Severe primary antiphospholipid syndrome

Antiphospholipid syndrome (APS) is characterised by recurrent venous or arterial thrombosis and/or fetal losses. In APS, the homeostatic regulation of blood coagulation is altered, however, the mechanism of thrombosis is not yet defined and it has varied manifestations. Deep vein thrombosis with or without pulmonary embolism is the most common manifestation followed by arterial occlusion of cerebral, coronary and other arteries including subclavian, retinal, renal and pedal arteries. We report a case of a 42 years old female, with severe primary APS, who presented with symmetrical peripheral gangrene, an uncommon presentation and was treated successfully.