Sei sulla pagina 1di 32

Autoimmune and

Connective Tissue
Disorders

Connective Tissue Disorders :


The cardinal feature of these conditions is
inflammation of the CT which leads to dermal atrophy
or sclerosis, arthritis and S.T. to abnormalities in other
organs. Basic to them all is a complex array of immune
responses that target or affect collagen or ground
substances.

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

specific labs native(Double


stranded) DNA, SM
antigen
ANA test positive in
95%
LE cells

SLE - Diagnostic Criteria

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

postprandial rinses with H2O2 and H2O

DLE: Discoid Lupus Erythematosus:


Benign
Face (most frequently)
Patches: disc like
various sized , reddish, well- defined, scaly (adherent )

with hyperkeratosis which tend to heal with


atrophy, scarring & pigmentary changes.
Hematological & Serological changes in about
half () the patients,
Female: male is 2: 1
Age of onset is 15 40 years

Precipitating factors: in DLE


Acronym: (ITEM)

1.
2.
3.
4.

Infection
Trauma
Exposure to cold
Mental stress.

* Sunlight is exacerbating factor.

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) +
-

history of Raynauds phenomenon, chilblains or poor peripheral


circulation

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:

Prevalence: 3 Females to 1 male

Localized sclerosis of the skin of unknown cause;

3 clinical types: Circumscribed, Linear & Frontoparietal:


(en coup de saber) with or without hemiatrophy of the
face.

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

Systemic sclerosis: (Systemic


scleroderma):

Face & Hands (most frequently) but the change may


extend proximally to involve the forearms & upper
arms,
Face: facial appearance: is characteristic: - The forehead is smooth & shiny
- The forehead lines are expressless.
- The nose becomes small & pinched.
- The mouth opening is constricted & radial furrows
appear.
- Small, mat like telangiectases are frequently
found on the face, occur in 75% of patients.
Hands & forearms
forearms skin will be thinner than normal.
Atrophy occurs 1st in the pulps of the fingers &
small painful ulcers are formed, heal with scars,
the nails are curving over the atrophic
phalanges.

Lichen sclerosis et atrophicans (L. S.


A): -

* 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

skin, muscle & B.V.s


Characteristic Erythematous & Edematous
changes in the Skin + Muscle Weakness &
Inflammation.

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

The rash in Dermatomyositis is


diagnostic:
- Face: A purplish- red heliotrope
erythema occurs on the face, especially
involving the eyelids, the upper cheeks,
the forehead & the temples associated
with the edema of the eyelids &
periorbital tissues that is why it should be
differentiated from Contact Dermatitis
(CD) of the face.
- Hands: Erythematous changes occur
on the backs of fingers especially over
the backs of the Joints & the

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

Potrebbero piacerti anche