Abstract

We report a case of progressive peripheral ischaemia and gangrene as a presenting feature of systemic lupus erythematosus. It developed in a previously asymptomatic 40-year-old woman following minor surgery to her toe. Eventually she required a below-knee amputation and despite systemic corticosteroids continued to deteriorate, presenting later with signs of systemic intravascular thromboses. Histopathology and immunofluorescence on vessels repeatedly failed to demonstrate any evidence for vasculitis. A full coagulation screen confirmed the presence of 'lupus' anticoagulant. A plasma exchange was performed to remove circulating immunoglobins and she made a rapid and sustained recovery. Peripheral gangrene has not previously been described in association with lupus anticoagulant. We would suggest that in all cases of systemic thrombosis or unexplained peripheral vascular ischaemia lupus anticoagulant should be considered.