The clinical spectrum of Henoch-Schönlein purpura: insights from two distinct presentations

Abstract

Henoch-Schönlein purpura (HSP), or IgA vasculitis, is an autoimmune small-vessel vasculitis caused by IgA immune complex deposition, primarily affecting children but rarely seen in adults. We present two contrasting cases to highlight its variable presentation and diagnostic challenges. The first case is a 44-year-old woman with recurrent purpuric rash, arthralgia, elevated CRP and ESR, and positive anti-RNP and anti-Sm antibodies, suggesting an autoimmune disorder. However, clinical features and biopsy confirmed IgA vasculitis. She was managed with corticosteroids, topical agents, and supportive care, emphasizing the need to distinguish HSP from other autoimmune diseases. The second case involves a 13-year-old girl with a known history of HSP who presented with hematemesis and melena. Investigations revealed marked leukocytosis, elevated CRP, and gastrointestinal involvement confirmed through endoscopy and biopsy, showing erosive gastritis and pancolitis consistent with GI vasculitis. She was treated with corticosteroid pulse therapy and cyclophosphamide. These cases demonstrate the spectrum of HSP in both adults and children and highlight the importance of correlating clinical symptoms with laboratory and imaging findings for timely and accurate diagnosis. They also reinforce the role of immunosuppressants in managing severe or refractory cases.