[Extensive cutaneous necrosis as an initial manifestation of secondary antiphospholipid syndrome (APS)]

Diffuse necrotic-hemorrhagic lesions limited to the skin in secondary antiphospholipid antibody syndrome (APS) to systemic lupus erythematosus (SLE) are not frequent. We report the case of a white woman, 37 years of age, hospitalized in September 2007 with a history of psychosis of several years' duration, presenting with polyarthritis and erythematous, maculopapular, bullous skin lesions on the upper and lower limbs, rapidly followed by extensive necrosis and skin ulceration on all four limbs who was diagnosed with SLE and positive lupus anticoagulant. The investigators highlight the occurrence of skin necrosis of catastrophic characteristics, as a possible initial manifestation of secondary APS without systemic vascular involvement that evolved satisfactorily with a combination treatment of anticoagulation and immunomodulation.