By LILLIANA BARILLAS-ARIAS, MD, ALEXA ADAMS, MD, and THOMAS J. A. LEHMAN, MD | August 31, 2008

Weill Medical College of Cornell University

Dr Barillas-Arias is an attending physician in the department of pediatric rheumatology at Schneider Children's Hospital in New Hyde Park, NY. Dr Adams is an assistant attending physician at the Weill Medical College of Cornell University and the Hospital for Special Surgery, both in New York City. Dr Lehman is professor of clinical pediatrics at Weill Medical College of Cornell University and chief of pediatric rheumatology, assistant attending, and senior scientist at the Hospital for Special Surgery.

ABSTRACT: Henoch-Schönlein purpura (HSP), the most common of the pediatric vasculitides, is an acute small-vessel vasculitis characterized by IgA-dominant immune deposits in target organs. The clinical triad that typically characterizes HSP includes nonthrombocytopenic purpura, arthralgias and/or arthritis, and colicky abdominal pain. The presence of palpable purpura is essential for diagnosis. Although HSP is usually self-limiting, significant renal and GI complications may occur. Risk factors for nephritis include disease onset after age 7 years, persistent purpuric lesions, severe GI symptoms, and low factor XIII activity. Scrotal symptoms may be the first manifestation of HSP. Classification criteria for HSP were recently updated. There are no distinctive laboratory abnormalities associated with HSP; however, laboratory testing can be helpful when trying to exclude other diseases and for disease monitoring. Biopsy, the only confirmatory test, is rarely necessary. Treatment is supportive and includes adequate hydration, nutrition, and pain control with mild analgesics and NSAIDs. Corticosteroids given early in the course of illness may be of some benefit, particularly if there is severe abdominal pain or kidney involvement.