Perforation of large and small bowel in Henoch‐Schonlein purpura

A 43‐year‐old woman with an unremarkable medical history was admitted with severe polyarthralgia, purpuric papules on her lower legs (Fig. 1), and recent pain with blue discoloration of her third left finger. She was taking no medication. At the time of presentation the abdominal examination was normal. Laboratory evaluation showed normal or negative values of BUN, creatinine, AST, WBC, Hb, VDRL, rheumatoid factor, HBSAg, ANA, ASO, cryoglobulins, cryofibrinogen, anticardiolipins, anti‐Sm, anti‐RNP, anti‐Ro, anti‐La, anti‐SCl70, and anti‐centromere. Hepatitis C titer was not obtained. Sedimentation rate was 61 mm/h and immune circulating complexes were present (14.2 <1.5μg/ml); normal, <1.5 μg/ml). Urine sediment analysis revealed the presence of cellular, hyalin, granular, and erythrocyteic casts. IgA level was 1.98 g/l (normal, 0.5–3.0 g/l). Skin biopsy showed extensive leucocytoclastic vasculitis. Deposits of IgA, IgM, and C3 were observed in the dermal vessels under direct immunofluorescence microscopy. The patient was put on prednisone 60 mg/day. Three days after admission she complained of acute abdominal pain. Increased abdominal tenderness was noted on examination. An abdominal angiogram was normal. Because of the deteriorating intestinal condition, she received methylprednisolone 1 g IV per day. Despite this treatment she developed acute peritonitis 2 days later. Laparotomy disclosed three perforations on the caecum and ascending colon, and one on the ileum. She underwent sub‐total colectomy with terminal ileostomy. Following surgery she was kept on parenteral methylprednisolone 1 g daily; cyclophosphamide 300 mg daily was started with progressive improvement of her condition. The histopathologic examination of the colonic segment disclosed vasculitis of the medium‐sized vessels (Fig. 2). Direct immunofluorescence microscopy revealed strong deposits of IgA and C3 in the vessel walls of the large intestine (Fig, 3).