Autoimmune connective tissue disease

Autoimmune connective tissue disease1. 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 SLEMalar rash SerositisDiscoid plaques Neurological involvementPhotosensitivity Haematological changesArthritis Immunological changesMouth ulcers Antinuclear antibodiesRenal changes

Investigation.Anti-nuclear antibody.Anti-double stranded DNA.Histology of biopsy.Direct immunofluorescense study.

Discoid LEInvolvement 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..4:1Age 30-50Unknown 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, TelangiectasiaDiffused 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); skinis without skin folds and bound down. Distal fingers are tapered (madonna fingers) B. Fingers show bothbluish erythema and vasoconstriction (blue and white): Raynaud phenomenon. Fingers are edematous, the skin isbound down. Distal phalanges (index and third finger) are shortened, which is associated with bony resorption.

calcinosisFIGURE 68-25 A. Scleroderma in an African-American man with distal hypopigmentation and depigmentationon bound down skin on 10 tapering fingers. B. Scleroderma in an African-American man withmarked clubbing o fingernails, hyperpigmentation of the skin, tapering of fingers, and ulceration of the fifthdigit with a serpigimou border around granulation tissue and an area of eschar. C. Scleroderma in anAfrican-American woman with widespread atrophic, scarred, hypopigmenterd plaques with brown bordersand some scale on the right hand with contractures. Courtesy of Atlantic Dermatology Conference, 2006.D. Scleroderma. Periungal hypopigmentation and digital pitting at the tips of the fingers in an African-American man. Courtesy of Department of Dermatology,Washington Hospital Center,Washington DC. Scleroderma in an elderlyAfrican-American man with salt and pepper perifollicularhypo- and hyperpigmentation with scaringin an androgenic distribution on the scalp.Courtesy of Department of Dermatology,Washington Hospital Center,Washington DC.19

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.22Dermatomyositis.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.

Heliotrope (reddish purple) erythema of upper eyelids and edema of thelower lids. This 55-year-old female had experienced severe muscle weakness of the shoulder girdle and presentedwith a lump in the breast that proved to be carcinoma25

Investigation.ANADefine 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