Pediatric Henoch-Schonlein Purpura

Author: Pamela L Dyne, MD; Chief Editor: Richard G Bachur, MD more...

Updated: Jan 4, 2011

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Background
Pathophysiology
Epidemiology
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Background

Henoch-Schönlein purpura (HSP) is an inflammatory disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. It is the most common vasculitis in children.

The syndrome takes its name from 2 German physicians. In 1837, Johan Schönlein first described several cases of peliosis rheumatica or purpura associated with arthritis. Thirty years later, Edouard Henoch described the GI manifestations, including vomiting, abdominal pain, and melena. Henoch-Schönlein purpura has also been referred to as rheumatica purpura, leukocytoclastic vasculitis, and allergic vasculitis.

A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles. Courtesy of Pamela L Dyne, MD.

A 7-year-old girl with Henoch-Schönlein purpura. Courtesy of Pamela L Dyne, MD.

Pathophysiology

The etiology of Henoch-Schönlein purpura is unclear. It is thought to be multifactorial with genetic, environmental, and antigenic components. More than 75% of patients report antecedent upper-respiratory, pharyngeal, or GI infections. Multiple bacterial and viral infectious agents have been associated with the development of Henoch-Schönlein purpura, and cases of Henoch-Schönlein purpura also have been reported after drug ingestions and vaccinations.

Henoch-Schönlein purpura is thought to be an immunoglobulin A (IgA)–mediated autoimmune phenomenon. An unknown antigenic stimulant has been postulated to cause a rise in IgA. The antigen-antibody complexes deposit locally throughout the body and activate pathways leading to necrotizing vasculitis.

Genetic research may reveal the potential role of cytokines, endothelia and nitric oxide metabolism in Henoch-Schönlein purpura.

Henoch-Schönlein purpura can involve nearly any organ system. Hallmarks of Henoch-Schönlein purpura include a characteristic rash, migratory polyarthritis, renal involvement, and GI involvement. The clinical manifestations of Henoch-Schönlein purpura are the result of antigen-antibody complexes depositing throughout the body, which cause migratory arthralgias, abdominal cramping, the petechial and/or vasculitic rash, and hematuria.

Frequency

United States

The rate is 14 cases per 100,000 population.

Mortality/Morbidity

Henoch-Schönlein purpura generally resolves without permanent complications. However, serious GI and renal complications may occur. GI complications include intussusception (usually ileoileal), bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, or massive GI bleeding.

Approximately 20% of patients have renal manifestations, and 5% develop end-stage renal disease (ESRD). Patients with only hematuria do not develop ESRD. About 15% of patients with hematuria and proteinuria develop ESRD. Approximately 50% of patients with nephritic or nephrotic syndrome develop ESRD. The long-term morbidity is predominantly attributed to renal involvement.

Sex

In children, the male-to-female ratio is 2:1. In adults, the male-to-female ratio is approximately 1:1.

Age

Henoch-Schönlein purpura primarily affects children. Adults are rarely affected. Approximately 75% of cases occur in children aged 2-11 years. The median age is 5 years. Older age at disease onset is associated with development of chronic renal disease.

Proceed to Clinical Presentation

Contributor Information and Disclosures

Author

Pamela L Dyne, MD Professor of Clinical Medicine/Emergency Medicine, David Geffen School of Medicine at UCLA; Attending Physician, Department of Emergency Medicine, Olive View-UCLA Medical Center

Pamela L Dyne, MD is a member of the following medical societies: American Academy of Emergency Medicine, American College of Emergency Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Stacy Sawtelle, MD Clinical Instructor, Department of Emergency Medicine, University of California, San Francisco, School of Medicine

Disclosure: Nothing to disclose.

Heather Kesler DeVore, MD Clinical Attending Physician, Assistant Professor Physician, Department of Emergency Medicine, Washington Hospital Center/Georgetown University Hospital

Heather Kesler DeVore, MD is a member of the following medical societies: Emergency Medicine Residents Association and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Debra Slapper, MD Consulting Staff, Department of Emergency Medicine, St Anthony's Hospital

Debra Slapper, MD is a member of the following medical societies: American Academy of Emergency Medicine

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Wayne Wolfram, MD, MPH Associate Professor, Department of Emergency Medicine, Mercy St Vincent Medical Center

Wayne Wolfram, MD, MPH is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Pediatrics, and Society for Academic Emergency Medicine

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

Richard G Bachur, MD Associate Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston

Richard G Bachur, MD is a member of the following medical societies: American Academy of Pediatrics, Society for Academic Emergency Medicine, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

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