P4994 - Adult-Onset Henoch-Schonlein Purpura (HSP) With Gastrointestinal Manifestations

Henoch-Schoenlein purpura (HSP) is a form of immune-mediated systemic small- vessel vasculitis characterized by deposition of immunoglobulin A (IgA) in affected organs. While HSP commonly affects pediatric population, adult-onset HSP is infrequent, often with diverse clinical manifestations. This case report delves into an adult-onset HSP featuring prominent gastrointestinal symptoms, present in approximately 48% of patients with adult-onset HSP.

A 43-year-old male presented with acute left testicular pain radiating to the left lower abdomen and hematuria lasting one day. CT imaging of the abdomen and pelvis demonstrated localized inflammation in the distal duodenum/proximal jejunum. The patient continued to experience abdominal pain 1 year and 6 months later and underwent a small bowel enteroscopy revealing jejunal and duodenal ulceration with pathology consistent with non-specific acute enteritis. Serologic markers for inflammatory bowel disease were reported as not consistent. The patient experienced further abdominal pain episodes, sought care, and follow-up imaging 1 year and 7 months after the initial presentation confirmed persistent moderate proximal jejunitis. The patient received corticosteroid treatment, resulting in the resolution of jejunal inflammation, observed on subsequent colonoscopy and MRI enterography. However, recurrent abdominal pain prompted repeat imaging 5 years and 6 months later, revealing inflammatory changes in the distal duodenum alongside a new bilateral lower extremity purpuric rash. A dermatology consultation and skin biopsy confirmed early leukocytoclastic vasculitis with patchy granular IgA deposition, consistent with Henoch-Schonlein purpura (HSP) diagnosis nearly six years after the initial onset.

This case report of Henoch-Schonlein Purpura (HSP) with significant gastrointestinal manifestations highlights the challenges associated with diagnosing and managing this rare condition in adults. A comprehensive literature review from 1970 to 2019 identified 283 adult cases featuring extracutaneous involvement of Henoch-Schonlein purpura (HSP). With this atypical presentation, we aim to heighten awareness regarding an uncommon etiology of abdominal pain and small bowel inflammation, thereby fostering early recognition and facilitation of the management of adult-onset Henoch-Schonlein Purpura (HSP).