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Patients with Henoch—Schönlein purpura are sometimes referred for a general surgical opinion because of abdominal pain or gastrointestinal bleeding. Surgical intervention is seldom needed.
A man of 41 was admitted by the medical team with a 4-day history of a purpuric rash covering his legs and buttocks, oedema of the ankles and wrists, generalized arthralgia, abdominal pain, rectal bleeding and vomiting. He was dehydrated and tachycardic; his abdomen was distended but non-tender; bowel sounds were absent; inflamed rectal mucosa was seen by rigid sigmoidoscopy. No free intra-abdominal gas was evident on plain abdominal and chest radiography. These findings were consistent with the diagnosis of ileus secondary to a generalized vasculitis. After two days of conservative management the patient became oliguric and developed proteinuria with deteriorating renal function. Parenteral steroid therapy was instituted and renal and skin biopsies were taken which subsequently confirmed the diagnosis of Henoch—Schönlein purpura (HSP). 4 days after admission his abdominal pain worsened and he developed tenderness with guarding in the right iliac fossa. Laparotomy disclosed hyperaemic small bowel, 2 litres of ascitic fluid and 45 cm of infarcted ileum. The infarcted bowel was resected, a double-barrelled ileostomy was formed and the patient was nursed in the intensive care unit for 48 hours. The ileostomy produced up to 7 litres of fluid per day, requiring careful fluid management; over 6 months the patient had to be readmitted intermittenly for rehydration. Steroids were stopped after 9 months and the stoma was closed 3 months later; he subsequently made a good recovery.
HSP is rare in adults; less than one-third of patients with HSP admitted to hospital are over 20. Typically, an acute phase is followed by intermittent relapses in the following months. The aetiology is unclear and bacteria, viruses, vaccines, drugs, foods and exposure to cold have all come under suspicion. The clinical manifestations are due to deposition of IgA immune complexes on the intima of small blood vessels, leading to complement activation, leukocyte recruitment and destruction of endothelial cells. Skin biopsy reveals a leukocytoclastic vasculitis with necrotic vessel walls containing neutrophils surrounded by fibrin strands.
Abdominal symptoms in HSP are caused by haemorrhage and oedema within the bowel wall and mesentery. They occur in up to 56% of adults and are the presenting feature in 15%1. Pain is typically colicky and poorly localized, sometimes accompanied by vomiting and bloody diarrhoea. In adults, surgical intervention is seldom necessary2, whereas in children HSP is complicated by intussusception (2%), perforation (0.5%) and infarction (0.5%). Only one adult case of intestinal infarction secondary to HSP has been reported to our knowledge; that patient died3.
Early involvement of the surgical team, enabling them to make frequent clinical assessments of the patient and identify deterioration, is probably the best way to avoid abdominal catastrophes in HSP.
We thank Mrs Ambika Anand for her help.