Henoch-Schönlein Purpura

1. Jessica Reid-Adam, MD 1. Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY 1. 1. Gedalia A Henoch-Schonlein Purpura. Gedalia A. Curr Rheumatol Rep. 2004;6(3):195–202 [OpenUrl][1][CrossRef][2][PubMed][3] 2. 1. Tizard EJ, 2. Hamilton-Ayres MJJ Henoch-Schonlein Purpura. Tizard EJ, Hamilton-Ayres MJJ. Arch Dis Child Educ Pract Ed. 2008;93(1):1–8 [OpenUrl][4][FREE Full Text][5] 3. 1. Narchi H Risk of Long Term Renal Impairment and Duration of Follow Up Recommended for Henoch-Schonlein Purpura with Normal or Minimal Urinary Findings: A Systematic Review. Narchi H. Arch Dis Child. 2005;90(9):916–920 [OpenUrl][6][Abstract/FREE Full Text][7] 4. 1. Hicks J, 2. Mierau G, 3. Wartchow E, 4. Eldin K Renal Diseases Associated with Hematuria in Children and Adolescents: A Brief Tutorial. Hicks J, Mierau G, Wartchow E, Eldin K. Ultrastruct Pathol. 2012;36(1):1–18 [OpenUrl][8][CrossRef][9][PubMed][10] Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that annually affects 10 to 20 children per 100,000 population. It is the most common vasculitis of childhood. Although HSP is seen in infancy through adulthood, most documented cases affect children; more than 75% of those diagnosed as having HSP are younger than 10 years, with a peak incidence at 4 to 6 years. White and Asian children are most commonly affected, whereas African American children are least commonly affected. Male predominance among children approaches 2:1. The most recent consensus criteria published in 2010 by the European League Against Rheumatism and the Paediatric Rheumatology European Society state that for diagnosis of HSP, palpable purpura should be accompanied by at least one of the following: abdominal pain, predominance of IgA deposition on a biopsy specimen, arthritis or arthralgia, or renal involvement indicated by hematuria/proteinuria. HSP often follows an upper respiratory tract infection, with most cases occurring in the fall and winter. Although many cases do not have a specifically identified infectious trigger, a variety of viral and bacterial agents have been associated with HSP, group A β-hemolytic streptococcus being the most common. Pathogenesis has been linked to IgA1, a subclass of IgA, and its deposition is noted in the glomerulus, the skin, and the blood vessels of the gastrointestinal (GI) tract. Abnormally O-glycosylated IgA1 has been identified in patients with HSP nephritis and in patients with IgA nephropathy, a disease indistinguishable from HSP on renal biopsy. The hallmark of HSP is cutaneous palpable purpura, often involving the lower extremities and buttocks. 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