• Henoch-Schönlein purpura (HSP) is the most common form of vasculitis in children.

  • It is a small-vessel vasculitis mediated by immunoglobulin A–containing immune complexes and characterized by nonthrombocytopenic purpura, abdominal pain, arthralgia, and renal disease.

  • Diagnosis of HSP is clinical, and no laboratory tests are specific for HSP.

  • Newborn screening has helped with early diagnosis and management, thus decreasing morbidity and improving longevity.

  • Treatment is supportive. Patients with severe abdominal and joint pain may be treated with steroids.

  • Steroids do not prevent renal disease in patients with HSP.

  • Patients with nephritic or nephrotic syndrome have a much higher risk of developing chronic kidney disease and should be referred to a nephrologist.

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