An Unusual Presentation of Henoch-Schonlein Purpura

Abstract

Henoch-Schonlein Purpura (HSP) is a leukocytoclastic vasculitis (LCV) which affects the small blood vessels of the skin, joints, gastrointestinal tract (GI) and kidneys. It is characterized by abdominal pain, arthritis, nephritis and a distinctive purpuric rash most pronounced on the legs and buttocks. We present an unusual case of HSP in which GI manifestations were confused initially with a disseminated infectious process versus Crohn's disease. Case Report: The patient is a 26 year old woman with a history significant for food allergies with associated urticaria and angioedema. She was admitted with a one week history of colicky abdominal pain, nausea, vomiting and diarrhea. On physical exam she was afebrile. She had facial edema and urticaria to the upper extremities. Diffuse abdominal tenderness on palpation was noted without distention, guarding or rebound tenderness. Her admission laboratory tests revealed guaiac positive stool, WBC count 15.7 and Platelet 521. Hematuria on UA. EGD showed multiple ulcerations in the duodenum. Colonoscopy demonstrated a 4 cm mass with superficial ulcerations at the ileocecal valve. There was patchy inflammation with friability throughout the GI tract. Abdominal CT scan revealed focal bowel-wall thickening in the cecum, duodenum and proximal jejunum. Biopsies of the duodenum, ileocecal area and colon showed acute ulcer only. Crohn's disease was suspected and treatment initiated. On hospital day five, new skin eruptions appeared on the lower legs which were large, purpuric, palpable and nonblanching. Skin biopsy of leg lesion showed acute LCV. The patient improved clinically and discharged on tapered steriods. Direct immunofluorescence later returned positive for IgA. Discussion: Our patient demonstrated several common features of HSP but also revealed unique findings. While HSP occurs most frequently in children (85%) and in males (2:1), our patient was a 26 year old woman. Furthermore, endoscopy in patients with HSP usually demonstrate an erosive, hemorrhagic mucosa with marked edema but a mass at the ileocecal valve has not been previously reported. Diseases such as Crohn's disease and infectious ileitis, however, have been described to affect the ileum and the ileocecal valve. Thus, in our patient, the atypical skin rash at initial presentation with the ulcerated mass at the ileocecal valve delayed the diagnosis of HSP.