Bhecets disease

Bhecets (pronounced betchets) syndrome was first recognize in Turkey

and was originally thougt to be a disease of Mediterrancan origin. However,
alarge number of cases have since been reported in Japan and other countries.
Women are more commonly affected than men. Bhecets syndrome is a rare
disease characterized by a clasical triad of RAS (any of the three clinical
variants), genital ulceration, and inflamatory eye lesion. Other manifestation
include skin, join, neurological, vascular, and intestinal disordes. Up to 90 per
cent of affected patiens have RAS. Genital ulcer are smilar to those of the oral
mucosa and healing may take plae wich scar formation and lead toresidual tissue
loss and deformity smlar to that in the oral cavity. The ocular involvment initially
takes the from of anterior uveitis, a superficial inflamatory lesion of the anterior
part of the eye, which may become more servere in later episodes and, perhaps,
progres to involve other structures of the eye. This may lead to parmanent
damage by scar formation or, even, to blindness.
The scquence of involvement of the oral an genital mucosa is variabel,
and the two may not involved simulataneously. Indeed, rhere may be a
considerabel innterval between the involvement. Ocular involvement, however,
is usually late, ocuring sometimes after many years of intermittent oral and
genital ulceration. In the generalized from of the disease, skin lesion, of various
kind mny appear, themost characteristic being papules that procced tobeing
papules that procced to pustule formation. Is the interesting to note that in some
patients with skin lesions there is a marked skin resction to trauma. The
tendency of sterile blisters to develop at venepuncture sites is kow as
pathergy , and it may be useful as a siagnostic indicator. Pathergy is less
commonly reprtted in UK patients with Bechets disease.
The neurological disease that may occure in these patients is the result of
the appearance of centers of inflamation and necrosis within the central nervous
system. The symptoms are variabel, depending upon the location of the lesion,
but in the early stages they may resemble those of multipel sclerosis. There is a
vasculitis, perhaps complicated by thrombotic episodes, that may be either
localized and minor or involve major vessels. The effect on the joint is that of
non-specific arthropathy. It has been suggested that major and minor criteria
should be used to arrive at a diagnosis of Bechets disease. The major criteria are
oral uleration, general ilceration,eye lesion, and skin lesion. The minor criteria
ainculde lesion of the nervous system, vascular system, joint, gastrointestinal
tract, and pulmonary system. However, there is no agreement as to the number
of type of lesion necessary to arrive at the diagnose. An international working
party has redefined the criteria for the diagnosis of Bechets as the presence of
RAS plus any two of : recurrent genital ulceration, eye lesions, skin lesions or
positive pathergy test. It is the authors viewpont that, as this a progresive
condition, often begining as RAS whitout any other system involvment, it is
impossible at any given time to differentiate eith any degree of accuracy
between uncomplicated RAS and RAS that might eventually proceed to
orogenital ulceration or Bechets disease.

Management
The managemnt of Bechets syndrome usually requires a multidisciplinary
apporoach. However , the local management of oral aphthae in Bechets
syndrome is exctacly that for all other forms of RAS and is smilarly limited in
effect. Systematic therapy is therefore required in most cases and drugs used
include: systemic steroid, azathioprine, cyclophospahamide, cochicine,
ciclosporin, and more recentl, anti-TNF therapy and mycophenolate.
Thalidomide appears to be succesful in some cases eith muocitaneous
involvment but its use is restricted because of its teratogenicity and side- effects
(see above).

Discussion of problem cases

Case 5.1 Discussion
Q1
How would you manage this gentelman and what therapeutic option are
avaialabel?
The history and clinical examination of the ulcers in this case consistent with
major RAS. It is important specifically to enquire about genital ulceration and
other symptoms, ehich might be suggestive of Bechets disease. The short
history of ulceration is, however, suggestie of a recent precipitating cause of
factor, which should be sought. It is important to establish whether the patients
stopped smokeing after his myocardial infarction and then devolped RAS, as
smoking cessation can precipitate RAS in some individuals. Oral ulceration has
been reported in patients on nicocardil, a potassium-channel activator that is
used for unstabel angina, and it is important to rule this out as causes of this
patients oral ulceration. (This must not be stopped before liaising wuth the
cardiologist.) Blood test should be arranged to check for raised inflamatory
markers, heamitinic deficiencies, and anti-endomysial aoutoantibodies. A full
biochemical and immunological profile is also advisabel.
In view of the serverty of RAS and this patiets poor quality of life, some
form of systemic therapy needs to be considered and the options include system
steroids, azathioprine, ciclosporin, thalidomide, and clochicine. In the case of a
young patient with a history of coronary artery disease, long-term prednisolone is
cintraindicated although a short, reducting course of prednisolone may give
short-term relife of ulceration. RAS does, however, eventually recur after
stoppingthe steroid. Topical analgesics may be required and an antifungal should
also be consider, particulary if there is a high oral carriage rate of candida
albicans.
Colchicine has been reported successfu therapy for RAS and azathioprine
may be worht considering, either alone or as a steroid-sparing agent. Another
treatmen option for this male patient is thalidomide, although this is associated

with a number of significant side-effect and the patient will need counselling and
close monitoring.

Q2

How would you manage this dental emergecy?

Ther are a number of factors to be considered when arraging extraction of this

mans tooth, including his medical and drug history (previous myocardinal
infraction, aspirin) and anxiety about dental treatment. He is probably best
treated under local abesthesia, with sedation if required. The aspirin may
predispose to postextraction haemorrhage but this is unlikely to be significant
and there is no indicaion to stop the medication. It is important to be check for
haemostasis after the extraction and, if necessary, suture and/ or pack the
socket. If the patient devalops agina then he should use his glyceryl trinitrate
spray sublingually, if there are more serve cardiac complications, then the
patient must be treated accordingly.

Cae 5.2 Discussion

Q1

How would you manage the patien in your practice?

Although the patient appears fit and well, she should be questioned about any
gut, eye, or skin problems and asked if she has suffered from ulceration. Her
doctor can arrange for blood tests but it is important to diplomatically piont out
that these should ibclude estimation of ferritin, folate, and B 12 levels, as a full
blood count and film are insusfficient. (It is important to establish a good working
relationship with general medical practitioner in your area)
If this patients clinical examination is consistent with a diagnosis of MiRAS
and thee is no inclination of sytemic disease, then there are number of treatment
option availabel that the dentist can prescribe. Analgesic rinses can be helpful,
particularly before meals, and an antiseptic rinse will reduce secondary infection
and aid plaque control, particulary if toothbrushing is painful. Hydrocortisone
pelletes can be used topicaly in the prodormal phase of ulceration and many
resolution of the ulcer. Triamcinolone paste is dificult to apply but may be useful,
if applied last thing at night. Any obivious caouse of mechanical truma due o
broken teeth or dental application should be eliminated, in case these are
precipitating the aphtouse ulcers. More potent topical steroid, in the form of
rinses o inhalers may be reqired. In most cases, these simpel measures can
reduce the discomfort and duration of RAS. It does, however, need to be pointed
out to this patient that there is not, at the prestent, any satisfactory cure for
this conditions and the risks of systemic therapy probably our weight the
benefits in her particular case.

Project

1. Find out whih systemic druhs have been resported as causing oral
ulceration, inclucing RAS.