Purpura fulminans in systemic lupus erythematosus.

摘要

A 61 -year-old woman with systemic lupus erythematosus (SLE) presented with acute pyelonephritis, secondary to Escherichia coli identified on urinary culture. Multiple blood cultures were negative and abdominal computed tomography scan showed no collections. She was commenced on IV gentamicin and ceftriaxone and showed gradual improvement with decreased temperatures and urinary symptoms.On day 8, the patient became acutely unwell and manifested digital ischaemia in the hands and feet, consistent with purpura fulminans (Fig. 1a,b). Blood tests were consistent with disseminated intravascular coagulation (DIG).Lupus anticoagulant and anti-cardiolipin antibodies were negative. She developed progressive thrombocytopenia (nadir 17 x 10~6) and was treated with low-dose i.v. heparin.Digital ischaemic changes, fevers and the thrombocytopenia progressed. IV methylprednisolone (500 mg) was initiated for treatment of active SLE, which was felt to be causing the DIC. She rapidly unproved with defervescence and increased platelet count (81 x 10~6) and the digital ischaemia was arrested.