Catastrophic antiphospholipid syndrome presenting with multiorgan failure and gangrenous lesions of the skin

Abstract

A 38-year-old woman with a 20-year history of systemic lupus erythematosus and positive anticardiolipin antibodies developed anuria and hypotension. 20 days before, she had discontinued 25 mg prednisone, but not warfarin, on medical advice. 3 days before admission, she developed extensive necrosis and blisters involving both arms and legs and a multiorgan failure. She improved after immunosuppressive and antibiotic therapy, 3 sessions of dialysis and 5 sessions of plasmapheresis. It was decided that she could be discharged after 45 days. Her skin lesions were complicated by several abscesses requiring surgical drainage and finally healed almost completely within 9 months. Catastrophic antiphospholipid syndrome is a distinctly rare dramatic condition characterized by widespread thrombosis of small vessels, which in the present case was likely triggered by the abrupt withdrawal of steroid therapy. It should be borne in mind in cases of multiorgan failure, which does not recognize a well-defined etiology.