Primary Antiphospholipid Syndrome in a Male Presents with Acute Digital Ischemia: Dramatic Response to Glucocorticoid

INTRODUCTIONAntiphospholipid Syndrome (APS) is an autoimmune disease characterized by the presence of antiphospholipid antibodies, namely, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or antibodies to the protein cofactor-β2 glycoprotein I (anti-β2GPI) which cause a hypercoagulable state resulting in recurrent venous, arterial or small vessel thromboembolism and/or pregnancy-related complications. Thrombus can be either venous (59%) or arterial (28%) or both arterial and venous (13%). It was first described by Hughes in 1983, hence also known as Hughes Syndrome. In 1992, another subset of APS (50%). APS is far more prevalent in women than men in the adult age group (5:1) whereas in the pediatric population, the ratio is 1:1. This paper reports a rare case of primary antiphospholipid syndrome in a young male who presented with acute digital ischemia and was successfully treated with a combination of anticoagulation and glucocorticoid.