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Connective Tissue
Disorders
Lupus Erythematosus
General
autoimmune multisystem disease
prevalence 1 in 2,000
9 to 1; female to male (1 in 700)
peak age 15-25
immune complex deposition
photosensitive skin eruptions, serositis, pneumonitis,
myocarditis, nephritis, CNS involvement
Systemic Lupus
Erythematosus
SLE
Head and Neck Manifestations
Malar rash first sign in 50% (Butter Fly
Rash)
Erythematous maculopapular eruption
after sun exposure
Oral ulceration
3-5% perforated nasal septum
larynx and trachea involvement rare
Vocal Cord thickening, paralysis,
cricoarytenoid arthritis, subglottic
stenosis
acute parotid enlargement 10%
-xerostomia occars
-neuropathy 15%
-discoid lupus
SLE
Treatment
avoid sun exposure
NSAIDS
topical and systemic steroids
antimalarials
low-dose methotrexate
azothioprine, cyclophosphamide restricted
symptomatic treatment
saliva substitutes, Klacks solution
1.
2.
3.
4.
Infection
Trauma
Exposure to cold
Mental stress.
Histopathology of DLE:
a. Liquefaction degeneration of the basal cell layer
b. Hyalinization, Edema & Fibrinoid change below
epidermis.
c. A patchy dermal lymphocytic infiltrate around the
appendages
Symptoms:
- Rash (Patches) +
-
Signs:
- The Face is most commonly affected &
the scalp, ears, nose, arms, legs & trunk to a lesser
extent.
-The circumscribed or discoid type is the most frequent &
occurs particularly on the cheeks, the bridge of the nose,
the ears, the side of the neck & the scalp.
- Unilateral, may be bilateral.
- Permanent alopecia + well defined erythematous
patches, varying in size,
- Scale: when the scale is removed its undersurface on
direct examination shows horny plugs (which have
occupied dilated pilosebaceous canals).
Raynauds phenomenon
D. D. of DLE: 1. Morphea
2. LSA ( Lichen Sclerosus et
Atrophicus):
3. PLE (polymorphic light eruptions).
4. Contact Eczema
5. Seborrheic. Ecz.
6. Psoriasis
7. Rosacea
8. Necrobiosis Lipoidica.
9. Lupus vulgaris (skin TB).
Treatment of DLE:
1. General measures:
The patients should avoid excessive exposure to
sunlight
They should wear a broad brimmed hat &
avoid short- sleeved shirts & shorts.
They should use a sun- screening cream &
lotion. Sunscreens are not for treatment once a
reaction has occurred.
Patients who note exacerbation of their lesions
with the cold should warp up well in the winter
Nifedipine may be helpful in those with
Raynauds phenomenon.
Small dose of sedative in mental stress.
2. Topical therapy: Moderately potent steroid cream
twice daily.
3. Intralesional Cs injects are helpful in resistant cases.
4. Oral antimalarials:
Morphea
Definitions:
Sclerosis:
is diffused or circumscribed INDURATION of the S. C.
tissue
may also involve the dermis, when the overlying
epidermis may be atrophic.
Scleroderma:
- ed depostion collagen in interstitium of small arteries
and CT
- sclerotic skin changes, often multisystem disease
- is sclerosis of the skin occurs in:
1. LE
2. Morphea (Localized or Generalized)
3. Systemic sclerosis
4. L.S.A
5. Dermatomyositis
Localized Morphea:
Clinical Features:
The lesions:
-indurated purplish,
spontaneous, usually multiple, round or oval, 2-15 cm
- lose their color, become thickened & waxy areas, ivory in
color with a characteristic lilac- colored edge, surface is usually
smooth & shiny
- in the lesion the hairs are absent, there will be No sweating
- on the trunk & limbs, face & genitalia.
- activity is usually between 3 5 years & spontaneous resolution
-can be generalized.
Treatment: local steroid, or Intralesional Steroid
* uncommon
*small, white areas on the skin associated with an
atrophic condition of the vulva & perianal skin.
Clinical features:
1- Non genital L S A:
- on the trunk, axillae & flexor surface of the wrists.
- small, ivory white, shiny & round macules or papules,
(a few mms in diameter)
- later atrophy & the surface of the lesions wrinkled.
2- Anogenital LSA in women between 45 60
- Irritation may be marked
- on the vulva & around the anus.
- ivory colored atrophic papules with
follicular hyperkeratosis on the vulva.
DDx:
1. Morphea
2. L.P.
3. DLE.
Dermatomyositis:
Is a disorder mainly
of
in childhood
Calcinosis is frequent & is usually ass. with a
more favorable prognosis for life.
In adults: commonly ass. with an underlying
carcinoma or reticulosis
- Usually the patient 1st notices aching &
weakness of the muscles as difficulty in going
upstairs or rising from a chair or in women
Sjogrens Syndrome
General
immune mediated
destruction of exocrine glands
primary
sicca syndrome- isolated d/o lacrimal and
salivary glands
secondary
sicca complex- assoc. with other CTD
Sjogrens Syndrome
General
prevalence
1% population
10-15% of RA patients
9 to 1 F>M
onset 40-60 yrs
increased risk of lymphoma
perhaps 44 times risk
Sjogrens Syndrome
General
clinical manifestations
xerophthalmia, keratoconjunctivitis
xerostomia
other areas
skin, vagina, genitalia, chronic bronchitis, GI
tract, renal tubules
diagnosis
minor salivary gland biopsy
labs
RF and ANA
SS-A/Ro 60%
SS-B/La 30%
Sjogrens Syndrome
Head and Neck
Manifestations
80% xerostomia,
most prominent
symptom
difficulty chewing,
dysphagia, taste
changes, fissures of
tongue and lips,
increased dental
caries, oral
candidiasis
Sjogrens Syndrome
Head and Neck
Manifestations
salivary
quantificationsalivary
scintigraphy
salivary gland
enlargement
Sjogrens Syndrome
eye complaints
dryness,
burning,
itching,
foreign body
sensation
keratoconjuncti
vitis sicca
corneal
abrasions -
Sjogrens Syndrome
Schirmer I
Schirmer II
nasal crusting,
epistaxis, hyposmia
Diagnostic:
history>
Schirmer I>Schirmer II
or rose bengal staining
or other supportive
evidence:
minor salivary gland
biopsy
Sjogrens Syndrome
Treatment
symptomatic
oral fluid intake
saliva substitutes
artificial tears
avoid
decongestants
antihistamines
diuretics
anticholinergic
Sjogrens Syndrome
Treatment
pilocarpine
clotrimazole/nysta
tin
close dental
supervision
surveillance for
malignancy
Behcets Disease
Vasculitis with triad
oral, genital ulcers,
uveitis or iritis
oral
aphthous-like
painful, clusters on
lips, gingiva,
buccal, tongue
less often palate,
oropharynx
genital
similar in
appearance
Behcets Disease
occular
uveitis, iritis
hypopyon
healing in days
to weeks some
scarring
symptoms
simultaneously,
months apart
Behcets Disease
other findings
tinnitus, vertigo
nasal, laryngeal, tracheal mucosal
ulceration
CNS involvement, bowel dysfunction,
large vessel arteritis
treatment
azothioprine, methotrexate possibly, not
documented
Thank You