The Hand in Autoimmune Systemic Diseases: Epidemiology and Clinical Phenotypes of Pigmented Skin

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Introduction: Systemic autoimmune diseases are non-organ-specific disorders with a heterogeneous clinical presentation. The clinical signs of these systemic diseases are varied on the hands. There are specific cutaneous or vascular manifestations of autoimmune diseases, and rheumatological manifestations such as polyarthritis, tendonitis and synovitis. The aim of this study was to evaluate the epidemiological aspects, clinical phenotypes and immunological abnormalities associated with the hand in autoimmune systemic diseases. Patients and methods: We conducted a cross-sectional, descriptive, multicenter study in the Dermatology departments of Dakar from June 01, 2021 to November 01, 2021.All patients followed for systemic autoimmune disease during the study period were registered. Among them all patients with clinical signs of systemic autoimmune disease on the hands were included in the study. Data entry and analysis were performed using Microsoft Excel 2016 and SPSS version 23. Results: We collected 66 cases of systemic diseases. Hand involvement was noted in 39 cases: systemic lupus erythematosus in 9 cases, systemic scleroderma in 17 cases, dermatomyositis in 2 cases and mixed connective tissue disease in 11 cases. In systemic lupus erythematosus, cutaneous signs included erythema in 2 cases, discoid plaques in 3 cases and annular plaques in one case. Vascular signs included Raynaud's syndrome in 2 cases, purpura in one case, palmar erythrosis in one case and digital necrosis in one case. Nail manifestations included longitudinal melanononychia in 3 cases. Systemic scleroderma was characterized by diffuse sclerosis of the hands in 13 cases, sclerodactyly in 10 cases, mottled achromia in 8 cases, Raynaud's syndrome in 15 cases and stellate pulpal scars in 8 cases. In 2 cases of dermatomyositis, there was transverse band erythema, Gottron's papule, nail set erythema with cuticle hypertrophy and Raynaud's syndrome. In mixed connective tissue disease, the following associations were observed: scleroderma with dermatomyositis in 4 cases, lupus with dermatomyositis in 3 cases, lupus with scleroderma in 3 cases, and Sharp's syndrome with a case of antiphospholipid antibody syndrome.
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