl uded before di agnosi ng a del usi onal state. Earl y recogni ti on of del usi onal
symptoms may protect the pati ent from undergoi ng protracted i nvesti gati ons and unnecessary
treatment.
A del usi on i s an abnormal bel i ef, from whi ch the i ndi vi dual cannot be di ssuaded and whi ch i s
not i n keepi ng wi th hi s or her cul tural background.
Dysmorphophobia
The term dysmorphophobi a i s rather mi sl eadi ng because i t i s not a phobi a. The pati ent wi th
dysmorphophobi a has a seri ous preoccupati on wi th an aspect of thei r physi cal appearance that
they feel i s defecti ve. The physi cal abnormal i ty may be a mi nor physi cal probl em or i t may be
i magi nary. The sufferer has a feel i ng of ugl i ness and a desi re f or correcti ve treatment.
Whi l st any part of the body may be targeted, a facial feature i s most commonl y i nvol ved, for
exampl e, the teeth or faci al profi l e. The effects of thi s di sorder on the sufferer are vari abl e
but the pati ent may become dysfuncti onal and requi re hospi tal i zati on. Sui ci de attempts can be
a feature. Pati ents wi th dysmorphophobi a may be relentl ess i n thei r pursui t for dental or
surgi cal treatment to recti fy thei r percei ved def ormi ty. Such therapi es may serve to enhance
the pati ent' s morbi d preoccupati on. Di ssati sfacti on, anger, and l i ti gati on may ensue. Cl i ni ci ans
shoul d caref ul l y expl ore a pati ent' s requests and expectati ons before embarki ng upon
cosmeti c procedures and be wary of pati ents who thinks that treatment i s a magi cal cure for
thei r probl ems. The pati ent wi th dysmorphophobi a i s not al ways easy to di agnose. The borders
between an acceptabl e and abnormal appearance are open to subjecti ve i nterpretati on. When
an underl yi ng psychol ogi cal probl em i s suspected as the mai n reason for the pati ent' s
di ssati sfacti on wi th thei r appearance, i t may be advi sabl e to seek a speci al i st opi ni on bef ore
commenci ng treatment.
Changes i n the cl assi fi cati on of psychi atri c di sease have redefi ned dysmorphophobi a i nto non-
del usi onal and del usi onal vari ants. The del usi onal type i s a psychoti c di sorder and the non-
del usi onal has been named body dysmorphi c syndrome, but f or the purposes of thi s
chapter no di sti ncti on has been made between these two vari ants.
Self-injurious behaviour
There are some pati ents who cause sel f -harm to their orofaci al ti ssuesthi s may or may not
be i ntenti onal . Sel f -i njuri ous behavi our i s wel l recogni zed i n several groups of pati ents wi th
devel opmental , physi cal , or l earni ng di sabi l i ti es. Exampl es of these condi ti ons are epi l epsy,
profound neurodi sabi l i ty, cerebral pal sy, auti sm, LeschNyhan syndrome, and Ri l eyDay
syndrome (congeni tal i ndi fference to pai n). Trauma to the l i p, cheek, and tongue are
commonl y seen i n these groups of pati ents. Tabl e 17. 8 suggests some management opti ons
that may be used, but treatment i s rarel y si mpl e and not al ways successful .
A facti ti ous di sorder i s a psychol ogi cal or psychi atri c di sorder characteri zed by the
compul si ve, vol untary producti on of si gns and symptoms of a di sease for the sol e purpose of
assumi ng a pati ent' s rol e and i n the absence of other secondary gai n.
Pati ents who del i beratel y cause sel f -muti l ati on present a di ffi cul t assessment and
management probl em. Sel f -harm i s commonl y associ ated wi th depressi ve di sorders. The
orofaci al l esi ons can be vari abl e i n presentati on and si te. Gi ngi vi ti s artefacta, whi ch consi sts
of destructi ve l esi ons to the gi ngi vae, i s wel l recogni zed i n the l i terature. Often such l esi ons
are caused wi th a f i nger nai l . Si mi l ar traumati c i njuri es, such as traumati c ul cerati on, may
occur on the oral mucosa (facti ti ous stomati ti s) and on the ski n (dermati ti s
artefacta). The di agnosi s can be di ffi cul t but the l esi on i s nearl y al ways i n an area
accessi bl e to the pati ent and the cl i ni cal features are often i nconsi stent wi th the hi story and
other orofaci al condi ti ons. Pati ents rarel y admi t to causi ng such l esi ons and i t can be di ffi cul t
to persuade the

pati ent that they are i n need of speci al i st hel p. Behavi oural therapy can be benef i ci al .
However, some pati ents may requi re psychotropi c medi cati on.
Eating disorders
Anorexi a nervosa and bul i mi a nervosa are common condi ti ons i n the Western worl d. Eati ng
di sorders present an enormous chal l enge to soci ety and the heal th-care prof essi ons. Eati ng
di sorders occur i n both femal es and mal es, but are more common i n femal es wi th a rati o of
10:1. Broadl y speaki ng, anorexi cs avoi d food and consequentl y l ose wei ght, i n contrast to
bul i mi cs who have epi sodes of bi nge eati ng and purgi ng. Purgi ng may i nvol ve the abuse of
l axati ves, enemas, di ureti cs, and exerci se; sel f -i nduced vomi ti ng i s al so common. Thi s may be
consi dered an oversi mpl i fi cati on as the di f ferences between the two condi ti ons are someti mes
not so cl ear-cut. Anorexi cs can al so suffer from sel f -i nduced vomi ti ng.
Eating disorders
Anorexi a nervosa: food avoi dance; underwei ght; di storted body i mage
Bul i mi a nervosa: bi nge eati ng and purgi ng; usual l y normal body wei ght
Anorexi a nervosa i s l ess common than bul i mi a and the medi an age of onset i s 17 years, but i t
has been reported i n pati ents as young as 8 years ol d. Comorbi di ty wi th other psychi atri c and
personal i ty di sorders i s wel l recogni zeddepressi on, soci al phobi a, and obsessi ve compul si ve
di sorder are common. Medi cal condi ti ons associ ated wi th mal nutri ti on occur, such as renal
fai l ure, l i ver dysfuncti on, amenorrhoea, and dehydrati on.
P. 213
Table 17.8 Management of self-injurious behaviour in
patients with disabilities
Local
Topi cal anaestheti cs
Topi cal anti septi cs
Sutures to cl ose wound
Bi te-rai si ng appl i ances
Occl usal guards and l i p bumpers to di spl ace sof t ti ssues
Restorati on of broken teeth
Dental extracti ons
Occl usal adjustment to teeth
Systemi c
Anal gesi cs
Anti bi oti cs for spreadi ng i nfecti ons
Bul i mi a nervosa i s more common than anorexi a and i s thought to aff ect approxi matel y 2 per
cent of young adul t women. Unl i ke anorexi cs, bul i mics are usual l y of normal body wei ght, but
many have a hi story of anorexi a or obesi ty. As wi th anorexi a there are hi gh l evel s of
associ ated psychi atri c or psychol ogi cal probl ems. Systemi c di sease can al so accompany
bul i mi a. Oesophageal erosi ons are common and frequent vomi ti ng and mi suse of l axati ves can
cause potassi um depl eti on. The resul tant hypokal aemi a predi sposes the pati ent to myocardi al
i nstabi l i ty and f atal arrhythmi as can occur.
Anorexi a and bul i mi a can both have a si gni fi cant i mpact on dental hard ti ssues. Aci d erosi on
of dental hard ti ssues i s descri bed i n Chapter 18. Sel f -i nduced vomi ti ng and a hi gh i ntake of
aci di c dri nks and foods are the pri me causes of tooth wear i n pati ents wi th eati ng di sorders.
Paroti d gl and enl argement can al so be a feature of eati ng di sorders, and mucosal l esi ons have
been reported i n pati ents wi th bul i mi a. Thi s i s because the fi ngers or objects used to i nduce
vomi ti ng can traumati ze the mucosa, i n parti cul ar the soft pal ate. Stomati ti s may al so occur
due to nutri ti onal or haemati ni c defi ci enci es. The denti st therefore not onl y has a rol e i n the
preventi on and management of oral di sease i n these pati ents, but al so i n the i denti fi cati on of
eati ng di sorders. The dental practi ti oner may be abl e to encourage the pati ent to acknowl edge
the need for professi onal hel p and l i ai se wi th the pati ent' s general medi cal practi ti oner.
Drugs and alcohol
Drug dependency, i ncl udi ng al cohol i sm, may present probl ems i n the oral medi ci ne context, as
i n many others. Perhaps the most si gni fi cant drug-rel ated probl em affecti ng denti stry i s the
i ntravenous drugHIVhepati ti s rel ati onshi p. It i s qui te cl ear that many drug-dependent
pati ents may have probl ems of nutri ti onal and i mmune abnormal i ty, resul ti ng i n oral changes
such as those di scussed i n earl i er chapters.
The term al cohol i sm i s used as a generi c term, approved by the Worl d Heal th
Organi sati on, to descri be al l types of al cohol -rel ated probl ems. It has been esti mated that 1 i n
10 members of the popul ati on has a seri ous al cohol probl em. Physi cal probl ems that may
resul t from excessi ve al cohol i ntake are wi despread and aff ect al l systems. A summary i s
gi ven i n Tabl e 17. 9. Many of the effects of al cohol abuse can i mpact upon the management of
the dental pati ent, but of parti cul ar rel evance to oral medi ci ne are nutri ti onal
defi ci enci esoften fol ate defi ci encythat may resul t i n stomati ti s and other probl ems, as
detai l ed i n Chapter 13. Psychol ogi cal probl ems may i ncl ude depressi on and may mani fest
themsel ves as orofaci al probl ems, such as the burning mouth syndrome or atypi cal faci al pai n.
Occasi onal l y, pati ents wi th chroni c pai n condi ti ons may resort to al cohol or other drugs i n the
hope that

they wi l l hel p control thei r pai n. The rol e of a high i ntake of al cohol together wi th tobacco use
i n the aeti ol ogy of oral mal i gnancy was di scussed i n Chapter 10.
P. 214
Table 17.9 Effects of alcohol abuse on general health
System affected Effect
Cardi ovascul ar
system
Hypertensi on
Cardi omyopathi es
Dysarrythmi as
Immune system Increased suscepti bi l i ty to i nfecti on
Increased suscepti bi l i ty to mal i gnanci es
Haematol ogi cal screeni ng i n an al cohol -dependent pati ent may show a macrocytosi s i n the
presence of normal fol ate l evel s. The reason for thi s i s not cl ear. Li ver functi on tests may very
wel l be normal as these tests show great i ndi vi dual vari ati on.
It shoul d be appreci ated that al cohol and drug addicti ons are wel l recogni zed i n the medi cal or
dental professi onal and thi s i ncl udes the student popul ati on (see Project 1 at the end of thi s
chapter).
A ful l hi story shoul d be taken. It i s i mportant to know i f the symptoms were attenuated or
absent when the dentures were l eft out. An i ntraoral exami nati on i s requi red to i denti fy i f
there i s any mucosal abnormal i ty present. The pati ent rel ates the onset of symptoms to the
provi si on of new repl acement compl ete dentures. The dentures, therefore, shoul d be assessed
for l ack of freeway space. The new dentures may have been made wi th an unacceptabl e
i ncrease i n the occl usal verti cal di mensi on. Al ternati vel y, the denture teeth may not have
been pl aced i n the neutral zone. It i s al ways hel pful to exami ne the pati ent' s ol d dentures (i f
she sti l l has them) to compare the denture desi gns i n rel ati on to a pati ent' s compl ai nts.
Haematol ogy Def i ci ency of haemati ni cs and cl otti ng agents
Metabol i sm Hypogl ycaemi a, el ectrol yte defi ci enci es, mal nutri ti on
Gastroi ntesti nal
system
Gastri ti s, ul cerati on, mal absorpti on, hepati ti s, cirrhosi s,
pancreati ti s
Neuromuscul ar
system
Myopathi es, neuropathi es, dementi a
Psychi atri c Depressi on, anxi eti es, sui ci dal i deati on, psychoses
Orodental ti ssues Tooth wear, sal i vary gl and swel l i ng, apthous stomati ti s
Fetus Fetal al cohol syndrome
Q1 How woul d you i nvesti gate thi s l ady' s symptoms and what are the possi bl e causes?
Table 17.10 Characteristics of pain in dental conditions
Pul pi ti s
Vari abl e presentati on from mi l d to severe pai n. Reversi bl e pul pi ti s may present as
It i s possi bl e that the pati ent has devel oped parafuncti onal habi ts, such as rubbi ng the tongue
agai nst the denture, si nce the dentures were fi tted. Pati ents are often unaware of thi s. If the
denture tooth posi ti on i s thought to be sati sfactory, a soft occl usal spl i nt may be hel pful i n
breaki ng the habi t.
Al l ergy to denture-base materi al s i s excepti onal l y rare, and a mucosal probl em i s more l i kel y
to be a resul t of an excess of uncured f ree monomer. If thi s i s the case, the symptoms shoul d
resol ve i f the dentures are not worn. In addi ti on, the monomer shoul d cease to l each out of
the denture wi th ti me. Some pati ents seem unabl e to tol erate dentures and a ful l denture
hi story i s essenti al . It i s necessary to enqui re i f the pati ent has successful l y worn dentures
before (i ncl udi ng parti al dentures).
sharp pai n wi th hot, col d, and sweet sti mul i (osmoti c or thermal changes i n
pressure). Pai n may be l ocal i zed to affected tooth or adjacent teeth, but can be
referred to affected si de of face (unl i kel y to cross mi dl i ne).
Def ecti ve restorati ons, cari es, or occl usal trauma are possi bl e causes. Anal gesi cs
can be hel pful but not anti bi oti cs. Chroni c pul pi tis can al so present as a poorl y
l ocal i zed dul l ache
Cracked tooth
Vague or i nconsi stent hi story of pai n duri ng eati ng; may be sensi ti ve (sharp pai n) to
thermal sti mul i . There may be an i ntermi ttent dul l ache. Pai n i s not al ways el i ci ted
by the same sti mul i , and i t may be di ffi cul t to el ici t pai n cl i ni cal l y.
Pai n usual l y l ocal i zed but can be referred (si mi l ar manner to pul pi ti s). Affected tooth
i s usual l y restored, crack(s) may be vi si bl e but are not necessari l y pathol ogi cal .
Rel i ef i s obtai ned when occl usal forces no l onger stress the fracture l i ne (e. g. pi ece
of cusp fractures off )
Peri radi cul ar peri odonti ti s
Throbbi ng pai n, mi l d to severe. Pai n shoul d be wel l l ocal i zed. The fol l owi ng may be
present: a si nus, a l ocal i zed al veol ar swel l i ng, a peri odontal pocket.
Anti bi oti cs and anal gesi cs may have been effecti ve i n reduci ng or el i mi nati ng the
pai n. The affected tooth i s usual l y restored or cari ous; al ternati vel y, there i s a
hi story of trauma. Rel i ef shoul d be obtai ned when the source of i nfecti on i s removed
(e.g. a necroti c pul p)
Peri coroni ti s
Dul l ache wi th acute exacerbati ons. Pai n tends to be wel l l ocal i zed. Most commonl y,
the mandi bul ar thi rd mol ar i s i nvol ved. Pai n can radi ate across the affected si de of
the jaw. Pai n worse on eati ng. Very rarel y i s sl eep di sturbed.
The pai nful tooth i s usual l y partl y erupted or has an opercul um wi th an associ ated
peri odontal stagnati on area. Anti bi oti cs and anal gesi cs may have been hel pful . Rel i ef
i s obtai ned when: (1) the tooth i s taken out of occl usi on (preventi ng trauma to the
opercul um); (2) the opercul um i s removed; (3) the tooth i s extracted; or (4) the
tooth erupts ful l y
If the pati ent' s dentures are found to be adequate and the oral mucosa appears normal , then
the pati ent shoul d be ful l y i nvesti gated for BMS as outl i ned i n Tabl e 17. 5. Tabl e 17. 4 l i sts the
underl yi ng condi ti ons that can cause BMS.
When a tooth has had many dental i nterventi ons or has a l arge restorati on present, i t i s often
al l too easy to fi nd an i mperfecti on present that may be thought to be responsi bl e for the
symptoms. When a l arge f i l l i ng or a crown i s present, i t i s reasonabl e to consi der that a pul p
coul d be i nfl amed or i nfected or that a pai nful root-fi l l ed tooth coul d requi re re-treatment or
peri radi cul ar surgery. In cl i ni cal practi ce, therefore, i t can often be di ffi cul t to excl ude a
dental cause i n a tooth wi th chroni c

symptoms. It i s al ways easi er to make a di agnosi s of atypi cal odontal gi a (a vari ant of atypi cal
faci al pai n) when a successi on of i nterventi ons have been carri ed out wi thout success. It i s
therefore essenti al that detai l ed dental and pai n hi stori es are taken. A pati ent wi th atypi cal
odontal gi a may have a hi story of repeated restorations, fol l owed by root treatment, crown
pl acement, and an api cectomy. It i s not unusual to fi nd out that adjacent teeth have had
si mi l ar treatments for chroni c pai n. Teeth may al so have been extracted. The cl i ni ci an needs
to establ i sh i f any treatment was benefi ci al . The effect of any medi cati ons that have

been used for the pai n shoul d be assessed. In atypical odontal gi a i t i s unl i kel y that si mpl e
anal gesi cs and anti bi oti cs wi l l have rel i eved the pai n. Despi te thi s, pati ents may sti l l have a
hi gh dai l y i ntake of anal gesi cs.
Q1 Di scuss how you mi ght di fferenti ate between pai n of dental ori gi n and atypi cal
odontal gi a i n thi s pati ent.
P. 215
P. 216
Table 17.11 Trigeminal neuralgia and atypical facial pain:
differential diagnosis
Trigeminal neuralgia Atypical facial pain
Age (years)
50+ 3060
Pai n i ntensi ty
Remarkabl y severe Intensi ty may vary from mi l d to
severe, but usual l y a
background, l ow-i ntensi ty pai n
i s present
Pai n descri ptors
Stabbi ng, sharp, l anci nati ng pai n of a few
seconds durati on. Paroxysms may f ol l ow
Dul l , throbbi ng, gnawi ng, naggi ng,
pul l i ng, occasi onal l y sharp
i n qui ck successi on Fl ori d and emoti ve adjecti ves are used
Descri ptors may be i nconsi stent
Locati on/di stri buti on/radi ati on of pai n
Mandi bl e or maxi l l a Maxi l l a i s the most common si te
Wel l l ocal i zed Not al ways wel l l ocal i zed and si te may
change
Uni l ateral Uni - or bi l ateral
Fol l ows anatomi cal di stri buti on of one
sensory di vi si on of the tri gemi nal nerve
(occasi onal l y more than one di vi si on i s
i nvol ved)
Does not usual l y fol l ow anatomi cal
di stri buti on of nerves
Can be bi l ateral and can cross mi dl i ne
Can radi ate over whol e of face
Durati on of pai n
Bri ef usual l y onl y seconds Conti nuous
Tri gger zones for pai n
Yes, wel l defi ned Not usual l y present
Peri odi ci ty of pai n
Intermi ttent Conti nuous or i ntermi ttent
Preci pi tati ng f actors
Movi ng or touchi ng a tri gger zone. Mi l d
sti mul i may provoke an attack
Stress, l i fe events
Rel i evi ng factors
Carbamazepi neresponse to treatment
usual l y good
Peri pheral nerve bl ock wi th l ocal
anaestheti c i njecti on temporari l y rel i eves
pai n
Commonl y used anal gesi cs are not
Anti depressantsresponse vari abl e
Local anaestheti c i njecti on i nto area
often fai l s to rel i eve pai n
Anal gesi cs are occasi onal l y some hel p,
but not al ways
Pai n may not be probl emati c duri ng
The nature of the pai n i s al so i mportant. Pati ents wi th atypi cal odontal gi a/faci al pai n may al so
gi ve a hi story of pai n and al tered sensati ons that are vari abl e. It i s not unusual for a pati ent
wi th atypi cal odontal gi a to have a defi ni te vi ew of what started the pai n. Dental causes for
consi derati on i n the di fferenti al di agnosi s are gi ven i n Tabl e 17. 10compare these wi th the
features of atypi cal faci al pai n l i sted i n Tabl e 17. 2.
To di sti ngui sh between tri gemi nal neural gi a and atypi cal faci al pai n the cl i ni ci an wi l l need to
l i sten careful l y to the pai n hi story and noti ce the pati ent' s demeanour and manneri sms. The
descri ptors that the pati ent uses to descri be the pai n are of ten very i mportant. A si mpl e pai n
questi onnai re such as the McGi l l pai n questi onnai recan be very hel pful . Tabl e 17. 11, whi l st not
exhausti ve, i s of val ue i n di fferenti ati ng between tri gemi nal neural gi a and atypi cal f aci al pai n.
Projects
1. The Bri ti sh Dental Associ ati on have produced a publ i cati on enti tl ed Drugs and al cohol :
addi cti on i n the dental professi on, probl ems and sol uti ons. What i s the rol e of the
denti sts' heal th support programme i n rel ati on to denti sts wi th al cohol and
addi cti on probl ems?
2. The Hospi tal Anxi ety and Depressi on (HAD) scal e can be usef ul when assessi ng a pati ent
wi th chroni c pai n. Obtai n a copy of thi s psychometri c questi onnai re and fi nd out how to
eval uate a compl eted f orm.
benefi ci al sl eep
Pai n behavi our
A frozen face appearance
Speech and faci al appearance are al tered
i f the associ ated faci al movements cause
pai n
Avoi ds touchi ng tri gger zone
Vari abl e presentati on
Pati ent may have devel oped habi ts,
e.g. touchi ng or pi cki ng the affected
si te
Hi story
Other chroni c pai n syndromes not usual l y
present
Frequentl y a l ongstandi ng hi story of
pai n
Frequent restorati ve and surgi cal
i nterventi on i s common
Hi gh preval ence of chroni c i l l heal th,
especi al l y other chroni c pai n
syndromes
Q1 What i nformati on from the pai n hi story and cl i ni cal exami nati on woul d you use to
di fferenti ate between these two condi ti ons?
> Tabl e of Cont ent s > 18 - Di sor der s of t he teeth and bone
18
Disorders of the teeth and bone
Problem cases
Case 18.1
A 14-year-ol d boy i s unhappy wi th the appearance of hi s f ront teeth. He does not l i ke thei r
bl otchy appearance. Cl i ni cal exami nati on reveal s the presence of smal l whi te fl ecks
on the enamel surf aces of most of the teeth. The enamel surfaces are not pi tted. The teeth
have no restorati ons present and are cari es-free. The di scol orati on i s i ntri nsi c.
Case 18.2
An 18-year-ol d gi rl presents to your surgery because she i s unhappy about the appearance of
her f ront teeth and compl ai ns that her f ront teeth are getti ng thi nner. She reports that her
teeth are someti mes sensi ti ve to hot and col d. Exami nati on reveal s exposed denti ne on the
pal atal surfaces of the maxi l l ary i nci sors and canines. There i s al so exposed denti ne on the
occl usal surfaces of the maxi l l ary fi rst permanent mol ar teeth.
Disorders of the teeth
The teeth are poor i ndi cators of general i zed di sease. Fol l owi ng thei r cal ci fi cati on, metabol i c
processes have l i ttl e eff ect on the structure of the teeth. Structural abnormal i ti es al most
i nvari abl y refl ect changes occurri ng i n the peri od duri ng whi ch the teeth were bei ng formed.
Apart from structural vari ati on, abnormal i ti es i n the numbers, si ze, and shape of the teeth
occasi onal l y occur, i n conjuncti on wi th abnormal i ties of the bones or of the ski n and other
epi dermal l y deri ved structures. When numbers of mi ssi ng teeth, supernumerary teeth, or
abnormal l y shaped teeth are observed, i t i s as wel l to consi der the possi bi l i ty of some such
compl ex associ ati on. It must al ways be remembered that teeth mi ssi ng from the arch may
ei ther be congeni tal l y absent, have been extracted, or may be unerupted. The major post-
erupti on changes that occur are the l osses of tooth substance caused by cari es, attri ti on,
erosi on, and abrasi on. Thi s chapter wi l l di scuss some of the di sorders menti oned. The l i st i s
not, however, exhausti ve and the reader i s ref erred to a textbook of oral pathol ogy. Tabl e
18. 1 hi ghl i ghts some dental anomal i es that are associ ated wi th geneti c or systemi c di sease.
Q1 What are the di fferenti al di agnoses for thi s boy' s dental condi ti on?
Q2 What addi ti onal i nf ormati on do you need to hel p you make a defi ni ti ve di agnosi s?
Q1 What are the possi bl e causes of tooth wear i n this case?
Q2 What condi ti on woul d you suspect i f the pati ent al so presented wi th swol l en paroti d
gl ands and appeared to have general i zed skel etal muscl e wastage?
Table 18.1 Dental anomalies and associated diseases
Hypodontia
Hypodonti a (ol i godonti a), the congeni tal absence of teeth, represented by the l oss of one or
two teeth wi th no apparent associ ated abnormal i ti es i s not uncommon. The most common
teeth to be mi ssi ng are the l ast i n each seri es. Most surveys, however, show that one or
more thi rd mol ars are mi ssi ng i n approxi matel y one-quarter of the popul ati on. A study
carri ed out i n an Engl i sh popul ati on and excl udi ng the thi rd mol ars has shown that the teeth
most l i kel y to be mi ssi ng are the l ower second premol ars

(40.9 per cent) fol l owed by the upper l ateral i nci sors (23.5 per cent) and by the upper
second premol ars (20. 9 per cent). The pattern of mi ssi ng teeth does, however, vary from
popul ati on to popul ati on. A common fi ndi ng i n hypodonti a i s the presence of smal l and
coni cal l y shaped teeth repl aci ng normal uni ts of the denti ti on (Fi g. 18. 1). Hypodonti a i n the
pri mary denti ti on i s a rel ati vel y rare occurrence.
Dental anomaly Associated systemic/genetic disease
Di sorders of tooth devel opment
Anodonti a, ol i godonti a Hypohi droti c ectodermal dyspl asi a,
Down' s syndrome
Supernumerary teeth Cl ei docrani al dyspl asi a, Gardner' s
syndrome
Macrodonti a, mi crodonti a,
taurodonti sm, di l acerati on, gemi nati on
Down' s syndrome, Kl i nefel ter' s
syndrome
Denti nogenesi s i mperf ecta,
amel ogenesi s i mperfecta, enamel
hypopl asi a, denti ne dyspl asi a, fl uorosi s
Osteogenesi s i mperfecta, congeni tal
syphi l i s, ri ckets, hypophosphatasi a
Di sorders of erupti on and sheddi ng
Premature erupti on Hyperthyroi di sm
Del ayed erupti on Creti ni sm, ri ckets, cl ei docrani al
dyspl asi a, heredi tary gi ngi val
fi bromatosi s, Down' s syndrome
Impacti on of teeth Cl ei docrani al dyspl asi a
P. 220
The congeni tal absence of teeth associ ated wi th abnormal i ti es of the bone or ectodermal
appendages i s rel ati vel y rare. The dyspl asi a i nvol ved may be attri buted to ectodermal l y
deri ved structures or to more compl ex syndromes i n whi ch there are both dermal and bony
abnormal i ti es.
Variation in eruption
The wi de normal range makes i t di ffi cul t to speci fy accuratel y the dates of erupti on of ei ther
the deci duous or the permanent teeth. Several f actors have been f ound to affect the date of
erupti on, i ncl udi ng raci al ori gi n and such unl i kel y i nfl uences as the soci o-economi c
envi ronment. In general , earl i er bodi l y devel opment i s ref l ected i n earl y erupti on of the
teeth.
Markedl y premature erupti on of the permanent teeth i s very rare. It has been suggested that
i t may occur i n cases of hypersecreti on of those hormones that i nfl uence devel opment. It i s
somewhat more common to note premature erupti on of the deci duous teeth. Frequentl y, no
systemi c factor i s found to account for thi s. It shoul d perhaps be menti oned that teeth
present at bi rth i n a f ew chi l dren (the neonatal teeth) do not represent premature erupti on.
These are supernumerary teeth and part of a separate predeci duous denti ti on.
Del ayed erupti on of the deci duous teeth may occur i n endocri ne def i ci ency states and i t has
been shown that, i n Down' s syndrome, not onl y are the erupti on dates somewhat retarded i n
general , but al so there i s often an unusual sequence of erupti on. It i s very di ffi cul t to ascri be
a characteri sti c dental pi cture to many of the endocri ne abnormal i ti es si nce, i n a number of
cases, varyi ng and contradi ctory ef fects have been descri bed. In many such pati ents the
most obvi ous abnormal i ty i s di sproporti on i n the sizes of the teeth and the jaws, thi s i n turn
l eadi ng to gross i rregul ari ty of the occl usi on. The presence of supernumerary or unerupted
teeth may del ay or prevent erupti on. Cl ei docrani al dyspl asi a, al though rare, i s a wel l known
syndrome i n whi ch there are mul ti pl e addi ti onal teeth associ ated wi th unerupted teeth (Fi g.
18. 2).
Fig. 18.1 A coni cal l y shaped tooth i n the posi ti on of an upper l ateral i nci sor.
Variation in the size of teeth
The si ze of the teeth of any i ndi vi dual i s determi ned l argel y by i nheri ted factors. Extreme
vari ati on i n si ze, ei ther i n the di recti on of smal l teeth (mi crodonti sm) or i n the di recti on of
l arge teeth (macrodonti sm) may be accompani ed by no other growth abnormal i ty.
Conversel y, endocri ne growth di sorders l eadi ng to gi ganti sm or dwarfi sm may be
accompani ed by no correspondi ng vari ati on i n tooth si ze. The si ze of the teeth al one bears no
rel ati onshi p to metabol i c factors i n the vast majori ty of i nstances.
Non-carious tooth surface loss
Attri ti on, erosi on, and abrasi on are commonl y seen i n dental pati ents. The cl i ni cal
appearance of the teeth may be suggesti ve of a speci fi c cause f or tooth wear but cauti on
shoul d be exerci sed i n the i denti f i cati on of a si ngl e aeti ol ogi cal agent. Tooth wear i s usual l y
a mul ti factori al process. The presence of attri ti on or erosi on may be of i nterest to the oral
physi ci an. Wear facets i n opposi ng arches that match i n parafuncti onal posi ti ons of the
mandi bl e are i ndi cati ve of bruxi sm. Thi s may be si gni fi cant i n the aeti ol ogy of a headache
that i s present on waki ng or i n myofaci al pai n.
In any i ndi vi dual , tooth wear usual l y has a mul ti factori al aeti ol ogy.
Erosi on i s the l oss of dental hard ti ssue by a chemi cal process that does not i nvol ve bacteri a
(Fi g. 18. 3). The causes of erosi on are l i sted i n Tabl e 18. 2. The acti ve substances may be
endogenous or exogenous. Aci di c foodstuffs and beverages are commonl y i mpl i cated. These
i ncl ude ci trus frui t and jui ce, and many soft dri nks i ncl udi ng frui t squashes and cordi al s,
mi xer-type dri nks, and many others. A hi ghl y si gni fi cant cause of erosi on i s the sel f -i nduced
vomi ti ng of bul i mi a nervosa, whi ch may resul t i n widespread l oss of enamel , parti cul arl y from
the pal atal surf aces of the

upper anteri or teeth and the cusps of permanent mol ars. Paroti d enl argement and a non-
Fig. 18.2 Mul ti pl e unerupted teeth i n cl ei docrani al dyspl asia.
P. 221
speci f i c mucosi ti s may al so be seen i n the bul i mi c pati ent. Pati ents wi th gastri c condi ti ons
that resul t i n chroni c aci d regurgi tati on wi l l present wi th thi s type of tooth wear.
Anorexi a nervosa can be associ ated wi th dental erosi on because of a hi gh i ntake of
aci d dri nks and frui t
Bul i mi a nervosa i s associ ated wi th dental erosi on because teeth are exposed to
i ntri nsi c (gastri c) aci d
Fig. 18.3 Aci d erosi on wi th cervi cal l esi ons and l oss of normal enamel contour.
Table 18.2 Causes of dental erosion
Extri nsi c aci ds
Beverages (e. g. f resh frui t jui ces, cordi al s, carbonated dri nks, wi ne, f rui t teas)
Foods (e. g. ci trus f rui t, pi ckl ed foods)
Industri al processes (e. g. wi ne tasti ng, battery manufacture, metal pl ati ng)
Intri nsi c aci ds
Gastro-oesophageal ref l ux di sease (GORD)*
Eati ng di sorderbul i mi a nervosa
Morni ng si ckness i n pregnancy
Rumi nati on (vol untary regurgi tati on)
*Thi s may be secondary to other condi ti ons, e. g. al cohol i sm.
Discoloration of the teeth
Wi despread col orati on of the teeth occurs i n a few di seases i n whi ch abnormal bl ood
pi gments ci rcul ate. Of these, i nfanti l e jaundi ce i s the most common. In thi s condi ti on the
deci duous teeth may be col oured bl ue-green due to the l ayi ng down of a pi gment i n the
i mmedi ate postnatal denti ne zone. A l ess common, and now vi rtual l y el i mi nated, cause of
tooth di scol orati on i s haemol yti c anaemi a of the newborn caused by rhesus i ncompati bi l i ty.
Fol l owi ng the haemol ysi s, pi gments may be deposi ted i n the ski n and i n the teeth, whi ch may
take on col orati on that vari es from grey to green-grey and to brown. Col orati on of the teeth
al so occurs i n some other consi derabl y rarer si tuati ons i n whi ch abnormal pi gments ci rcul ate,
for exampl e, i n porphyri a. However, a f ar more common cause of tooth di scol orati on i s
tetracycl i ne stai ni ng. It need hardl y be sai d, however, that the number of young pati ents
wi th tetracycl i ne stai ni ng i s rapi dl y reduci ng wi th the al most total wi thdrawal of thi s group of
anti bi oti cs from at-ri sk groups.
Disturbances of the structure of enamel and dentine
When the normal sequence of enamel matri x formati on and cal ci fi cati on i s di sturbed, a seri es
of abnormal i ti es may be produced. These may be di sti ngui shed as hypopl asi a, when the
quanti ty of enamel i s reduced, or as hypocal ci fi cati on, i n whi ch the degree of cal ci fi cati on i s
unsati sfactory. The two condi ti ons may be combi ned and vari ous cl i ni cal condi ti ons may be
di f ferenti ated. A paral l el range of di sturbances i n the formati on of denti ne may al so occur,
but these are not so wel l di ff erenti ated as i n enamel . The use of the term hypopl asi a
can be conf usi ng and requi res expl anati on, si nce i t i s used both i n the stri ctl y sci enti f i c sense
as menti oned above and al so i n a cl i ni cal sense to descri be a general i zed di sturbance of
enamel structure caused by some f orm of systemi c di sturbance.
Thi s group of condi ti ons can be conveni entl y di vi ded as fol l ows:
1. enamel or denti ne devel opmental def ects resul ti ng from l ocal i zed di sturbances;
2. enamel or denti ne devel opmental defects resul ti ng from general i zed di sturbances;
3. geneti cal l y determi ned defects of enamel f ormati on or geneti cal l y determi ned def ects of
denti ne f ormati on.
Defects due to local causes
When an i nf ecti on occurs i n associ ati on wi th a deci duous tooth the permanent successi onal
tooth devel opi ng bel ow i t may undergo a di sturbance of devel opment. In such cases the
enamel i s usual l y di storted and pi tted. Thi s condi ti on i s easi l y recogni zed by i ts restri cti on to
a si ngl e successi onal tooth.
Defects due to generalized causes
Wi despread i nfecti ons or nutri ti onal di sturbances duri ng the tooth enamel devel opment
peri od may adversel y affect the l ayi ng down of enamel . Such condi ti ons affect al l the teeth
devel opi ng at the ti me and i t i s often possi bl e to ti me accuratel y the onset of the di sturbance
by the posi ti on of the defect on the teeth. In general , the defi ci ent enamel forms a band
around the tooth correspondi ng to the peri od of di sturbance (Fi g. 18. 4). The band may be
wi de or narrow and, i n some ci rcumstances, the bandi ng may be i ncompl ete. It has been
suggested that the enamel opaci ti es seen i n some pati ents may represent a mi nor form of
thi s condi ti on. Unl ess the di sturbance of tooth formati on i s unusual l y severe, the teeth do
not seem to be undul y suscepti bl e to attack by caries. Hi stol ogi cal l y, the denti ne that
devel oped at the same
P. 222
ti me as the aff ected enamel may show sl i ght def i ci enci es, but thi s does not resul t i n cl i ni cal
abnormal i ty.
Prenatal syphi l i s may produce defects i n tooth devel opment. The spi rochaete may l odge i n
the enamel organ and i nterf ere di rectl y wi th the formati on of the enamel . The ef fects are
general l y conf i ned to the anteri or permanent teeth and to the fi rst permanent mol ar. The
typi cal mol ar form of the syphi l i ti c tooth i s the mul berry mol ar i n whi ch the shape of the
tooth i s wel l expressed by i ts name; these teeth are al so termed Moon' s mol ars. The typi cal
vari ati on i n the anteri or teeth takes the f orm of a coni cal , screw-dri ver shape wi th a notched
i nci sal edge cal l ed a Hutchi nson' s i nci sor (see Chapter 4).
If the devel opment of the teeth occurs when l arge amounts of f l uori des are i ngested,
mottl i ng of the enamel may occur. The effect of f l uorosi s may be recogni zed by the presence
of opaque whi te patches i n the enamel , often arranged i n a band-l i ke f ormati on. Unl i ke the
teeth i n other forms of hypopl asi a, the teeth af fected by f l uorosi s are suscepti bl e to a brown
di scol orati on (Fi g. 18. 5) that may resembl e amel ogenesi s i mperfecta. Si mi l ar i di opathi c
mottl i ng may occur i n teeth of pati ents from non-fluori de areas, but thi s i s rare.
Hypopl asi a or hypocal ci fi cati on of the enamel may occur i n pati ents suff eri ng f rom
general i zed ectodermal di sease (such as epi dermol ysi s bul l osa) or from di sturbances of
cal ci um metabol i sm such as hypoparathyroi di sm. These condi ti ons cl earl y represent the f i nal
resul t of abnormal tooth germ formati on and abnormal cal ci fi cati on, respecti vel y. Many other
di seases may produce dental abnormal i ti es of a si mil ar type. These changes are, however,
rare. It i s unl i kel y that the general i zed di sease process wi l l have gone unrecogni zed by the
ti me that the tooth abnormal i ty has become evi dent i n the majori ty of these pati ents (Fi g.
18. 6).
Fig. 18.4 Enamel hypopl asi a as a resul t of a general di sturbance duri ng devel opment.
It wi l l be evi dent that the di agnosi s of the abnormal i ti es of tooth structure descri bed above
depends l argel y on the recogni ti on of the cl i ni cal appearance of the teeth. Radi ography
apart, there i s very l i ttl e i n the way of suppl ementary tests or i nvesti gati ons that wi l l add to
a caref ul l y carri ed out cl i ni cal exami nati on of the pati ent, an exami nati on that shoul d
evi dentl y i ncl ude a careful medi cal and fami l y hi story. In vi rtual l y al l cases l aboratory tests
prove unproducti ve.
Defects due to genetic causes
AMELOGENESIS IMPERFECTA
Amel ogenesi s i mperfecta i s a heredi tary devel opmental defect of enamel . The condi ti on may
show as ei ther a hypopl asi a of the enamel or as a hypocal ci f i cati on. If an earl y phase of
enamel formati on i s di sturbed, the amount of matri x l ai d down i s reduced, but the
Fig. 18.5 Fl uorosi s.
Fig. 18.6 Hypopl asi a i n a pati ent wi th di sturbed cal ci um metabol i sm.
cal ci f i cati on i s compl ete. We thus have a thi n and i rregul ar l ayer of hard enamel . Thi s i s the
hypopl asti c type (Fi g. 18. 7). Severe attri ti on may occur earl y i n l i f e. In the hypocal ci f i ed
type

a l ater stage of enamel formati on i s di sturbed and we have a normal l y thi ck l ayer of poorl y
cal ci f i ed enamel . In thi s case, the whol e of the enamel i s soft and eroded wi th l oss of much
enamel by attri ti on and exposure of the denti ne (Fi g. 18. 8). In both f orms the deci duous and
permanent denti ti ons may be affected. The denti ne retai ns i ts normal structure i n both
cases.
DENTINOGENESIS IMPERFECTA
In thi s condi ti on there i s fai l ure of devel opment of the denti ne wi th normal enamel
devel opment. It i s a heredi tary condi ti on and affects both the deci duous and permanent
P. 223
Fig. 18.7 Hypopl asti c enamel . The enamel i s i mperfect, but is hard.
Fig. 18.8 Hypocal ci fi ed enamel . The enamel i s soft and eroded.
denti ti ons. The teeth erupt usual l y wi th normal morphol ogy, but have a grey or brown col our.
They show a rather i ri descent col orati on, whi ch l eads to the term heredi tary opal escent
denti ne. The pul p chambers are often reduced i n si ze and may be obl i terated. Al though
the enamel i s of normal structure, i t readi l y breaks away, l eavi ng the denti ne exposed.
Occasi onal l y, thi s condi ti on occurs as part of a general i zed condi ti on of osteogenesi s
i mperfecta i n whi ch the i mperf ect cal ci fi cati on of the bones l eads to f requent f ractures. Often
i n such cases there i s a defi ci ency i n the scl era of the eye l eadi ng to a bl ue col orati on.
Disorders of bone
Di seases of bone have tradi ti onal l y been di vi ded i nto three groups: geneti c, i nf l ammatory,
and metabol i c. In real i ty, thi s cl assi fi cati on i s rather si mpl i sti c. For exampl e, the
cl assi fi cati on of Paget' s di sease i s probl emati c because the aeti ol ogy of thi s condi ti on i s
uncertai n. Nonethel ess, the cl assi f i cati on remai ns a useful one f or many condi ti ons.
Rel ati vel y f ew di sorders of bone present wi th any frequency for i nvesti gati on i n the oral
medi ci ne cl i ni c. Fi brous dyspl asi a and Paget' s di sease are seen onl y occasi onal l y. However,
some geneti cal l y determi ned di seases have an i mpact on denti stry i n general , because they
are part of general i zed syndromes i nvol vi ng bone and epi dermal appendages (i ncl udi ng
teeth). In these compl ex syndromes the predomi nant oral condi ti on i s vari ati on i n the si ze,
number, morphol ogy, and, someti mes, structure of the teeth. Cl ei docrani al dyspl asi a and
osteogenesi s i mperfecta are exampl es of such syndromes.
Inf l ammatory bone di sease i s margi nal l y wi thi n the fi el d of oral medi ci ne, al though the
cl assi c presentati on of osteomyel i ti s of the jaws is now very rare. In thi s chapter,
Table 18.3 Blood chemistry in diseases of bone
Levels in blood of
Calcium Phosphate Alkaline
phosphatase
Normal 2. 22. 7
mmol /l
0. 81. 4
mmol /l
Vari abl e

Paget' s di sease N N ++
Monostoti c fi brous dyspl asi a N N N
Pol yostoti c fi brous dyspl asi a + N +
Pri mary hyperparathyroi di sm
wi th bone l esi ons
+ N/ +
* N, Normal ; +, a moderate ri se ++, a marked ri se; , a moderate f al l .

Al kal i ne phosphatase val ues: normal l evel s for the age group shoul d be
determi ned from the speci fi c l aboratory. Usual adult val ues up to 125 IU/l .
cl ei docrani al dyspl asi a and f i brous dyspl asi a are respecti vel y cl assi f i ed as i nheri ted
and devel opmental condi ti ons. The metabol i c bone di seases di scussed i n thi s chapter
are gi ganti sm, acromegal y, osteoporosi s, and osteomal aci a. Hyperparathyroi di sm i s
essenti al l y an endocri ne di sease but has bony mani festati ons i n the jaws that are of obvi ous
i mportance.
The screeni ng procedure that i s l i kel y to provi de the f i rst evi dence of a metabol i c bone
abnormal i ty i s an esti mati on of serum cal ci um, phosphorus, and al kal i ne phosphatase. Tabl e
18. 3 gi ves detai l s of the l evel s i n the condi ti ons menti oned i n thi s chapter. When i nterpreti ng
the resul ts for al kal i ne phosphatase i t i s i mportant to be aware that there can be wi de
vari ati ons dependi ng upon the age of the pati ent and the acti vi ty of the di sease.
Inherited and developmental disturbances
Cleidocranial dysplasia
In cl ei docrani al dyspl asi a (dysostosi s) there i s an abnormal i ty of membrane bone f ormati on.
The changes observed are l ack of cal ci f i cati on of the cl avi cl e, fl atteni ng of the frontal bone,
and the presence of a number of supernumerary teeth. These teeth are often of compl ex
form, resembl i ng uni ts of the normal denti ti on, and frequentl y remai n unerupted (Fi g. 18. 2).
It may appear that the pati ent i s suf feri ng from hypodonti a because of the fai l ure to erupt of
l arge numbers of teeth wi thi n the jaws. Radi ographs of the skul l (showi ng wormi an bones)
and cl avi cl es (demonstrati ng cl avi cul ar apl asi a or hypopl asi a) shoul d confi rm the di agnosi s.
The absence of cl avi cl es enabl es pati ents to bri ng thei r shoul ders

forward to approxi mate i n the mi dl i ne. A f l attened nasal bri dge and a hi gh-arched narrow
pal ate may al so be present.
Fibrous dysplasia
Fi brous dyspl asi a i s a f i bro-osseous l esi on of the bone and may i nvol ve one (monostoti c) or
several (pol yostoti c) bones i n the body. The cause of the condi ti on i s unknown and i t i s
general l y regarded as a devel opmental di sorder. Di agnosi s i s based upon cl i ni cal symptoms,
radi ol ogi cal appearance, bi ochemi cal i nvesti gati ons, and possi bl y hi stopathol ogy.
Fibrous dysplasia
Monostoti c fi brous dyspl asi a i s essenti al l y a si ngl e l esi on af fecti ng the jaws wi thout any
other skel etal or other general i zed abnormal i ty.
Pol yostoti c fi brous dyspl asi a i s a general i zed condi ti on that may aff ect the jaw bones.
MONOSTOTIC FIBROUS DYSPLASIA
Thi s i s a condi ti on that may ari se i n ei ther mal e or f emal e pati ents and i s associ ated wi th
very l i ttl e other di sturbance of bone or any other ti ssue. It i s more common than the
pol yostoti c f orm. The l esi ons occur more often i n the maxi l l a than i n the mandi bl e. The
essenti al change i n thi s condi ti on i s the repl acement of the normal bone archi tecture by a
parti al l y cal ci fi ed fi brous mass wi th a hi stol ogy suggesti ng an accel erati on of the normal
bone metabol i sm of osteocl asi s and osteogenesi s. The degree of ossi fi cati on of the l esi on i s
wi del y vari abl e. Ti ssue removed from a l esi on may vary from a very sof t and haemorrhagi c
speci men to a rel ati vel y hard and wel l ossi fi ed ti ssue. Thi s process i s not associ ated wi th any
general i zed change. In parti cul ar, the accepted bl ood chemi stry determi nants (cal ci um,
phosphorus, and al kal i ne phosphatase) remai n unchanged. In the case of wel l -demarcated
l esi ons of fi brous dyspl asi a, there i s a great deal of di scussi on as to whether these represent
beni gn, neopl asti c changes. Thi s questi on has been, to some extent, si destepped by the
P. 224
adopti on of the term fi bro-osseous l esi on.
The characteri sti c feature of fi brous dyspl asi a i s of an otherwi se symptoml ess swel l i ng of the
mandi bl e or maxi l l a (Fi g. 18. 9). In the case of the maxi l l a, the swel l i ng may encroach on the
antral cavi ty (Fi g. 18. 10). There i s vi rtual l y no other compl ai nt and al l i nvesti gati ons of a
bi ochemi cal nature prove to be unproducti ve. The onl y usef ul i nvesti gati on i s radi ography,
al though the appearance of fi brous dyspl asi a may be very vari abl e. In general , the basi c
pathol ogi cal process of decal ci f i cati on and recal cif i cati on i s ref l ected i n a mottl ed appearance
of the bone on radi ography. Thi s, however, depends on the stage and the rapi di ty of the
process. In those pati ents i n whom the process i s sl ow and i ncl udes a si gni fi cant el ement of
cal ci f i cati on, the mottl i ng wi l l be mi ni mal and the expanded bone wi l l have an al most normal
appearance. If the decal ci fi cati on of bone i s predomi nant at the ti me of radi ography, then
the radi ographi c appearance wi l l refl ect thi s fact.
Fig. 18.9 Fi brous dyspl asi a. A monostoti c l esi on of the ri ght maxi l l a.
Management of monostoti c fi brous dyspl asi a shoul d be hi ghl y conservati ve. There i s no
medi cal treatment avai l abl e and, si nce the l esi ons progress to a sel f -l i mi ti ng stati c phase
over a few years, i t i s al most al ways better to awai t events before carryi ng out surgery. The
extent of thi s surgery shoul d be determi ned enti rely by cosmeti c factors. Si nce neopl asti c
change i s vi rtual l y unknown i n thi s condi ti on, there i s no need to attempt to remove the
whol e of the l esi on and, i n fact, thi s i s often an al most i mpossi bl e task. It i s general l y
accepted, therefore, that si mpl e cosmeti c contouri ng of the faci al skel eton i s best carri ed out
after growth i s compl ete.
POLYOSTOTIC FIBROUS DYSPLASIA
Thi s condi ti on i s very much l ess common than monostoti c fi brous dyspl asi a. It i s vi rtual l y
al ways associ ated wi th wi despread changes throughout the skel eton and i n other systems of
the body

that are col l ecti vel y known as Al bri ght' s syndrome. In thi s condi ti on areas of bone
throughout the body are repl aced by f i brous ti ssue wi th wi del y di fferi ng amounts of new
ossi fi cati on i ncl uded wi thi n them. Thi s often l eads to mul ti pl e fractures and to gross
di storti on of the skel eton. In Al bri ght' s syndrome, the bony l esi ons are associ ated wi th
patchy mel anoti c ski n pi gmentati on (caf -au-l ai t spots) and, i n the case of femal es, sexual
Fig. 18.10 Fi brous dyspl asi a. Radi ograph of l esi on i n the l eft maxi l l a.
P. 225
precoci ty. Thi s condi ti on i s a far more acti ve one than that of monostoti c fi brous dyspl asi a
and thi s i s refl ected i n the bl ood chemi stry changes. In pol yostoti c fi brous dyspl asi a the
serum al kal i ne phosphatase i s of ten greatl y el evated, as i n the serum cal ci um. Thi s i s i n
contrast to monostoti c fi brous dyspl asi a when no such changes can be shown.
Albright' s syndrome
Pol yostoti c fi brous dyspl asi a
Mel anoti c ski n pi gmentati on
Precoci ous puberty i n femal es
Metabolic and endocrine disorders
Gigantism and acromegaly
Hypersecreti on of growth hormone i s usual l y associ ated wi th an adenoma of the anteri or
pi tui tary gl and. The cl i ni cal mani festati ons of the condi ti on are dependent upon the ti me of
onset of the hypersecreti on. In chi l dren wi th open epi physes, gi ganti sm occurs, l eadi ng to
general i zed overgrowth of the skel eton, organs, and soft ti ssues. When the epi physes have
fused, overproducti on of growth hormone causes acromegal yonl y bones wi th the potenti al
for growth wi l l enl arge. Acromegal y i s accompani ed by renewed growth of the mandi bul ar
condyl e, hands, and feet wi th overgrowth of some soft ti ssues. Condyl ar growth resul ts i n
mandi bul ar prognathi sm. As a consequence of thi s, teeth, when present, become spaced.
Changes occur i n the faci al ti ssuesthe l i ps and nose become thi ckened, resul ti ng i n a
coarseni ng of the face. The tongue may al so enl arge (macrogl ossi a). Acromegal y i s al so
accompani ed by seri ous systemi c compl i cati ons such as, rai sed i ntracrani al pressure,
headaches, bl i ndness, hypertensi on, cardi omyopathy, and di abetes mel l i tus. The orofaci al
features of acromegal y are summari zed i n Tabl e 18. 4. An enl arged tongue i s a commonl y
reported feature of acromegal y. The denti st may therefore have an i mportant rol e to pl ay i n
the di agnosi s of the di sease (see Chapter 6). Rarel y, pati ents wi th acromegal y may present
wi th faci al pai n.
Treatment of acromegal y depends upon the cause of the condi ti on. Ideal l y, growth hormone
l evel s need to revert to normal . Treatments i ncl ude surgery (usual l y transphenoi dal resecti on
of the tumour) and radi otherapy to the tumour. Drug therapi es (for exampl e, bromocri pti ne
Table 18.4 Acromegaly: orofacial features
Macrogl ossi a
Faci al pal sy
Mandi bul ar growth
Faci al pai n
Temporomandi bul ar joi nt pai n
Mal occl usi on
Procl i nati on of anteri or teeth
Increased i nterdental spaces
Posteri or and anteri or cross-bi tes
Hypercementosi s
Coarseni ng of faci al features
Ski n hyperhi drosi s, acne
or somatostati n anal ogues such as octreoti de) may al so be used. Surgery may al so be
requi red to correct some of the skel etal and dental consequences of the di sease.
Hyperparathyroidism
Hyperparathyroi di sm i s caused by oversecreti on of parathormone (PTH). Parathormone
mobi l i zes cal ci um from bone to i ncrease the serum cal ci um l evel s. Thi s i s achi eved by
i ncreased i ntesti nal absorpti on of cal ci um and i ncreased reabsorpti on of cal ci um by the renal
tubul es, but mostl y by i ncreased osteocl asti c bone resorpti on.
Pri mary hyperparathyroi di sm i s usual l y due to an adenoma of the parathyroi d gl ands.
Secondary hyperparathyroi di sm i s i ncreasi ng i n preval ence and i s a consequence of chroni c
depressed pl asma cal ci um l evel s. Thi s i s most frequentl y due to chroni c renal f ai l ure
(Chapter 13) or l ongstandi ng mal absorpti on (for exampl e, coel i ac di sease; see Chapter 12).
The management of pri mary hyperparathyroi di sm i s usual l y surgi cal . The treatment of
secondary hyperparathyroi di sm i s dependent upon treati ng the cause.
PRIMARY HYPERPARATHYROIDISM
Pri mary hyperparathyroi di sm may be mi l d and asymptomati c but i n severe cases may be l i fe-
threateni ng due to uncontrol l ed hypercal caemi a and renal f ai l ure. The condi ti on i s frequentl y
di agnosed i nci dental l y when cal ci um i s measured as part of a bi ochemi stry profi l ethi s i s
general l y before there i s extensi ve destructi on of the bone. Cyst-l i ke, osteol yti c swel l i ngs
(brown tumours) of the jaws can devel op; these are hi stol ogi cal l y i ndi sti ngui shabl e from
gi ant cel l granul omas of the jaws. Radi ographi cal l y, they are wel l defi ned radi ol ucenci es and
may be mul ti l ocul ar. They occur more f requentl y i n the mandi bl e and the maxi l l a. In
hyperparathyroi di sm there may be l oss of the l ami na dura and general i zed raref acti on of the
jaw bones.
The di agnosi s shoul d be confi rmed wi th i nvesti gati on of bl ood chemi stry, whi ch reveal s rai sed
pl asma cal ci um and rai sed PTH. The pl asma phosphate l evel i s of ten l ow but may be normal ,
parti cul arl y i f there i s an el ement of renal f ai l ure. Pl asma al kal i ne phosphatase i s onl y rai sed
when there i s bone i nvol vement.

Hypoparathyroidism
Thi s condi ti on i s usual l y i atrogeni c, fol l owi ng removal of the parathyroi d gl ands. Earl y-onset
hypoparathyroi di sm can af fect cal ci fi ed ti ssues and resul t i n hypopl asti c enamel , short roots,
and i ncompl ete hypomi neral i zati on of denti ne (see earl i er secti on on teeth). Al l of these
changes are due to hypocal caemi a.
Osteoporosis
Osteoporosi s i s a condi ti on i n whi ch the skel etal bone structure undergoes degradati on both
of bone matri x and cal ci um, that i s, a reducti on i n bone mass per uni t vol ume. Serum
bi ochemi stry i ndi ces are normal . There i s skel etal rarefacti on and l ow trauma fractures often
occur, i ncl udi ng vertabral fractures. Osteoporosi s i s probabl y the most common di sease of
bone. Women are more l i kel y to be af fected than men. In the edentul ous pati ent wi th
osteoporosi s the resi dual al veol ar ri dges may resorb more rapi dl y. Osteoporosi s may l i mi t
the si tes avai l abl e f or i mpl ant pl acement and adversel y af fect the prognosi s of endosseous
i mpl ants. Conversel y, the presence of functi oni ng impl ants may reduce the rate of
progressi on of osteoporosi s.
There are many ri sk factors that have been i denti f ied: i ncreasi ng age, postmenopause
(especi al l y earl y menopause); steroi d therapy (Chapter 3); hyperthyroi di sm and pri mary
hyperparathyroi di sm; and i mmobi l i zati on. The di sease occurs most commonl y i n
postmenopausal f emal es, and hormone repl acement therapy can be hel pful i n preventi on and
P. 226
treatment. Bi phosphonate therapy i s wi del y used and has been shown to i ncrease bone
densi ty and reduce fracture ri sk.
A wel l documented i atrogeni c cause of osteoporosi s i s systemi c steroi d therapy and 50 per
cent of pati ents on predni sol one (5 mg/day or more) for more than 3 months of each year
are l i kel y to devel op osteoporosi s. The prophyl axi s of osteoporosi s for pati ents on l ong-term
steroi d therapy i s f ul l y di scussed i n Chapter 3.
Rickets and osteomalacia
Ri ckets i s a condi ti on that occurs i n chi l dren as a resul t of defi ci ent cal ci f i cati on of the bones
and (unusual l y) the teeth. It i s essenti al l y due to l ack of vi tami n D, because of ei ther
nutri ti onal defi ci ency, mal absorpti on, or i mpai red metabol i c processes. In thi s condi ti on the
bones are poorl y f ormed and, as a resul t, badl y shaped. It i s sai d that the teeth are not
aff ected i n thi s condi ti on, but i n fact, some pati ents wi th a hi story of ri ckets have si gns of
hypopl asi a of the teeth (Fi g. 18. 11). Osteomal aci a i s the adul t equi val ent of thi s condi ti on,
that i s, defecti ve bone mi neral i zati on i n bone that has stopped growi ng. Osteomal aci a may
occur i n pregnancy (parti cul arl y i n those of Asi an ori gi n), i n mal absorpti on (such as i n
coel i ac di sease), or i n renal di sease. There i s a fai l ure of mi neral i zati on duri ng normal bone
turnover. Cal ci um pl asma l evel s tend to be l ow, phosphate l evel s can be normal or l ow, and
al kal i ne phosphatase l evel s are rai sed. Osteomal aci a i s rarel y di agnosed i n the oral medi ci ne
cl i ni c.
Disorders of unknown aetiology: Paget's disease
Paget' s di sease (ostei ti s deformans) i s a wi despread condi ti on of ol d age and i s f ound i n a
l arge proporti on of ol der pati ents at autopsy. Radi ographi c si gns of Paget' s di sease may be
seen i n about 3 per cent of the popul ati on aged over 40 yearsmost wi l l be asymptomati c.
Paget' s di sease i s si mi l ar to fi brous dyspl asi a, i n that both represent an i mbal ance of the
osteogeni c and osteol yti c processes occurri ng i n bone f ormati on. Acti vi ty i s not rel ated to
physi ol ogi cal requi rements and the fi nal resul t i s bone growth. Paget' s di sease can affect any
Fig. 18.11 Dental hypopl asi a i n a pati ent wi th a hi story of ri ckets.
bone i n the bodythose most commonl y af fected are the pel vi s, spi ne, sacrum, femur,
ti bi a, and skul l . It often occurs i ni ti al l y i n the skul l and f aci al bones. The cl assi c compl ai nt of
a pati ent i s that hi s or her hat has become too ti ght, but an equal l y common compl ai nt i s of
dentures that are becomi ng too smal l . The bone growth i n Paget' s di sease
parti cul arl y affects the vaul t of the skul l and maxi l l a, al though the mandi bl e may al so be
i nvol ved (Fi g. 18. 12). The expansi on of the bone of the base of the skul l l eads to cl osure of
the forami na and resul tant neurol ogi cal changes such as deafness. Nerve compressi on may
al so l ead to neural gi a-l i ke symptoms i n the tri gemi nal nerve. Thi s shoul d al ways be
consi dered as a possi bl e di agnosi s i n ol der pati ents, parti cul arl y when there are other
symptoms such as deafness. Apart from neural gi a-l i ke symptoms the pati ent may compl ai n of
pai n wi thi n the bone i tsel f . Thi s i s a common symptom known as bone pai n. Radi ographi cal l y,
the bone i s reputed to have the appearance of cotton wool . There i s l oss of normal bone
trabecul ati on and, i n l ater stages, areas of scl erosi s can be seengi vi ng ri se to the cotton
wool appearance. Si nce cementum i s essenti al l y bone, thi s i s al so aff ected by the changes of
Paget' s di sease, and hypercementosi s i s a common findi ng i n these pati ents. Mal i gnant
change i n the affected bone i s a reputed compl i cation, al though the i nci dence of
osteosarcoma i s l ow and usual l y occurs i n pati ents wi th pol yostoti c Paget' s di sease. Cardi ac
fai l ure because of hi gh output i nto the expanded bl ood spaces of the bone i s a wel l -recorded,
but rare compl i cati on.

The cause of Paget' s di sease i s unknown. Vi ral and geneti c factors have been i mpl i cated.
Fig. 18.12 Paget' s di sease. Radi ograph showi ng expansi on of the skul l and cotton
wool appearance of the bone.
P. 227
Di agnosi s of Paget' s di sease i n the f i rst i nstance i s cl i ni cal , conf i rmed by i magi ng
(radi ography and a radi oi sotope bone scan) and by bl ood chemi stry, the characteri sti c
fi ndi ng bei ng an i ncreased (someti mes greatl y i ncreased) serum al kal i ne phosphatase l evel .
Other bone markers are often abnormal i n Paget' s di sease. For exampl e, uri nary
deoxypyri di nol i ne i s el evated i n acti ve Paget' s di sease and f al l s i n response to treatment.
Pati ents wi th suspected Paget' s di sease are usual l y referred to a speci al i st i n metabol i c bone
di sease. Bi sphosphonates (for exampl e, pami dronate or ri sedronate) are very effecti ve i n the
treatment of Paget' s di sease because of the i nhi bi tory eff ects thi s cl ass of drug has on
osteocl asts. The net resul t i s a reducti on i n bone turnover and, consequentl y, i n progressi on
of the di sease.
There are a number of i mportant dental consi derati ons when treati ng pati ents wi th Paget' s
di sease. Extracti on of teeth may be di ff i cul t because of hypercementosi s and there may be
profuse postoperati ve haemorrhage because of the i rregul ar and i ncreased bl ood suppl y to
the new bone. Paget' s di sease shoul d, i f possi bl e, al so be rendered bi ochemi cal l y i nacti ve by
bi sphosphonates pri or to surgery, as thi s wi l l reduce the vascul ari ty of the af fected bone.
Pati ents wi th thi s condi ti on are al so more suscepti bl e to i nfecti on fol l owi ng any form of
i nterventi on and any extracti on or oral surgery procedure shoul d be covered by anti bi oti c
prophyl axi s. These probl ems of haemorrhage and i nf ecti on l ead to great cauti on i n taki ng
bi opsi es i n Paget' s di sease. Si nce thi s i s a condi ti on that can be di agnosed by non-
i nterventi ve means, the questi on of confi rmatory bi opsy shoul d be approached wi th great
reserve. A summary of the sal i ent cl i ni cal features of Paget' s di sease i s gi ven i n Tabl e 18. 5.
Pri or to exodonti a Paget' s di sease shoul d be rendered chemi cal l y i nacti ve by
bi sphosphonatesthi s wi l l reduce the vascul ari ty of the aff ected bone.
Table 18.5 Summary of clinical features in Paget's
disease
Predomi nantl y a di sease of l ater l i fe, rare under 40 years, often asymptomati c
Resul ts i n errati c i ncreased bone growth, bone densi ty, and deformati on
Skul l frequentl y aff ected
Hat si ze becomes too smal l
Crani al nerve defi ci ts possi bl e
Serum al kal i ne phosphatase may be el evated
Radi ographi c features
Cotton wool appearance
Hypercementosi s, possi bl e root resorpti on
Further dental consi derati ons
Appearance of di astemas between teeth, occl usal derangement, l i p i ncompetence
Dentures become too ti ght
Possi bi l i ty of di ffi cul t extracti ons
Ri sk of postextracti on bl eedi ng and/or i nfecti on
Can cause f aci al pai n or paral ysi s
Q1 What are the di fferenti al di agnoses for thi s boy' s dental condi ti on?
The opaci ti es are present on most teeththeref ore i t i s unl i kel y that thi s i s chronol ogi cal
hypopl asi a. The most l i kel y di agnosi s of thi s 14-year-ol d' s condi ti on i s dental fl uorosi s, but
the denti st needs to consi der geneti c causes such as amel ogenesi s i mperfecta.
On rare occasi ons, i t may not be possi bl e to di ff erenti ate between fl uorosi s and amel ogenesi s
i mperfecta. However, the i nformati on gai ned by asking the questi ons bel ow usual l y al l ows a
def i ni ti ve di agnosi s to be made.
1. Was the pri mary denti ti on af fected?
2. Do any other members of the f ami l y have a si mi l arl y af fected denti ti on?
3. Has the pati ent l i ved i n an area where the water suppl y was fl uori dated or had a
natural l y hi gh fl uori de l evel ?
4. Were fl uori de suppl ements used duri ng the peri od of cal ci fi cati on of the crowns of the
teeth?
The presence of di scol orati on i n the pri mary denti ti on woul d support geneti c i nfl uences,
al though the pri mary denti ti on i s not i nvari abl y aff ected. A heredi tary condi ti on i s al so
supported by the occurrence of the condi ti on i n a fami l y member who bel ongs to a di f ferent
generati on or l i ves i n a di f ferent geographi cal area. The presence of enamel opaci ti es i n
si bl i ngs i s not a hel pful di scri mi nator.

Dental fl uorosi s i s a common enamel defect, resul ting from an i ncrease i n concentrati on of
fl uori de i n the mi croenvi ronment of amel obl asts duri ng enamel formati on. Fl uorosi s therefore
resul ts from systemi c i ntake of fl uori de duri ng enamel formati on. Investi gati on of the
fl uori de hi story i s essenti al . Level s of fl uori de i n the water suppl y can be obtai ned f rom the
appropri ate water suppl i er. It i s al so possi bl e that fl uorosi s coul d be due to fl uori de
suppl ements (toothpaste; fl uori de drops, tabl ets, or mouthwashes).
Tooth wear usual l y has a mul ti f actori al aeti ol ogy. The cl i ni cal si gns decri bed i n case 18. 2,
however, are hi ghl y suggesti ve of dental erosi on. Thi s does not negate the possi bi l i ty of
nutri ti on and abrasi on bei ng i mportant co-destructive aeti ol ogi es. A ful l medi cal and dental
hi story, i ncl udi ng a di etary anal ysi s wi l l be requi red to try to i denti f y i f the aci d i s di etary or
gastri c i n ori gi n. The i ntake of food and beverages wi th erosi ve potenti al needs to be
assessed. A hi story of aci d refl ux and vomi ti ng shoul d al so be expl ored. It shoul d be
remembered that gastri c refl ux may be si l ent (asymptomati c) and that pati ents may not
admi t to sel f-i nduced vomi ti ng.
A pati ent who presents wi th dental erosi on, enl arged paroti d gl ands, and general i zed muscl e
wastage i s l i kel y to have an eati ng di sorder, and bul i mi a nervosa shoul d be suspected.
Projects
1. Li st the di sorders of teeth that you are l i kel y to see i n pati ents. Di vi de these di sorders
Q2 What addi ti onal i nformati on do you need to hel p you make a defi ni ti ve di agnosi s?
P. 228
Q1 What are the possi bl e causes of tooth wear i n this case?
Q2 What condi ti on woul d you suspect i f the pati ent al so presented wi th swol l en paroti d
gl ands and appeared to have general i zed skel etal muscl e wastage?
i nto: (a) those wi th a prenatal and postnatal aeti ol ogy; (b) those that are congeni tal or
acqui red condi ti ons.
2. Identi fy the di ff erent causes and cl i ni cal presentati ons of tooth di scol orati on.
3. Undertake a l i terature search usi ng key words Paget' s di sease and
denti stry. What are the di f ferent ways i n whi ch pati ents present wi th
undi agnosed symptomati c Paget' s di sease to dental , oral medi ci ne, or maxi l l ofaci al
cl i ni cs? What are the probl ems i n managi ng a dental pati ent who has a di agnosi s of
Paget' s di sease?
> Tabl e of Cont ent s > 19 - Medical emer genci es i n dent i st r y
19
Medical emergencies in dentistry
Li fe-threateni ng emergenci es are rare but can occur at any ti me i n dental practi ce. Denti sts
must be abl e to provi de acutel y i l l pati ents wi th li fe-savi ng measures pri or to the arri val of
speci al i st hel p. It i s essenti al , therefore, that they are trai ned i n the management of medi cal
emergenci es that they mi ght preci pi tate or encounter.
Medical emergencies that may be encountered in dental practice and that
require prompt management
Vasovagal attack
Sei zures
Angi na
Asthma attack
Hypogl ycaemi a
Myocardi al i nfarcti on
Anaphyl axi s
Cardi ac arrest
The prevention of medical emergencies
Medi cal emergenci es are usual l y unexpected but rarel y occur wi thout warni ng. The decl i ne i n
a pati ent' s heal th whi l st i n the dental chai r i s l ikel y to be preceded by si gns and symptoms
and earl y recogni ti on of an unwel l or deteri orati ng pati ent can someti mes abort an acute
probl em.
Denti sts exami ne and treat a popul ati on of pati ents wi th vari abl e heal th status. Advances i n
medi cal care and a trend f or l ongevi ty means that an i ncreasi ng number of medi cal l y
compromi sed pati ents present for dental care. It i s i mportant that the ri sks associ ated wi th
the management of these pati ents are assessed. A detai l ed medi cal hi story i s of paramount
i mportance. When assessi ng the si gni fi cance of a pati ent' s medi cal probl ems, i t i s hel pf ul to
consi der the fol l owi ng questi ons.
What are the eff ects, i f any, of the medi cal condi ti on(s) on the proposed treatment?
What eff ect wi l l the proposed treatment have on the di sease?
Are adverse drug reacti ons or i nteracti ons anti ci pated?
It i s not possi bl e to be f ami l i ar wi th al l si gni f i cant drug i nteracti ons and the cl i ni ci an shoul d
have i mmedi ate access to a regul arl y updated ref erence text, such as the Bri ti sh Nati onal
Formul ary. Another i nval uabl e source of advi ce, i n the UK, i s the Drug Informati on Servi ce,
whi ch has a uni t dedi cated to the management of drug-rel ated probl ems associ ated wi th
dental treatment.
Dental pati ents may become severel y anxi ous and experi ence acute somati c symptoms pri or
to, or duri ng dental treatment. A pani c attack i s an extreme acute mani festati on of thi s.
Physi ol ogi cal and psychol ogi cal stressors may present a seri ous ri sk to a medi cal l y
compromi sed pati ent. Methods of pai n and anxi ety control are of paramount i mportance i n
the management of medi cal l y compromi sed pati ents. For exampl e, i n a dental phobi c who
suff ers f rom hypertensi on and angi na, i t i s i mportant to amel i orate a pati ent' s physi ol ogi cal
response to anxi ety-provoki ng proceduresthe use of sedati on can be benefi ci al i n thi s
si tuati on.
An awareness of potenti al probl ems or compl i cati ons associ ated wi th medi cal condi ti ons can
someti mes prevent a cri ti cal i nci dent. For exampl e, hypogl ycaemi a i s an acute compl i cati on
of both type 1 and 2 di abetes mel l i tus. The ti mi ng of dental appoi ntments shoul d be
di scussed wi th the pati ent so that they are compati bl e wi th the pati ent' s di abeti c
management, al l owi ng adherence to usual eati ng schedul es. If a dental procedure (f or
exampl e, mul ti pl e extracti ons) i s l i kel y to resul t i n di etary restri cti ons, consul tati on wi th the
pati ent' s physi ci an or di eti ti an may, on occasi ons, be advi sabl e. However, i n many di abeti cs,
i t i s the pati ent who has the requi red expert knowledge.
Denti sts use l ocal anaestheti cs on a dai l y basi s and these agents have an excel l ent saf ety
record. It i s, however, i mportant that cl i ni ci ans do not become compl acent about thei r si de-
eff ects and toxi ci ty. The maxi mum recommended dosage of a l ocal anaestheti c must not be
exceeded and cauti on shoul d be exerci sed when usi ng more than one preparati on. It shoul d
al so be remembered that topi cal anaestheti cs such as l i docai ne gel s or mouthwashes wi l l be
absorbed and wi l l , theref ore, contri bute to drug l evel s i n the systemi c ci rcul ati on. The
maxi mum saf e dose of l ocal anaestheti cs shoul d be si gni f i cantl y reduced i n pati ents who
have

concurrent di sease, are on certai n medi cati ons, or are very young or el derl y.
Ci rcul atory col l apse has been reported i n pati ents undergoi ng surgi cal procedures and thi s
has rarel y been attri buted to adrenal i nsuffi ci ency that has resul ted from steroi d therapy.
Some authors advocate that pati ents who are taki ng steroi ds regul arl y or have done so
wi thi n the past 6 months shoul d recei ve a pretreatment dose of steroi d or doubl e thei r dai l y
dose peri operati vel y. The use of steroi d cover i s a contenti ous i ssue and pretreatment
protocol s are not uni versal l y accepted. It i s the authors' opi ni on that the rol e of steroi d
prophyl axi s for dental procedures has, i n the past, been overstated. Evi dence-based research
i s requi red to settl e thi s debate.
The recogni ti on of the unwel l pati ent and the assessment of hi s or her sui tabi l i ty f or
treatment are i mportant ski l l s for a cl i ni ci an to devel op. Denti sts wi l l onl y encounter a
l i mi ted number of emergenci es and al l of these can resul t i n the col l apse of the
cardi ovascul ar system. Therefore, i t i s essenti al that denti sts regul arl y update thei r
knowl edge on the preventi on and management of rel evant medi cal emergenci es. The best
P. 232
When consi deri ng the maxi mum dose of l ocal anaestheti c consi der the f ol l owi ng.
Pati ent factors
Age
Wei ght
Vascul ari ty of the ti ssues bei ng anaestheti zed
Concurrent di sease
The l ocal anaestheti c
Anaestheti c agent
Concentrati on
Manufacturer' s i nstructi ons
Vasoconstri ctor
way of addressi ng thi s i s by havi ng regul ar scenari o trai ni ng wi th al l of the dental team.
Whi l st rehearsal of basi c l i fe support usi ng mani kins i s mandatory for any cl i ni ci an,
respi ratory and cardi ac arrest shoul d not be the onl y emergenci es that are the subj ect of
scenari o trai ni ng. It shoul d al so be remembered that col l apse may occur anywhere on the
premi ses and may affect a member of staff or a compani on of a pati ent. It i s not al ways
possi bl e, therefore, to have i mmedi ate access to the medi cal hi story of the casual ty.
Denti sts have an ethi cal and professi onal duty to be cogni zant of appropri ate gui del i nes for
the management of acute medi cal probl ems that mi ght occurthey al so need to have the
ski l l s and resources to f ol l ow thi s gui dance.
When assessi ng the ri sks that are associ ated wi th treati ng a pati ent i t can be hel pful to
grade the l evel of systemi c di sease that a pati ent has i n terms of how that condi ti on l i mi ts
the dai l y acti vi ti es of a pati ent. The Ameri can Soci ety of Anesthesi ol ogi sts (ASA)
cl assi fi cati on f or fi tness for anaesthesi a can be hel pful i n assessi ng the si gni fi cance of a
pati ent' s medi cal condi ti on (Tabl e 19. 1). Thi s system i s al so used by dental sedati oni sts for
pati ent assessment. It i s unl i kel y that a pati ent who i s ASA I or II wi l l requi re any
modi fi cati ons to dental treatment whi l st a pati ent who i s ASA III may have a si gni fi cant drug
hi story and may be suscepti bl e to potenti al drug i nteracti ons.
Clinical risk management
Cl i ni cal ri sk management i s a systemati c process for the i denti fi cati on, anal ysi s, and control
of adverse events or potenti al ri sks. Cl i ni ci ans shoul d al ways:
take a ful l documented medi cal hi story;
be aware of possi bl e adverse reacti ons (i f unsure seek expert advi ce);
know the pri nci pl es i n the preventi on and management of medi cal emergenci es;
regul arl y rehearse and devel op the management of emergenci es wi th the dental team
and record al l trai ni ng sessi ons;
be fami l i ar wi th thei r worki ng envi ronment;
ensure that regul ar checks are carri ed out on emergency equi pment and the expi ry
dates of emergency drugs; these checks shoul d be recorded.
Table 19.1 Classifications of the American Society of
Anesthesiologists (ASA)
Classification Description
I Fi t and wel l
II Mi l d systemi c di sease that does not i nterf ere wi th day to day
acti vi ty (e. g. wel l control l ed asthma)
III Moderate to severe systemi c di sease that i s l i mi ti ng but not
i ncapaci tati ng. Day to day acti vi ty may be al tered (e. g. wel l -
control l ed i nsul i n-dependent di abetes mel l i tus, angi na pectori s,
chroni c bronchi ti s). The medi cal status may be upset by treatment
Administration of drugs
There are several routes avai l abl e for the admi ni strati on of emergency drugs and these are
expanded upon i n Tabl e 19. 2. It i s worth noti ng that some routes are more usef ul than
others and i t i s the fi rst f our techni ques that have most rel evance to dental practi ce. Drugs
can be admi ni stered vi a an enteral or parenteral route. Enteral routes requi re that a drug i s
absorbed from the gastroi ntesti nal tract. Thi s i ncl udes drugs that are del i vered by buccal ,
oral , or rectal admi ni strati on.
IV Severe systemi c di sease that i s a constant threat to l i fe; the
di sease i s severel y l i mi ti ng and i ncapaci tati ng (e.g. unstabl e
angi na, severe haemophi l i a). The medi cal condi ti on i s unstabl e
and unpredi ctabl e
V Mori bund, not expected to survi ve 24 hours
Table 19.2 Routes of drug administration
Route Onset of
action
Example
Oral 30120 mi n Aspi ri n, gl ucose
Inhal ati on 15 mi n Oxygen, ni trous oxi de, sal butamol
Subl i ngual /buccal 12mi n GTN, gl ucose gel
Intramuscul ar 515 mi n Adrenal i ne (epi nephri ne; for anaphyl axi s)
Gl ucagon
Subcutaneous 1520 mi n Hydrocorti sone
Intravenous 2030 s Adrenal i ne (epi nephri ne; for cardi ac
arrest)
Di azepam
Rectal 620 mi n Di azepam supposi tori es

Routes of drug administration
Oral administration
The onset of acti on of a drug i s usual l y sl ow fol l owi ng oral admi ni strati on and can be
adversel y af fected by the presence of food and the l ow pH. Oral admi ni strati on i s onl y
sui tabl e for the consci ous pati ent and i s not appropri ate for the majori ty of drugs used i n
medi cal emergenci es. There are, however, two acute si tuati ons when the use of an oral l y
admi ni stered drug may prevent seri ous sequel ae. Aspi ri n shoul d be used as soon as possi bl e
i n the post-myocardi al i nf arcti on pati ent and gl ucose shoul d be gi ven i n the consci ous
hypogl ycaemi c pati ent.
Inhalation administration
The onset of acti on fol l owi ng the admi ni strati on of an i nhal ed drug i s very rapi d. Oxygen and
bronchodi l ators such as sal butamol are used successful l y by thi s method. Whereas i n a
consci ous pati ent suppl ementary oxygen i s usual l y gi ven at a f l ow rate of 26 l /mi n, i n
resusci tati on procedures a mi ni mum fl ow rate of 812 l /mi n i s used.
Sublingual and buccal administration
Drugs gi ven by the buccal or subl i ngual routes are absorbed rapi dl y across the mucosa whi ch
i s hi ghl y vascul ari zed. These routes are parti cul arl y useful when a si gni fi cant proporti on of
the drug i s metabol i zed by the l i ver when i t i ni ti al l y enters the hepati c ci rcul ati on. Thi s i s
referred to as the fi rst-pass eff ect. Gl yceryl tri ni trate (GTN) has a si gni fi cant
fi rst-pass eff ect and i s i neffecti ve when swal l owednearl y 100 per cent of i t i s
i mmedi atel y metabol i zed by the l i ver after absorption f rom the al i mentary tract. It i s
essenti al , therefore, that pati ents do not swal l ow tabl ets that are i ntended f or absorpti on
across the oral mucosa.
Intramuscular (IM) administration
The i ntramuscul ar route has a sl ower rate of onset of acti on than i ntravenous (IV)
admi ni strati on and i s greatl y i nfl uenced by the l ocal ti ssue perfusi on. The mi d-del toi d regi on
of the upper arm i s a good si te f or an IM i njecti on because i t i s easi l y accessi bl e and i s wel l
perfused. The tongue i s of ten ci ted as a conveni ent pl ace for the i ntramuscul ar
admi ni strati on of emergency drugs. However, l ocal bl eedi ng and possi bl e swel l i ng coul d
exacerbate ai rway probl ems and, as a consequence, thi s techni que i s not advocated by the
authors.
Intratracheal 15 mi n Adrenal i ne (epi nephri ne)
Intraosseous Up to 5 mi n Adrenal i ne (epi nephri ne)
P. 233
Intramuscul ar admi ni strati on i s the route of choi ce f or adrenal i ne (epi nephri ne) i n
anaphyl axi s. Hydrocorti sone, gl ucagon, and chl orpheni rami ne may be gi ven by the IM route i f
IV access i s not possi bl e. The maxi mum vol ume that can be gi ven at any one i njecti on si te i s
usual l y 5 ml . However, thi s i s unl i kel y to be rel evant to denti sts i n an emergency si tuati on.
Subcutaneous (SC) administration
The subcutaneous route i nvol ves pl aci ng a drug i n the adi pose ti ssue beneath the dermi s.
The onset of acti on of drugs gi ven subcutaneousl y i s much sl ower than for drugs gi ven by the
IM route, because adi pose ti ssue i s usual l y poorl y perfused.
Intravenous (IV) administration
Intravenous access al l ows a drug to be admi ni stered qui ckl y and rel i abl y. It i s the preferred
route of drug admi ni strati on i n advanced l i f e support. The most f requentl y used method of
achi evi ng secure venous access i s wi th a pl asti c cannul a mounted over a needl e. The needl e
i s removed once the cannul a i s correctl y l ocated. The si ze of cannul a depends upon whether
i t i s to be used for rapi d fl ui d admi ni strati on (when a l arge di ameter i s i ndi cated) or f or drug
admi ni strati on al one (i n whi ch case smal l er-gauge cannul ae can be used). In an emergency
si tuati on the l argest vei n and cannul a avai l abl e shoul d be used. A metal butterfl y needl e i s
not recommended, as venous access i s not secure.
The superfi ci al vei ns i n the arm are most frequentl y used to si te an IV cannul a. Intravenous
drug admi ni strati on requi res speci al i st ski l l s and regul ar practi ce. Many denti sts do not
undertake IV cannul ati on on a routi ne basi s and woul d be unl i kel y to gai n vascul ar access i n
an emergency si tuati on when the venous ci rcul ati on may be compromi sed.
Rectal administration
The absorpti on of drugs from the rectum i s si mi l ar to that obtai ned fol l owi ng the
i ntramuscul ar admi ni strati on of a drug. Thi s route can be benefi ci al when a drug i s broken
down by gastri c and i ntesti nal enzymes or i s pH-sensi ti ve, and al so when a pati ent cannot
take medi cati on oral l y. In poorl y control l ed epi l epti cs, especi al l y pati ents wi th l earni ng
di sabi l i ti es, di azepam admi ni stered rectal l y may be used to control sei zures.
Tracheal administration
It i s not al ways possi bl e to obtai n i ntravenous access i n a col l apsed pati ent. Thi s can be
parti cul arl y probl emati c i n i ntravenous

drug abusers and pati ents who have severe hypovol aemi a. Adrenal i ne (epi nephri ne),
atropi ne, and l i docai ne can be successful l y admi ni stered by the i ntratracheal route. It i s
i mpl i ci t i n thi s techni que that the pati ent has been i ntubated wi th an endotracheal tube. A
l aryngeal mask ai rway wi l l not faci l i tate the i ntratracheal admi ni strati on of drugs.
P. 234
P. 235
Table 19.3 Emergency drugs: indications for use and
mechanisms of action*
Drug Indications
(dose)
Mechanism of
action
Comments
Oxygen Any medi cal To suppl ement Oxygen can be used
emergency oxygen i ntake and
prevent cerebral
hypoxi a
i n most emergenci es
Adrenal i ne

(IM) 1:1000
(1mg/ml )
Anaphyl acti c
shock (0. 5mg,
repeated at 5
mi n i nterval s
i f requi red)
Thi s di rectl y acti ng
sympathomi meti c
ami ne has - and
-adrenergi c
acti vi ty. The -
agoni st acti vi ty
reverses
peri pheral
vasodi l atati on and
preserves bl ood
fl ow to essenti al
organs. The -
acti vi ty rel axes
bronchi al smooth
muscl e (di l ates the
ai rways),
i ncreases coronary
bl ood fl ow and the
force of myocardi al
contracti on, and
suppresses
hi stami ne and
l eukotri ene rel ease
IM route i s much
more ef fecti ve than
the SC route
Adrenal i ne

(IV) 1:10 000
(1mg/10ml )
Cardi ac arrest
(1 mg
repeated
every 3 mi n)
Thi s di rectl y acti ng
sympathomi meti c
ami ne has - and
-adrenergi c
acti vi ty. The
objecti ve i s to
i ncrease cerebral
and coronary
perfusi on
IV route preferred
Gl ucagon (IM) Di abeti c
hypogl ycaemi a
(unconsci ous)
(1mg)
Thi s pol ypepti de
hormone i ncreases
serum gl ucose by
mobi l i zi ng
gl ycogen stores
IV or SC routes can
be used, but very
few denti sts are
ski l l ed i n IV access
and absorpti on i s
del ayed wi th the SC
route
Sal butamol
(i nhal er)
Asthma (100
g)
2
-adrenergi c
agoni st acti vi ty
rel axes bronchi al

smooth muscl e
Gl yceryl
tri ni trate spray
(subl i ngual )
Cardi ac/chest
pai n (400 g
metered dose)
Vasodi l atati on of
the coronary
arteri es occurs

Gl ucose (oral ) The consci ous
hypogl ycaemi c
pati ent
Rapi d absorpti on
el evates serum
gl ucose l evel s
Gl ucose gel s f or
rapi d buccal
absorpti on are
avai l abl e
Di azepam (IV) Status
epi l epti cus (10
mg)
Benzodi azepi ne
that faci l i tates the
acti on of -
ami nobutyri c aci d
(an i nhi bi tory
neurotransmi tter)
Cauti on: sl ow IV
i njecti on, ri sk of
respi ratory
depressi on. Other
anti convul sants may
be requi red
Chl orphenami ne
(IV)
Adjuncti ve
treatment for
anaphyl axi s
(1020 mg)
Hel ps reverse
hi stami ne-
medi ated
vasodi l atati on
Sl ow IV i njecti on,
can gi ve IM. Thi s i s
a second-l i ne drug
Hydrocorti sone
(IV)
Adjuncti ve
treatment for
anaphyl axi s
Adrenal shock
(200 mg)
Hel ps reduce l ate
sequel ae of
anaphyl axi s by
reduci ng capi l l ary
permeabi l i ty,
reduci ng l eukocyte
and macrophage
mi grati on and
i nhi bi ti ng the
medi ators of
i nfl ammati on
Can gi ve IM
Aspi ri n (oral ) Myocardi al
i nfarcti on
(150300
mg)
Anti -pl atel et acti on
decreases pl atel et
aggregati on;
anti thromboti c
eff ect reduces
mortal i ty after
cardi ac i nfarcti on
If gi ven, i nform
paramedi cs/hospi tal
staff
Sol vents (e. g.
water)
Sol vents may be
requi red to
di ssol ve drugs that
are presented as

Intraosseous administration
Thi s route i s often used i n paedi atri c resusci tati on. It can be usef ul i n adul ts when no other
method of access i s possi bl e. A speci al cannul a i s usual l y i nserted i nto the medul l ary cavi ty
of the ti bi aaspi rati on of bone marrow i ndi cates correct posi ti oni ng. Admi ni strati on of
drugs must be fol l owed by a f l ush of f l ui d.
Emergency drugs and equipment
There are of ten l ocal , regi onal , and nati onal vari ati ons i n the gui dance i ssued rel ati ng to
whi ch emergency drugs shoul d be kept i n a dental surgery. Cl i ni ci ans shoul d exerci se thei r
professi onal judgement i n the l i ght of current practi ce and the rel evant contemporaneous
gui del i nes and recommendati ons from authori tati ve bodi es. Practi ti oners need to assess the
drugs most appropri ate to thei r needs and thi s wi l l be i nfl uenced by the type of practi ce they
have. For exampl e, addi ti onal drugs wi l l be requi red i f i ntravenous sedati on or general
anaesthesi a i s undertaken on the premi ses.
The cl i ni ci an needs to be fami l i ar wi th the preparati on and admi ni strati on of drugs i n an
emergency si tuati on. A si ngl e drug i s often avai l abl e i n several presentati onsfor exampl e,
hydrocorti sone i s avai l abl e as a powder for reconsti tuti on wi th water or as a l i qui d i n a gl ass
ampoul e or a prel oaded syri nge. Some emergency drugs are avai l abl e i n prel oaded syri nges
and these have several advantages. They requi re the mi ni mum of preparati on (thi s can save
val uabl e ti me), can reduce the possi bi l i ty of operator error, and si mpl i fy trai ni ng protocol s.
It i s prudent to ensure that al l emergency drugs are capabl e of a natural rubber l atex-free
del i very. The frequentl y recommended drugs for use i n the dental surgery i n an emergency
are gi ven i n Tabl e 19. 3. The mechani sms of acti on of the drugs are al so bri efl y summari zed.
There i s meri t i n denti sts onl y carryi ng drugs that are essenti al for the fi rst-l i ne management
of acute medi cal probl ems. It i s the authors' opi ni on that the fol l owi ng drugs are the most
useful , fi rst-l i ne drugs for the i mmedi ate management of an emergency: oxygen, adrenal i ne
(1mg/ml ), sal butamol i nhal er, GTN, gl ucose f or oral admi ni strati on, and gl ucagon. Tabl e 19. 4
l i sts emergency equi pment that shoul d be present i n a dental surgery. Thi s l i st i s a gui de and
i s not i ntended to be exhausti ve.
Thi s chapter has not taken i nto account drugs that are used i n sedati on. If sedati on wi th
benzodi azepi nes i s carri ed out, then fl umazeni l shoul d be readi l y avai l abl e i n case the pati ent
has been oversedated. It shoul d al so be remembered that a combi nati on of 50 per cent
ni trous oxi de wi th 50 per cent oxygen may be a useful anal gesi c and anxi ol yti c i n a pati ent,
post myocardi al i nfarcti on.
powder (e. g.
gl ucagon,
hydrocorti sone)
* Drug protocol s are constantl y bei ng updated and modi fi ed as new sci enti fi c
i nformati on becomes avai l abl e. It i s the duty of the cl i ni ci an to keep up to date
wi th current gui dance. Onl y adul t doses are gi ven in thi s tabl e.

Adrenal i ne i s al so known as epi nephri ne.
It i s the responsi bi l i ty of the denti st to ensure that regul ar checks are undertaken on al l
emergency equi pment and emergency drugs. These checks shoul d be recorded.
Management of emergencies
Tabl e 19. 5 detai l s the management of medi cal emergenci es that may occur i n dental
practi ce. Fai nti ng (vasovagal attack, syncope) i s the most frequentl y encountered cause of
col l apse i n dental practi ce. The bri ef l oss of consci ousness that occurs i n fai nti ng i s due to an
abrupt f al l i n cardi ac output that l eads to a reducti on i n cerebral bl ood f l ow. The dental team
shoul d recogni ze the characteri sti c si gns that precede col l apse and take appropri ate
acti onsometi mes thi s may prevent l oss of consci ousness. It shoul d be remembered that,
occasi onal l y, syncope may not have a beni gn cause and can be associ ated wi th seri ous
cardi ac dysrhythmi as or a transi ent i schaemi c attack (mi ni -stroke).
Al gori thms for cardi opul monary resusci tati on are constantl y bei ng revi sed and the cl i ni ci an
needs to be aware of current protocol s. Tabl e 19. 6 outl i nes the basi c procedure f or the
assessment and management of the col l apsed pati ent. When a pati ent has l ost consci ousness
the cl i ni ci an needs to constantl y assess and moni tor the pati ent' s ai rway, breathi ng, and
ci rcul ati on. Thi s sequence of acti ons i s of ten abbrevi ated to ABC, and thi s acts as an
ai de-mmoi re. Thi s enabl es the cl i ni ci an to qui ckl y ascertai n if the pati ent i s breathi ng and
sti l l has a cardi ac output. If the pati ent has suffered a respi ratory or cardi ac arrest, then
speci al i st hel p i s urgentl y requi red. In the meanti me the dental team must perf orm
cardi opul monary resusci tati on to ensure some cerebral bl ood fl ow. Thi s wi l l hopeful l y prevent
i rreversi bl e hypoxi c brai n

damage. In the event of a cardi ac arrest, a successful outcome i s greatl y i nf l uenced by the
Table 19.4 Emergency equipment required in the dental
surgery*
Pocket mask

Sel f -i nfl ati ng bag, val ve, and mask wi th reservoi r

Oropharyngeal ai rways

Nasopharyngeal ai rways

Oxygen therapy masks
Tubi ng and appropri ate connectors to attach oxygen cyl i nders to oxygen masks
Syri nges and needl es to del i ver drugs by a parenteral route i n an emergency
IV cannul ae and adhesi ve tape
Independentl y powered portabl e sucti on apparatus with wi de-bore aspi rati on ti ps.
Bl ood pressure moni tor
* Equi pment shoul d be free from natural rubber l atex.

These wi l l al l ow the provi si on of i ntermi ttent posi ti ve pressure venti l ati on to the
l ungs.
A range of si zes shoul d be avai l abl e.

Thi s has not usual l y been recommended as an essenti al i tem. However, i t may be
hel pful i n the assessment of the unwel l pati ent.
P. 236
P. 237
P. 238
earl y appl i cati on of advanced l i f e support ski l l s. The majori ty of pri mary adul t cardi ac arrests
present i n ventri cul ar fi bri l l ati on and pati ent survi val i s dependent upon earl y def i bri l l ati on.
Table 19.5 Medical emergencies and their management
Causes Signs Management
Fai nt
Transi ent
hypotensi on and
cerebral i schaemi a
Predi sposi ng
factors i ncl ude
hypogl ycaemi a,
anxi ety, fear, pai n,
and fati gue
Weakness, di zzi ness,
pal l or, sweati ng,
nausea, confusi on,
tachycardi a f ol l owed
by a bradycardi a,
l oss of
consci ousness.
Mi nor convul si ons or
i nconti nence can
occur.
Pl ace pati ent i n a supi ne posi ti on
wi th the l egs el evated above the
l evel of the heart to i mprove
cerebral f l ow
A pati ent who i s si tti ng may
l ower thei r head by pl aci ng i t
between thei r knees, thi s i s not
as effecti ve as l yi ng a pati ent
down.
Lay a pregnant pati ent on her
si de.
Admi ni ster oxygen.
Reassure the recoveri ng pati ent,
a gl ucose-ri ch dri nk may be
hel pful .
When a member of the dental
team recogni zes that a pati ent i s
l i kel y to fai nt the pati ent shoul d
be pl aced i n a supi ne
posi ti onthi s may prevent l oss
of consci ousness.
If the pati ent fai l s to regai n
consci ousness promptl y other
causes of l oss of consci ousness
must be consi dered.
Hypogl ycaemi a
Anxi ety, i nfecti on Col d and cl ammy
ski n, trembl i ng.
Irri tabi l i ty,
confused,
aggressi on, and
uncooperati ve
behavi our.
Drowsi ness and
di sori entati on.
Consci ous: gl ucose dri nk, tabl ets
or gel
Unconsci ous: i ntramuscul ar
gl ucagon (1 mg/ml ) or an IV
gl ucose i nfusi on (25 ml of 50%
sol uti on).
Admi ni ster oxygen
Al ways moni tor pati ent and
mai ntai n ai rway.
Transfer to hospi tal .
Epi l epti c sei zure
Known epi l epti c
Poorl y control l ed or
non-compl i ance
wi th drug regi me
Stress,
hypogl ycaemi a;
may accompany a
fai nt. Overdose of
l ocal anaestheti c
may cause sei zures.
Loss of
consci ousness
Muscl e ri gi di ty
fol l owed by jerki ng
movements;
i nconti nence may
occur.
Confusi on may be
present duri ng
recovery
Protect from i njury (remove
potenti al l y harmful objects, use
pi l l ows around pati ent i f these
ai d thei r protecti on). Admi ni ster
oxygen and mai ntai n ai rway i f
possi bl e. If the pati ent can be
di scharged home, ensure that
they are accompani edthey
mi ght have post-i ctal conf usi on.
Status epi l epti cus i s probabl e i f
sei zure conti nues i n excess of 7
mi nutes; therefore emergency
servi ces shoul d be cal l ed. If
status epi l epti cus i s di agnosed
di azepam (up to 10 mg) by sl ow
IV i njecti on may be gi ven; i t i s
not al ways effi caci ous
Asthma
Pre-exi sti ng di sease
that i s poorl y
control l ed, anxi ety,
i nfecti on, exerci se,
exposure to an
anti gen
Breathl essness wi th
wheezi ng on
expi rati on. If
untreated, breathi ng
may become
i ncreasi ngl y di ff i cul t
Sal butamol i nhal er or nebul i zer
and oxygen. Pl ace the pati ent i n
a comfortabl e posi ti on. If there i s
no i mprovement summon
emergency servi ces
Hydrocorti sone IV or IM may be
gi ven
Status asthmati cus i s a l i f e-
threateni ng condi ti on
Chest pai n
Angi na, myocardi al
i nfarcti on
Usual l y a crushi ng
retrosternal pai n,
i rregul ar pul se, may
experi ence
breathl essness,
nausea, or vomi ti ng
Subl i ngual GTN, oxygen, Pl ace
pati ent i n a comf ortabl e
posi ti onconsul t wi th the
pati ent. In a pati ent wi th a
known hi story of angi na ask i f
the symptoms are typi cal . Cal l
emergency servi ces i f pai n does
not subsi de i n 3 mi nutes
(possi bi l i ty of a myocardi al
i nfarcti on (MI)). Possi bl e
admi ni strati on of oral aspi ri n
(300 mg) i f MI suspected; ni trous
oxi de and oxygen, i f avai l abl e,
can be hel pful to reduce pai n and
anxi ety. Moni tor. If l oss of
consci ousness fol l ow the protocol
for cardi opul monary
resusci tati on.
Hyperventi l ati on
Stress, pai n, or
expectati on of pai n.
Thi s i s of ten a
response to
unf ocused fears.
Can be associ ated
wi th chroni c
general i zed anxi ety
di sorder
Rapi d breathi ng,
tachycardi a,
trembl i ng, di zzy,
fai nt, sweati ng
Paraesthesi a, muscl e
pai n/sti ff ness
Can l ead to tetany.
Pati ents can
compl ai n of chest
pai n
Reassure
Ensure comfortabl e posi ti on
Stop treatment
Rebreathe expi red ai r
Anaphyl axi s
Exposure to an
anti gen to whi ch
the pati ent has
been sensi ti zed,
commonl y drugs
(most notabl y
peni ci l l i ns) or
natural rubber
l atex. Anaphyl axi s
to l ocal anaestheti c
i s extremel y rare
Ini ti al f l ushi ng of the
ski n may occur
fol l owed by oedema
of the head and
neck. Al tered
sensati ons such as
paraesthesi a around
mouth and f i ngers.
Pal l or, cyanosi s wi l l
accompany acute
breathi ng di ff i cul ti es
wi th bronchospasm
and/or severe
hypotensi on. Loss of
consci ousness and
cardi ac arrest can
occur
Lay fl at, el evate l egs
Mai ntai n ai rway and admi ni ster
oxygen
Cal l expert assi stance
Immedi ate adrenal i ne (0. 5 ml of
1:1000) IM; repeat i f necessary
Hydrocorti sone and
chl orpheni rami ne are 2nd-l i ne
drugs
Cerebrovascul ar acci dent
Ischaemi a,
haemorrhage, or
embol i sm i n a
cerebral artery
A stroke (parti al or
total weakness on
one si de of the
body), dysarthri a,
aphasi a, hemi pl egi a,
and possi bl e l oss of
consci ousness
Lay fl at and admi ni ster oxygen,
mai ntai n ai rway, moni tor
Summon expert hel p.
Local anaestheti c toxi ci ty
Overdose Li ghtheadedness;
vi sual or heari ng
di sturbances.
Agi tati on, conf usi on,
sei zures, respi ratory
di stress. Loss of
consci ousness,
respi ratory and
cardi ac arrest.
Admi ni ster oxygen, mai ntai n a
comfortabl e posi ti on
Cal l emergency servi ces
Moni tor pati ent
Adrenal shock (Addi soni an cri si s)
Stress i n a pati ent
who has adrenal
suppressi on (e. g.
i nduced by di sease
or l ong term steroi d
therapy)
Pal l or, rapi d weak
pul se, rapi dl y fal l i ng
bl ood pressure, l oss
of consci ousness
Lay pati ent fl at.
Admi ni ster oxygen, 200 mg
hydrocorti sone (IV i s the
preferred route but IM can be
used)
Summon expert hel p.
Thi s i s rareconsi der other
causes
Respi ratory arrest
Status asthmati cus,
ai rway obstructi on
No breathi ng;
central pul se present
(i ni ti al l y)
Fol l ow basi c l i fe support
al gori thm for rescue breathi ng.
If untreated, wi l l become a
cardi ac arrest
Cardi ac arrest
Myocardi al
i nfarcti on
Ci rcul atory col l apse
Anaphyl axi s
Hypoxi a
Respi ratory arrest
Unconsci ous; no
central pul se
Fol l ow basi c l i fe support
al gori thm; thi s wi l l i nvol ve
assessment of responsi veness,
ai rway, breathi ng, and ci rcul ati on
(ABC). Expert assi stance shoul d
be summoned as soon as possi bl e
to ensure earl y
def i bri l l ati onarrest (i f i ndi cated)
and admi ni strati on of emergency
drug (advanced l i fe support
al gori thms)
Some authori ti es suggest that an IV benzodi azepi ne shoul d be gi ven to the pati ent
who has sei zures fol l owi ng overdose of l ocal anaestheti c. Thi s i s not advi sed
because i t carri es the ri sk of respi ratory depressi on
Table 19.6 Assessment and management of the collapsed
adult patient.
Assess Action Comments
Is the pati ent
consci ous?
If yes, i s expert hel p requi red?
If no, cal l for hel p f rom staff
Ensure that i t i s safe to
approach before
assessi ng the pati ent
Ai rway Establ i sh and mai ntai n ai rway* Is there debri s to
remove from the mouth?
Breathi ng (l ook,
l i sten, and feel )
If breathi ng, i s expert hel p
requi red?
If not breathi ng, cal l
emergency servi ces

and start
arti fi ci al venti l ati on
Ci rcul ati on If yes, conti nue wi th venti l ati on

If no, start external cardi ac
compressi ons
Pal pate a major pul se
(e. g. caroti d pul se)
* An endotracheal tube or l aryngeal mask ai rway shoul d onl y be used i f the
operator i s ski l l ed i n thei r use.

If a person i s avai l abl e send them to meet the emergency team at the entrance
to the bui l di ng or cl i ni c.
Posi ti ve pressure venti l ati ons shoul d i deal l y be undertaken wi th a sel f -i nfl ati ng
bag connected to oxygen. A si ngl e rescuer may prefer to use expi red ai r wi th a
pocket mask attached to an oxygen suppl y.
Projects
1. Pati ents who requi re speci al i st care fol l owi ng thei r medi cal emergency wi l l need to be
transferred to an appropri ate uni t. In the UK paramedi cs wi l l transfer the pati ents.
Speci f y what i nformati on you woul d need to gi ve i n the hand-over of the
pati ent to the emergency/speci al i st care team.
2. Identi fy the expert commi ttees/panel s that i ssue advi ce on the emergency drugs and
equi pment that shoul d be hel d at your dental practice. Ascertai n how frequentl y the
drugs and equi pment are checked i n the cl i ni cal envi ronment i n whi ch you work.
> Back of Book > Resources
Resources
Appendix
These mouthwashes shoul d be kept i n the refri gerator. The shel f-l i f e of mouthwashes i s
approxi matel y 2 weeks.
Chlortetracycline mouthwash
Formul ary. To make 200 ml (10 ml tds), use:
chl ortetracycl i ne, 4 g
muci l age of tragacanth, 50 ml
di sti l l ed water to 200 ml
Moni tor pati ent for oral candi dosi s.
Triamcinolone (plain) mouthwash
Formul ary. To make 200 ml (10 ml tds) use:
tri amci nol one for the concentrati on requi red (see fol l owi ng tabl e)
muci l age of tragacanth, 50 ml
di sti l l ed water to 200 ml
Moni tor the pati ent f or oral candi dosi s. Systemi c absorpti on of thi s mouthwash i s l i kel y,
parti cul arl y i n cases of wi despread oral ul cerati on and at hi gh concentrati on of steroi d.
Triamcinolone with 2 per cent chlortetracycline mouthwash
Formul ary. To make 200 ml (10 ml tds), fol l ow the i nstructi ons f or tri amci nol one pl ai n but
Concentration Amount of steroid
0. 25 mg 5 mg tri amci nol one
add 4 g chl ortetracycl i ne.
Moni tor for oral candi dosi s. Systemi c absorpti on i s l i kel y, parti cul arl y i n cases of wi despread
oral ul cerati on and at hi gh concentrati on of steroid.

Suggestions for further reading and reference
sources
Books
Oral Pathol ogy: JV Soames and JC Southam, 3
rd
Edi ti on, Oxford Uni versi ty Press
(1988).
Medi cal Probl ems i n Denti stry: C Scul l y, RA Cawson, 4
t h
Edi ti on, Wri ght (1998).

Oral Di seases: C Scul l y, S Fl i nt, SR Porter, M Duni tz, 2
nd
Edi ti on (1996).

Col our Atl as of Oral Di seases: G Laskari s Thi eme 2
nd
Edi ti on (1994).

Col our Atl as of Oral Medi ci ne: Wi l l i am R Tyl desl ey, 2
nd
Edi ti on, Mosby-Wol fe (1994).

Essenti al s of Mi crobi ol ogy for Dental Students: T Bagg, TW MacFarl ane, IR Poxton, CH
Mi l l er, AJ Smi th, Oxford Uni versi ty Press (1999).
Textbook of Dermatol ogy: A Rook, DS Wi l ki nson, FGJ Ebl i ng (Edi tor). 5
t h
Edi ti on (4
vol umes) Bl ackwel l Sci enti fi c Publ i cati ons, Oxford (1992).
(NB Pl ease consul t the most up-to-date edi ti on. )
Journals:
Oral Di seases
Oral Oncol ogy
Oral Medi ci ne and Pathol ogy
Oral Medi ci ne, Oral Surgery, Oral Pathol ogy and Oral Radi ol ogy
Cri ti cal Revi ews i n Oral Bi ol ogy and Oral Medi ci ne
Useful Website Addresses:
Cochrane Col l aborati on http://www.cochrane.org
Cochrane Oral Heal th Group contai ns l i nk to OHG abstracts wi thout searchi ng the whol e
Cochrane Li brary http://www.cochrane-oral .man. ac.uk
Cl i ni cal Evi dence http://www.cl i ni cal evi dence. com
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Authors: Field, Anne; Longman, Lesley

Title: Tyldesley's Oral Medicine, 5th Edition

Authors: Field, Anne; Longman, Lesley

* Equi pment shoul d be free from natural rubber l atex.

A range of si zes shoul d be avai l abl e.

Ci rcul ati on If yes, conti nue wi th venti l ati on

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