Henoch-Schönlein purpura (HSP) is an acute IgA-mediated vasculitic disorder of predominantly pediatric population. It is characterized by generalized small vessel vasculitis involving the skin, gastrointestinal (GI) tract, kidneys, joints, and rarely other organ system. Skin rash is usually the first sign of HSP and is almost always present. While GI symptoms such as abdominal pain and vomiting are seen in approximately 60% of patients, histologic documentation of the affected GI segment is rare. Moreover, HSP presenting as bowel ischemia without rash is exceedingly rare. An 8-year-old girl presented with abdominal pain and diarrhea following recent upper respiratory tract infection. Upper GI obstruction was suspected, thus laparotomy was performed and ischemic portion of jejunum was resected. Within two weeks of her small bowel resection, she developed malignant hypertension, hematuria, and proteinuria. No skin rash was noted. Kidney biopsy was performed for definitive diagnosis. The resected bowel specimen showed ischemic necrosis and patchy leukocytoclastic vasculitis of small submucosal vessels. A suspicion of primary vasculitis was raised, but serological markers were inconclusive for specific diagnosis. Kidney biopsy revealed diffuse proliferative glomerulonephritis. No vasculitis, necrotizing lesion or crescent was seen. Immunofluorescent study showed predominantly mesangial IgG (3+), IgA (3+), C3 (2+), and C1q (2+) deposits. Predominance of IgA deposits was consistent with HSP. Electron microscopy showed scattered glomerular deposits. Additional serologic studies for lupus were negative. Patient was treated with anti-hypertensive and steroids following which the patient recovered. We report a rare initial presentation of HSP without skin rash. While the presence of mesangial C1q deposits raise the possibility of coexisting autoimmune component, its significance or association with clinical presentation remains unknown. This case underscores the significance of high index of suspicion for primary vasculitic process in pediatric population with GI symptoms.
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