eMedicine Specialties > Emergency Medicine > Hematology & Oncology

Henoch-Schonlein Purpura

Author: Philip Bossart, MD, Professor, Department of Surgery, Division of Emergency Medicine, University of Utah Hospital, University of Utah School of Medicine
Contributor Information and Disclosures

Updated: Jun 18, 2009

Introduction

Background

Henoch-Schönlein 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-Schönlein purpura. However, the illness is named after the 2 German physicians who further characterized this vasculitis.

In 1837, Johan Schönlein 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-Schönlein 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

Frequency

United States

Approximately 14 cases of Henoch-Schönlein purpura occur per 100,000 school-aged children. Henoch-Schönlein purpura also occurs in adults, although less commonly than in children.1

Mortality/Morbidity

In general, Henoch-Schönlein 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 are aged 2-11 years. In some series, as many as 27% of the patients are adults.4

Clinical

History

The most common symptoms of Henoch-Schönlein purpura include the following:

  • Rash (95-100%), especially involving the legs, may not be present on initial presentation
  • Subcutaneous edema (20-50%)
  • Abdominal pain and vomiting (85%)
  • Joint pain (60-80%), especially involving the knees and ankles
  • Scrotal edema (2-35%)
  • Bloody stools

Physical

  • Palpable purpura, particularly on the buttocks and legs (see Media files 1-2)


Typical rash distribution of Henoch-Schönlei...

Typical rash distribution of Henoch-Schönlein purpura.

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Typical rash distribution of Henoch-Schönlei...

Typical rash distribution of Henoch-Schönlein purpura.



Characteristic rash of Henoch-Schönlein purp...

Characteristic rash of Henoch-Schönlein purpura.

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Characteristic rash of Henoch-Schönlein purp...

Characteristic rash of Henoch-Schönlein purpura.

  • Edema of the hands, feet, scalp, and ears
  • Arthritis, most commonly involving the knees and ankles
  • Abdominal tenderness
  • Gastrointestinal bleeding
  • Acute scrotal edema that may mimic testicular torsion

Causes

  • The etiology of Henoch-Schönlein purpura is unknown.
  • About 50% of patients have a preceding upper respiratory illness (URI).
  • Multiple infectious agents as well as drugs, foods, and insect bites may trigger Henoch-Schönlein purpura.
  • Antistreptolysin O titers are raised in 20-50% of patients.

More on Henoch-Schonlein Purpura

Overview: Henoch-Schonlein Purpura
Differential Diagnoses & Workup: Henoch-Schonlein Purpura
Treatment & Medication: Henoch-Schonlein Purpura
Follow-up: Henoch-Schonlein Purpura
Multimedia: Henoch-Schonlein Purpura
References
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References

  1. Blanco R, Martínez-Taboada VM, Rodriguez-Valverde V, García-Fuentes M, Gonzalez-Gay MA. Henoch-Schonlein 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-Schönlein 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. [Best Evidence] Chartapisak W, Opastiraku S, Willis NS, Craig JC, Hodson EM. Prevention and treatment of renal disease in Henoch-Schönlein purpura: a systematic review. Arch Dis Child. Feb 2009;94(2):132-7. [Medline].

  7. Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. Mar 24 2007;369(9566):976-8. [Medline].

  8. Huber AM, King J, McLaine P, Klassen T, Pothos M. A randomized, placebo-controlled trial of prednisone in early Henoch Schönlein Purpura [ISRCTN85109383]. BMC Med. Apr 2 2004;2:7. [Medline].

  9. [Best Evidence] Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. Aug 2006;149(2):241-7. [Medline].

  10. Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline].

  11. [Best Evidence] Weiss PF, Feinstein JA, Luan X, Burnham JM, Feudtner C. Effects of corticosteroid on Henoch-Schönlein purpura: a systematic review. Pediatrics. Nov 2007;120(5):1079-87. [Medline].

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Further Reading

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Keywords

Henoch-Schonlein purpura, Henoch-Schönlein purpura, HSP, symptoms, treatment, vasculitis, small-vessel vasculitis, purpura, arthritis, skin rash, spots, abdominal pain, hematuria, nonthrombocytopenic purpura, purpura rheumatica, upper respiratory illness, URI, HSP nephritis, immunoglobulin A nephropathy, IgA nephropathy, rash, subcutaneous edema, scrotal edema, palpable purpura, HSP-related intussusception, idiopathic thrombocytopenic purpura, ITP, thrombotic thrombocytopenic purpura, TTP

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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.

Medical Editor

Edmond A Hooker II, MD, DrPH, FAAEM, Assistant Professor, Department of Health Services Administration, Xavier University; Associate Clinical Professor, Department of Emergency Medicine, University of Louisville; Assistant Clinical Professor, Department of Emergency Medicine, Wright State University
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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

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

Steven C Dronen, MD, FAAEM, Director of Emergency Services, Director of Chest Pain Center, Department of Emergency Medicine, Ft Sanders Sevier 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.

 
 
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