Special presentation of antiphospholipid syndrome

Abstract

Antiphospholipid antibody syndrome (APAS) is a condition of acquired thrombophilia due to autoantibodies directed against membrane phospholipids and/or their cofactors. It may be primary or part of a systemic autoimmune disease, such as systemic lupus erythematosus (SLE). Dermatological lesions during APS are frequent, although non-specific, sometimes inaugural, and may be the only clinical manifestation. However, extensive cutaneous necrosis is rare and treatment is based on anticoagulants and appropriate local care. Herein, we report a case of multiple extensive cutaneous necroses in a female with SLE. The particularity of our case is the presence of two types of lesions, necrotizing plaques surmounted by hemorrhagic bullae surrounded by a purpuric border specific to antiphospholipid syndrome and ecchymotic plaques evolving according to the color of the biligenesis, which may be consistent with coagulopathy, in particular, a protein C or S deficiency, hence the interest in good knowledge and semiological analysis. Key words: Anti-phospholipid antibody syndrome; Extensive skin necrosis; Systemic lupus erythematosus