The antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy

Antiphospholipid antibody syndrome is characterized by venous and/or arterial thrombosis and/or pregnancy morbidity associated with antiphospholipid antibodies (aPL), such as anticardiolipin antibodies, anti beta 2 glycoprotein I antibodies and positive lupus anticoagulant test. This syndrome may potentially affects any organ system including the skin. Livedo reticularis is the most frequently observed cutaneous lesion; other lesions, by order of frequency, are ulcerations, digital gangrene, subungueal splinter hemorrhages, superficial venous thrombosis, thrombocytopenic purpura, pseudovasculitic manifestations, extensive cutaneous necrosis and primary anetoderma. Skin lesions are more frequently observed in the catastrophic antiphospholipid syndrome, characterized by widespread microvascular occlusions involving multiple organs simultaneously. Patients with antiphospholipid associated thrombosis should receive long-term oral anticoagulants. The intensity of anticoagulation should be guided according to the nature of the thrombotic event (venous vs. arterial thrombosis). Patients with aPL-associated pregnancy morbidity should be treated with aspirin plus heparin and closely monitored during pregnancy. The treatment of the catastrophic antiphospholipid syndrome remains unsatisfactory. High dose intravenous steroids and parenteral anticoagulation should be supplemented by intravenous gammaglobulin and repeated plasma exchanges using fresh frozen plasma early on in the course of the syndrome.