Henoch-Schonlein purpura is a systemic hypersensitivity vasculitis involving the skin, joints, renal system, and gastrointestinal tract. Methods of treatment of this purpura have not been agreed upon, but corticosteroids and dapsone have been useful in uncontrolled studies [1] of patients with cutaneous and digestive manifestations. Rostoker and colleagues [2] recently reported their experience using intravenous immunoglobulin therapy for severe IgA nephropathy and Henoch-Schonlein purpura. We report the efficacy of intravenous immunoglobulin in severe gastrointestinal manifestations of this condition.

A 56-year-old man was hospitalized for palpable purpura, abdominal pain, and nausea that were associated with arthralgia and swelling of the joints of the knee, ankle, and wrist. The patient had no clinically significant medical history. Physical examination showed epigastric tenderness, and rectal examination showed bloody stool. The patient also had necrotic purpura of the lower extremities and buttocks. Urinalysis showed hematuria without proteinuria.

Examination of skin biopsy specimens showed hypersensitivity vasculitis; results of immunofluorescent staining were positive for IgA in dermis. The patients' hemoglobin level was 10 g/dL, his leukocyte count was 14 700 cells/mm³ (with 85% polymorphonuclear leukocytes and 8% lymphocytes), and his platelet count was 458 000 cells/mm³. Other laboratory measures included sodium level, 128 mmol/L; serum creatinine level, 62 mmol/L; blood urea nitrogen level, 6 mol; and serum albumin level, 28 g/L. No further information was obtained from tests for antineutrophil cytoplasmic, antinuclear antibodies, human parvovirus B19, Epstein-Barr virus, cytomegalovirus, or hepatitis B and C virus or from serologic tests for the human immunodeficiency virus or blood cultures. Cryoglobulin was not detected. Total complement, C3, and C4 levels were normal. The serum IgA level was 4.5 g, the total IgG level was 9.7 g (IgG1 level was 5.45 g, IgG2 level was 2.77 g, IgG3 level was 0.66 g, and IgG4 level was 0.27 g), and the IgM level was 0.89 g. Esophagastroduodenoscopy showed purpuric duodenitis. A few hours later, adynamic ileus developed with gross abdominal distention and vomiting.

Ten days after admission, despite nasogastric suction and total parenteral nutrition, the abdominal syndrome worsened and was associated with a temperature of 38 °C, bloody diarrhea, a weight loss of 3 kg, and persistent adynamic ileus. Results of a barium study of the small bowel were normal and did not show obstruction or perforation. After administration of intravenous immunoglobulin, 1 g/kg of body weight per day for 2 days, the patient's purpura and abdominal syndrome improved dramatically. Serum IgG subclasses were not significantly modified after treatment. Four weeks after therapy, the purpura recurred without abdominal symptoms and resolved spontaneously. The patient remains well after a follow-up of 1 year without treatment.

Henoch-Schonlein purpura is a hypersensitivity vasculitis of unknown origin and pathogenesis. Several theories about its cause have been proposed, including the presence of deposits of IgA-containing immune complexes, deficiency in an IgG subclass (particularly IgG1) [2], and the presence of antiendothelial cell antibodies. Various mechanisms of action have been presumed for intravenous immunoglobulin therapy, all of which have an immunomodulatory effect [3]. Intravenous immunoglobulin therapy has been used in several autoimmune diseases, IgA nephropathy, Kawasaki disease, and in antineutrophil cytoplasmic antibody-positive vasculitis [4]. Severe gastrointestinal complications of Henoch-Schonlein purpura are rare in adults. Our patient had persistent adynamic ileus, fever, and leukocytosis. In this context, we avoided corticosteroids in favor of intravenous immunoglobulin, which resulted in dramatic and rapid improvement without adverse reaction. This treatment only suspended the condition; the patient had a relapse of purpura 4 weeks later. Serum IgG subclasses were not modified before or after treatment [2]. We found only one case report [5] on the efficacy of intravenous immunoglobulin for treating severe gastrointestinal complications of Henoch-Schonlein purpura. Despite its cost, intravenous immunoglobulin could be an alternative for steroids or dapsone in such critical complications.