2658 Henoch-Schonlein Purpura Duodenitis

Abstract

INTRODUCTION: Henoch-Schonlein Purpura (HSP) is an IgA mediated systemic, small and medium vessel vasculitis that typically presents with palpable purpura, arthralgias, abdominal pain and hematuria. Gastrointestinal manifestations occur in about 20% of patients prior to skin involvement. We report a case of a 51-year-old woman that presented with HSP duodenitis. CASE DESCRIPTION/METHODS: A 51-year-old female presented with a several week history of nausea, vomiting and epigastric abdominal pain. In addition, she had been experiencing dysphagia, arthralgias, fever and, later on, a palpable, petechial and purpuric rash on her face, ears and all extremities. She had no previous medical history. Physical exam showed an extensive palpable, ecchymotic and purpuric rash involving her arms, face, right ear and purpura on plantar surface of her feet (See Figure 1). Her wrist, elbow, knee and ankle joints were tender to palpation, warm and swollen. She had epigastric tenderness to palpation with no rebound or guarding. Laboratory workup was notable for a white blood cell count of 18.32 M/μL (4.20–5.60 M/μL). Computed tomography of the abdomen showed bowel wall thickening at the junction of duodenum and jejunum with fat stranding in the central mesentery. Biopsy of the skin showed features of leukocytoclastic vasculitis with associated perivascular necrotizing inflammation and hemorrhage (See Figure 2). EGD showed diffuse circumferential erythema with boggy mucosa as well as friability with touch bleeding consistent with ischemic type changes from distal bulb to the second portion of the duodenum (See Figure 3). Biopsies of the duodenum showed evidence of intramucosal hemorrhage, a mixed inflammatory infiltrate of neutrophils, lymphocytes and some plasma cells within the lamina propria. There were also fragments of advanced necrosis with evidence of small vessel thrombosis and vasculitis. Her abdominal pain and oral intake improved after treatment with 1 g methylprednisone for three days. The patient was discharged from the hospital with an oral steroid taper. DISCUSSION: HSP is rare in adults with roughly 20-fold fewer cases than in children. The range of endoscopic findings includes gastritis, ulceration, duodenitis, and submucosal hemorrhage with the second portion of the duodenum being involved more than the duodenal bulb. The end capillaries of small intestinal villi are particularly susceptible to ischemic injury. HSP is a self-limiting disease and majority of the patients improve spontaneously.