Autoimmune connective tissue

disease

1. Lupus erythematosus.
2. Systemic sclerosis.
3. Dermatomyositis.

Lupus Erythematosus.
An autoimmune connective tissue
disease.
It occurs in two main forms which are
Systemic Lupus Erythematosus and
Discoid Lupus Erythematosus.

Systemic Lupus
Erythematosus.
It is a multisystem disoder which may affects
skin, joints, heart, lung, kidney, brain and
haemopoeitic system.

It can affect any one especially childbearing

women.

Although wide range of organ can be affected,

in three quarters of patients, the skin is involved
generally with an erythematous eruption occurring
bilaterally on the face in butterfly distribution.

Mucocutaneous lesions include

oropharyngeal ulceration, diffuse alopecia,

Raynauds phenomenon and
photosensitivity.

Criteria for diagnosing SLE

Malar rash
Serositis
Discoid plaques
Neurological involvement
Photosensitivity
Haematological changes
Arthritis
Immunological changes
Mouth ulcers
Antinuclear antibodies
Renal changes

Investigation.
1. Anti-nuclear antibody.
2. Anti-double stranded DNA.
3. Histology of biopsy.
4. Direct immunofluorescense study.

Discoid LE
Involvement of the skin only.
Located commonly on the face, scalp, neck,

and ears. But can be present in a wide

spread distribution.

Treatment.
Immunosuppressent. Prednisolone and

azathioprine.
Sun block.
Avoid sun exposure.
Topical steroid and oral hydroxychloroquine
for DLE.

Systemic sclerosis.
A multisystem disorder characterized by
inflammatory, vascular and sclerotic
changes of the skin and various internal
organs, especially the lungs, heart, and GI
tract.
Limited Systemic sclerosis and Diffused
Systemic Sclerosis.
Female:male ratio..4:1
Age 30-50

Unknown cause.

Endothelial cell damage leads to

inflammation and fibrosis.

Raynauds phenomenon is frequently the

presenting signs. The skin of the fingers,
forearms, and lower legs becomes tight, waxy
and stiff.

Facial signs include perioral furrowing,

telangiectasia, beak-like nose, and restricted
mouth opening.

Internal organ involvement.

Limited sclerosis.

Limited sclerosis comprise 60%. Patient

usually female, older than diffused sclerosis,
with long history of Raynauds phenomenon
with skin involvement limited to hands, feet,
face and forearms, and high incidence of
anticentrometic antibody.

It includes CREST syndrome and systemic

involvement may not appear for years. Patient

usually die due to other causes.
Calcinosis, Raynauds, Esophageal dismotility,

Sclerodactyly, Telangiectasia

Diffused sclerosis.
Rapid onset and diffused invovement.
Not only hands. Also included trunk and
face, and early onset of internal organ
involvement.
Anticentromere antibodies are uncommon.

FIGURE 14-28 Scleroderma (lSSc):

acrosclerosis A. Hands and fingers are
edematous (nonpitting); skin
is without skin folds and bound down. Distal
fingers are tapered (madonna fingers) B.
Fingers show both
bluish erythema and vasoconstriction (blue and
white): Raynaud phenomenon. Fingers are
edematous, the skin is
bound down. Distal phalanges (index and third
finger) are shortened, which is associated with

calcinosi
s

Investigations.
Anti nuclear antibody.
Autoantibodies react with centromere

proteins or DNA topoisomerase I.

Anticentromeric autoantibodies occur in
21% of dss and 71% of CREST patients.
DNA topoisemerase I antibodies in 33% of
dss and 18% in CREST patients.

Treatment.
Mainly supportive treatment.
Nifedipine can help in raynauds

phenomenon.
Systemic steroid, penicillamine and
immunosuppresant have been used with
little benefit.
Photopheresis.

Dermatomyositis.
Inflammation of skin and muscle gives a

distinctive eruption, with muscle weakness

with varying severity.
Rare. May affect juvenile and adult(>40).
Clinical presentation: skin changes and
muscle weakness.
Underlying malignancy is also found.
The cause is unknown. But autoimmune
mechanisms are proposed. In adult,
underlying neoplasia may be the cause.

The typical eruption is blue discoloration

around the eyelids, cheeks, and forehead

often with oedema. (heliotrope erythema).
Bluish red papules or streaks on the dorsal
aspect of the hands, elbows and knees are
seen, sometimes with pigmentation and nail
fold telangiectasia.
Photosensitivity is common.
An association with malignancy exists in
patient above 40 years. 40% usually with
malignancy of lung, breast, and stomach.

Investigation.
ANA
Define the degree of myositis.(elevation of

creatine phosphokinase).60% of cases.

Aldolase, LDH,
Exclude possibility of underlying neoplasia.

Treatment.
Systemic steroids in moderate doses.
Immunosuppressive: azathioprine