Premium
Antiphospholipid antibody syndrome (Hughes' syndrome)
Author(s) -
Francès Camille,
Piette JeanCharles
Publication year - 2001
Publication title -
dermatologic therapy
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.595
H-Index - 68
eISSN - 1529-8019
pISSN - 1396-0296
DOI - 10.1046/j.1529-8019.2001.014002117.x
Subject(s) - medicine , livedo reticularis , antiphospholipid syndrome , thrombosis , dermatology , catastrophic antiphospholipid syndrome , vasculitis , deep vein , surgery , pathology , disease
A wide variety of dermatologic manifestations have been described in the antiphospholipid syndrome (APS). The most common is livedo reticularis, which is associated not only with cerebrovascular events but also with systemic hypertension and heart valve abnormalities. Its mechanism remains unclear and no treatment is effective. Lower limb ulcers may be related to deep vein or skin vessel thrombosis with clinical features of livedoid vasculitis. The latter frequently persist despite antiplatelet therapy, thus requiring anticoagulation. The pseudovasculitis lesions are frequently misdiagnosed if skin biopsies are not performed, especially in systemic lupus erythematosus‐related APS. Antiplatelet therapy is prescribed in the absence of large vessel thrombosis. Widespread cutaneous necrosis and digital gangrenes may be life threatening. They require full anticoagulation. When these lesions appear concomitantly to multiple vascular occlusions in the setting of “catastrophic” APS, steroids and therapy which can achieve a prompt reduction of antiphospholipid antibodies titers should probably be added to anticoagulation.