Fatal adult Henoch-Schönlein purpura due to small intestinal infarction.

Abstract

A 57-year-old woman presented with classical symptoms of Henoch-Schönlein purpura including arthralgia, abdominal pain, palpable purpuric rash and abnormalities of the urinary sediments. The clinical course was subsequently complicated by severe paralytic ileus leading to respiratory embarrassment. Laparotomy confirmed patchy areas of small bowel infarction due to necrotizing vasculitis. Skin biopsy revealed extensive leukocytoclastic vasculitis with granular deposits of IgA in the blood vessels. Anti-neutrophil cytoplasmic autoantibodies (ANCA) were not detectable. The patient continued to deteriorate despite bowel resection, intensive immunosuppressive therapy and plasma exchange, and eventually died. Gastrointestinal involvement in adult Henoch-Schönlein purpura has previously been reported to have a favourable clinical course. However, our case illustrates the rate but lethal complication of intestinal infarction in patients who fail to respond to conservative treatment.