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Abstract

Background  Primary Antiphospholipid Antibody Syndrome (PAAS) is characterized by detection of antiphospholipid antibodies associated with venous or arterial thrombosis and/or miscarriages by patients with no other associated disease such as systemic lupus erythematosus (SLE). Primary Antiphospholipid Antibody Syndrome has many clinical manifestations of which dermatological ones are probably the most common. The purpose of this study was to determine the frequency of each cutaneous lesion, describing clinical features essential for diagnosis, in patients with Antiphospholipid Antibody Syndrome (AAS) attending Walter Cantídio University Hospital.

Methods  Sixty patients with clinical findings suggestive of AAS were screened, and submitted for clinical and laboratory evaluations including lupus anticoagulant (KCT), anticardiolipin antibodies (IgG and IgM: ELISA), routine laboratory tests and screening tests for possible associated conditions.

Results  Twenty-five cases of primary and 14 cases of secondary AAS were diagnosed by clinical and laboratory evidences. Persistent elevated antiphospholipid antibodies without history of thromboembolic events or miscarriages were demonstrated in 21 patients. Forty percent of the patients with AAS had a cutaneous feature as the major complaint. These were dermographism (15), acrocyanosis (13), urticaria (9), diffuse alopecia (9), livedo reticularis (seven), Raynaud's phenomenon (three), purpura (two), ulcers and necrosis (four), nodules (four), pterygium ungueum (one) and subungual hemorrhage (one).

Conclusions  Dermatological complaints are very frequent in patients with AAS and may be the first clue to the syndrome. Therefore a careful history and detailed physical examination are essential to diagnose AAS. All dermatologists should investigate the possibility of AAS when facing cutaneous findings related to venous or arterial thrombosis or microthrombosis.