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Henoch-Schonlein Purpura

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Henoch-Schonlein Purpura
Author: Philip Bossart, MD; Chief Editor: Steven C Dronen, MD, FAAEM more... Updated: Mar 7, 2013

Background
Henoch-Schnlein purpura (HSP) is a small-vessel vasculitis characterized by purpura, arthritis, abdominal pain, and hematuria.[1, 2] In his 1801 book, On Cutaneous Diseases, Heberden described a 5-year-old boy with "bloody points" over the skin of his legs, abdominal pain, bloody stools and urine, and painful subcutaneous edema. This may be the first published case of Henoch-Schnlein purpura. However, the illness is named after the 2 German physicians who further characterized this vasculitis. In 1837, Johan Schnlein described the association of nonthrombocytopenic purpura and joint pain, which he called purpura rheumatica. Later, his student, Eduard Henoch, noted the gastrointestinal and renal involvement in this disease.

Pathophysiology
Henoch-Schnlein purpura (HSP) is a small-vessel vasculitis characterized by immunoglobulin A (IgA), C3, and immune complex deposition in arterioles, capillaries, and venules. HSP and IgA nephropathy are related disorders. Both illnesses have elevated serum IgA levels and identical findings on renal biopsy; however, IgA nephropathy almost exclusively involves young adults and predominantly affects the kidneys only. HSP affects mostly children and involves the skin and connective tissues, gastrointestinal tract, joints, and scrotum as well as the kidneys.[2,
3, 4]

Epidemiology
Frequency
United States Approximately 14 cases of Henoch-Schnlein purpura occur per 100,000 school-aged children. Henoch-Schnlein purpura also occurs in adults, although less commonly than in children.[1]

Mortality/Morbidity
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In general, Henoch-Schnlein purpura (HSP) is a benign self-limited disorder. Less than 5% of cases cause chronic symptoms. Less than 1% of cases progress to end-stage renal failure.

Sex
The male-to-female ratio is about 2:1.

Age
Approximately 75% of patients affected by Henoch-Schnlein purpura are aged 2-11 years. In some series, as many as 27% of the patients are adults.[4]

Contributor Information and Disclosures

Author Philip Bossart, MD Professor, Department of Surgery, Division of Emergency Medicine, University of Utah Hospital, University of Utah School of Medicine Philip Bossart, MD is a member of the following medical societies: American College of Emergency Physicians Disclosure: Nothing to disclose. Specialty Editor Board Edmond A Hooker II, MD, DrPH, FAAEM Associate Professor, Department of Health Services Administration, Xavier University, Cincinnati, Ohio; Assistant Professor, Department of Emergency Medicine, University of Cincinnati College of Medicine Edmond A Hooker II, MD, DrPH, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine, American Public Health Association, Society for Academic Emergency Medicine, and Southern Medical Association Disclosure: Nothing to disclose. Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference Disclosure: Medscape Salary Employment Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Physicians Disclosure: Nothing to disclose. John D Halamka, MD, MS Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine Disclosure: Nothing to disclose. Chief Editor
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Henoch-Schonlein Purpura

Steven C Dronen, MD, FAAEM Chair, Department of Emergency Medicine, LeConte Medical Center Steven C Dronen, MD, FAAEM is a member of the following medical societies: American Academy of Emergency Medicine and Society for Academic Emergency Medicine Disclosure: Nothing to disclose.

References
1. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, Garcia-Fuentes M, Gonzalez-Gay MA. HenochSchonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. May 1997;40(5):859-64. [Medline]. 2. Szer IS. Henoch-Schonlein purpura. Curr Opin Rheumatol. Jan 1994;6(1):25-31. [Medline]. 3. Gedalia A. Henoch-Schonlein purpura. Curr Rheumatol Rep. Jun 2004;6(3):195-202. [Medline]. 4. Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. May 2002;13(5):1271-8. [Medline]. 5. O'Brien WM, O'Connor KP, Horan JJ, Eggli DF, Gibbons MD. Acute scrotal swelling in Henoch-Schonlein syndrome: evaluation with testicular scanning. Urology. Apr 1993;41(4):366-8. [Medline]. 6. Bogdanovic R. Henoch-Schonlein purpura nephritis in children: risk factors, prevention and treatment. Acta Paediatr. Dec 2009;98(12):1882-9. [Medline]. 7. Chartapisak W, Opastirakul S, Hodson EM, Willis NS, Craig JC. Interventions for preventing and treating kidney disease in Henoch-Schonlein Purpura (HSP). Cochrane Database Syst Rev. Jul 8 2009;CD005128. [Medline]. 8. [Best Evidence] Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch-Schonlein purpura: a systematic review. Arch Dis Child. Feb 2009;94(2):132-7. [Medline]. 9. Saulsbury FT. Clinical update: Henoch-Schonlein purpura. Lancet. Mar 24 2007;369(9566):976-8. [Medline]. 10. Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo-controlled trial of prednisone in early Henoch Schonlein Purpura [ISRCTN85109383]. BMC Med. Apr 2 2004;2:7. [Medline]. [Full Text]. 11. [Best Evidence] Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in HenochSchonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. Aug 2006;149(2):241-7. [Medline]. 12. Bowman P, Quinn M. Question 1: Should steroids be used to treat abdominal pain caused by HenochSchonlein purpura?. Arch Dis Child. Nov 2012;97(11):999-1000. [Medline]. 13. Flynn JT, Smoyer WE, Bunchman TE, Kershaw DB, Sedman AB. Treatment of Henoch-Schonlein Purpura glomerulonephritis in children with high-dose corticosteroids plus oral cyclophosphamide. Am J Nephrol. Mar-Apr 2001;21(2):128-33. [Medline]. 14. Augusto JF, Sayegh J, Delapierre L, Croue A, Tollis F, Cousin M, et al. Addition of plasma exchange to glucocorticosteroids for the treatment of severe Henoch-Schnlein purpura in adults: a case series. Am J Kidney Dis . May 2012;59(5):663-9. [Medline]. 15. Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline]. 16. [Best Evidence] Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroid on Henoch-Schonlein purpura: a systematic review. Pediatrics . Nov 2007;120(5):1079-87. [Medline].
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