Henoch-Schonlein Purpura

An 18-year-old woman was admitted with three days of diffuse, colicky abdominal pain that evolved into sharp periumbilical pain. Review of systems was notable for nausea, diarrhea, subjective fevers, chills, and pruritic violaceous papules on her bilateral lower extremities. She initially presented to an urgent care center for her abdominal complaints and a CT scan was performed which reportedly showed evidence of enteritis. She was discharged on metronidazole, cefuroxime and hydromorphone but her symptoms did not improve. She presented to the hospital for further evaluation. On admission, vital signs were normal and labs were notable for a leukocytosis of 14.6 with 83% neutrophils, CRP 9.42, and ESR 14. CT scan of the abdomen showed jejunal wall thickening, mesenteric fat stranding, and lymphadenopathy. A skin biopsy of the lower extremity lesions demonstrated an IgA vasculitis. She underwent an EGD which revealed patchy, congested duodenal mucosa without active or stigmata of bleeding. She was treated for Henoch-Schönlein purpura (HSP), also known as IgA vasculitis, with assistance from rheumatology. HSP is an immune-mediated systemic vasculitis primarily affecting children 3-15 years of age, with a male predominance. HSP should be suspected in patients with palpable purpura, arthralgia, renal disease, and abdominal pain, however patients rarely manifest the classic tetrad of symptoms. A retrospective study of 150 subjects with HSP found that purpura was the most common presenting symptom (74%) and abdominal pain was the least common at 12%. Notably however, abdominal pain is just one of several GI symptoms associated with HSP. Zhang et al retrospectively analyzed 90 adult patients with HSP and found that HSP is also associated with melena/hematochezia (62%), diarrhea (17%), emesis (13%), and hematemesis (8%). The epigastrium was found to be the most common location of pain, followed by the RLQ and periumbilical regions. Among those undergoing EGD, 48% had abnormal findings and the second portion of the duodenum was the most commonly affected segment of the upper GI tract. Triggers of HSP including infections, insect bites, and certain drugs share an immunologic pathway. Treatment is symptom driven as the efficacy of various medications has yet to be fully validated. There is evidence that IVIG is efficacious for the treatment of HSP with severe GI symptoms (intense pain, hemorrhage, protein losing enteropathy), however larger trials are required.