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

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