Introduction: Henoch-Schönlein Purpura (HSP) is the most common pediatric vasculitis. It classically presents with petechiae in the absence of thrombocytopenia and/or palpable purpura in addition to at least one of the four clinical symptoms of: abdominal pain, arthralgia, hematuria, and proliferative glomerulonephritis or leukocytoclastic vasculitis with predominantly IgA on histological evaluation. HSP presents primarily in pediatric populations, often in children prior to the age of 10. Adult-onset HSP has an incidence of 4/100,000. Common gastrointestinal symptoms include nausea, vomiting, and abdominal pain. Gastrointestinal manifestations occur in about 20% of patients prior to skin involvement. We present the case of a 30 year old female who presented with Adult Onset HSP Duodenitis.
Case Description/Methods: A 30 year old female presented with three episodes of hematemesis and two days of generalized abdominal pain. In addition she reported arthralgias and a lower extremity rash. Her exam revealed a palpable petechial rash on her bilateral buttocks, thighs, and legs as well as generalized abdominal tenderness. Initial labs revealed a white blood cell count of 19.29 K/μL. Contrast-enhanced computed tomography (CT) scan revealed wall thickening, submucosal edema, and surrounding fat stranding centered at the duodenum and proximal jejunum consistent with duodenitis. An esophagogastroduodenoscopy (EGD) was performed which revealed friable mucosa and numerous nonbleeding cratered and linear ulcers throughout the duodenum with the most prominent ulcers located in the second portion. Duodenal biopsies were consistent with mucosal ulceration. Left buttock biopsy revealed a leukocytolastic vasculitis with IgA deposition in vessel walls confirming the diagnosis of HSP. The patient was treated with intravenous corticosteroids for four days and had significant clinical improvement with resolution of her abdominal pain and vomiting. She was discharged with an oral steroid taper.
Discussion: Although HSP remains primarily a disease of childhood, adult-onset HSP does occur and often with non-classical presentations. The small intestinal epithelial villi are most commonly affected due to the presence of end capillaries, making it susceptible to ischemic injury. It is therefore important to consider HSP in patients with duodenitis and extra-intestinal manifestations of arthralgias and rashes. The disease course is often self-limited however corticosteroids may lead to more rapid improvement in symptoms.
