[Multiple and extensive skin necrosis revealing anti phospholipid antibody syndrome]

Antiphospholipid antibody syndrome (APS) is an acquired thrombophilic state resulting from autoantibodies activity against phospholipides and/or their cofactors. It may be primary or associated with various diseases, including systemic lupus erythematosus (SLE), which represents a potential severity marker. We here report the case of a young girl with multiple cutaneous necrosis suffering from APS secondary to SLE. Skin lesions associated with APS are common, polymorphic, sometimes inaugural and may be the only clinical manifestation of the syndrome. However, skin necrosis are rare; their treatment is based on anticoagulation therapy and appropriate local care. Rigorous long term follow up associated with closer cooperation between the dermatologist and the internist is justified by unpredictable outcomes and poor prognosis.