A cutaneous manifestation of antiphospholipid antibody syndrome.

Abstract

Antiphospholipid antibody syndrome is a disease defined by the presence of a hypercoagulable clinical state in association with antiphospholipid antibodies. This syndrome can involve large-vessel thrombosis or thrombotic microangiopathy. Approximately 40% of patients will present with a cutaneous manifestation as the first indication of this disease, with a marked number of patients concurrently developing multiorgan involvement. The authors present a patient with extensive cutaneous necrosis-a devastating sequela of antiphospholipid antibody syndrome. Additionally, multiorgan involvement developed, requiring systemic anticoagulation and supportive care. When medically stable, plastic surgical intervention was necessary to treat extensive areas of cutaneous necrosis. Widespread cutaneous necrosis is a rare but known sequela of this syndrome. Studies have demonstrated that prophylaxis for thrombosis is not effective. However, long-term medical management with oral anticoagulants has been shown to reduce the risk of recurrent thrombosis. Occasionally, plastic surgical intervention is needed to treat the sequelae of this disease. Therefore, knowledge of the presentation and manifestations of this disease is critical in the early detection and prompt treatment of patients to prevent life-threatening consequences of this catastrophic disease process.