Rush University Medical Center Chicago, IL, United States
Zoe Post, MD, Sanmeet Singh, MD, Thayer Hamoudah, MD, Kenika R. Robinson, MD Rush University Medical Center, Chicago, IL
Introduction: Henoch Schonlein Purpura (HSP) is the most common systemic vasculitis in young children, but is less commonly seen in adults. Diagnosis is based on the presence of palpable purpura without thrombocytopenia and with at least one other disease manifestation (arthritis, renal involvement, and/or abdominal pain). Gastrointestinal (GI) manifestations rarely precede the classic rash. Here we describe a young adult who initially presented with solely GI involvement of HSP.
Case Description/Methods: A 25-year-old healthy male presented with two weeks of nausea, persistent sharp peri-umbilical abdominal pain of variable severity, and melena. Labs were notable for WBC 10.9 K/uL with neutrophilic predominance, CRP 66 mg/L, ESR 4 mm/hr, creatinine 0.88 mg/dL. Abdominal X-ray with mildly dilated gas-filled small bowel loops in central abdomen. CT abdomen/pelvis was unrevealing. EGD revealed antral-predominant nodular erythematous mucosal lesions in the stomach but otherwise normal appearing gastric mucosa (Fig 1a), histologically with surface hemorrhage. In the duodenum, diffuse erythematous mucosa with spontaneous hemorrhage was seen (Fig 1b), pathology consistent with duodenitis and erosions/ulcerations. Colonoscopy revealed right-sided erythema and granularity with biopsies showing non-specific inflammation. Interestingly, the day after endoscopic evaluation the patient developed bilateral lower extremity petechial rash (Fig 1c). Punch biopsies were diagnostic of IgA-mediated leukocytoclastic vasculitis. The overall presentation was consistent with HSP without renal involvement. Further work-up showed low C3 (consistent with HSP), negative ANA screen and ANCA, normal gastrin level, negative cryoglobulins, and unremarkable infectious work up.
Discussion: HSP in adults is rare, especially if it presents with isolated GI disease. However, clinicians should maintain a high index of suspicion in adults with manifestations of small bowel inflammation. HSP mainly affects the small intestine, because the villi contain end-capillaries that get obstructed by immune complexes and cause necrosis. The rest of the GI tract is generally not involved by medium-vessel vasculitis due to its redundant circulation. The skin and epiphyseal plate have similar capillaries and are therefore frequently affected by HSP. Treatment with corticosteroids is controversial as most patients improve with supportive care. In this case, decision was made to treat with corticosteroids as patient returned with worsening disease activity.
Figure: Figure 1. a) Endoscopic view of gastric antrum/pylorus with multiple small nodular erythematous mucosal lesions; b) Endoscopic view of second portion of the duodenum showing diffuse erythematous mucosa with many erosive purple-hued mucosal lesions in a diffuse distribution with associated spontaneous bleeding; c) Classic purpuric rash on medial aspect of right foot.
Zoe Post indicated no relevant financial relationships.
Sanmeet Singh indicated no relevant financial relationships.
Thayer Hamoudah indicated no relevant financial relationships.
Kenika Robinson indicated no relevant financial relationships.
Zoe Post, MD, Sanmeet Singh, MD, Thayer Hamoudah, MD, Kenika R. Robinson, MD. P2013 - Case of a Young Adult With Small Intestine Predominant Henoch Schonlein Purpura, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.