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Catastrophic antiphospholipid syndrome is a rare but rapidly progressing form of antiphospholipid antibody syndrome with high mortality. The syndrome causes multiorgan failure associated with diffuse micro-thromboses. Necrotic-appearing ecchymotic lesions emerging from the distal aspect of both lower extremities and progressing to the upper leg region, and to the upper extremities developed in a 58-year-old woman. Histopathological examination of the biopsy specimen revealed intravascular microthrombi in the dermis. Laboratory findings for anti-cardiolipin antibodies IgM and lupus anticoagulant resulted positive. Lung and kidney involvement was observed. The clinical course progressed very quickly, and catastrophic antiphospholipid syndrome was diagnosed with these findings. Two-thirds of catastrophic antiphospholipid syndrome cases develop due to secondary causes, the most common being infections. Catastrophic antiphospholipid syndrome may also accompany autoimmune diseases, particularly systemic lupus erythematosus. This report is presented to emphasize that Sjogren's syndrome should be considered in the etiology of catastrophic antiphospholipid syndrome.

Sjögren sendromu ve şiddetli cilt tutulumu mevcut olan katastrofik antifosfolipid sendromu