Henoch-Schonlein Purpura
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Henoch-Schonlein Purpura

(Anaphylactoid Purpura; HSP; Vascular Purpura)

Pronounced: Hen-awk-shern-line purr-purr-ah

En Español (Spanish Version)

Definition

Henoch-Schonlein purpura (HSP) is an inflammation of the blood vessels in the skin and other body organs. Skin involvement often results in a rash, typically occurring on the buttocks and legs. The rash often resembles bruising or bleeding into the skin, a condition referred to as “purpura.”

People of all ages may develop HSP, but it is most common in children.

Causes

The exact cause of HSP is unknown. It is believed to be a disorder of the immune system. It may be triggered by bacterial or viral infections, certain medications, vaccines, or perhaps even insect bites. HSP frequently occurs in the spring, often after a respiratory infection. It is not contagious.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition. Risk factors for HSP include:

  • Recent upper respiratory illness, such as a cold
  • Age: 2 to 11
  • Recent exposure to vaccines, chemicals, cold weather, or insect bites
  • Sex: male children are twice as likely as females to develop the disease; the risk is equal in adults.

Symptoms

Symptoms may last for 4 to 6 weeks. These may include:

  • Skin rash of reddish-purple spots, usually on the buttocks or legs, occasionally on the elbows that is not itchy
  • Pain in the joints, especially knees and ankles
  • Abdominal pain
  • Blood or protein in the urine caused by kidney inflammation
  • Swelling of the ankles
  • Swelling of the scrotum in males
  • Fever
  • Blood in the stool
  • Vomiting

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include:

  • Blood tests
  • Urinalysis
  • Stool sample
  • Skin biopsy from an area of the rash

Skin Biopsy

Skin proceedure

© 2008 Nucleus Medical Art, Inc.

Treatment

HSP usually gets better on its own. However, under some circumstances your doctor may prescribe specific medications to reduce symptoms and prevent complications. These may include:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs)—to lessen joint pain and arthritis
  • Cortisone medication—for significant abdominal pain or kidney disease
  • Antibiotics—to treat infection
  • Cyclophosphamide ( Cytoxan )—to suppress the immune system when you have symptoms of severe kidney disease

Prevention

There are no guidelines for the prevention of HSP. Relapse occurs in about 50% of cases.

It is important to make sure that you have long-term, follow-up visits with your doctor to be sure that kidney disease doesn't develop.

RESOURCES:

American Academy of Family Physicians
http://www.familydoctor.org

American Autoimmune Related Diseases Association
http://www.aarda.org

CANADIAN RESOURCES

Caring for Kids
http://www.caringforkids.cps.ca/

College of Family Physicians of Canada
http://www.cfpc.ca/

References:

American Academy of Family Physicians website. Available at: http://www.familydoctor.org .

Dillon MJ. Henoch-Schonlein purpura (treatment and outcome). Cleve Clin J Med . 2002;69(Suppl 2):SII121-SII123.

National Library of Medicine website. Available at: http://www.nlm.nih.gov/ .

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 . 2006;149:241-247.

Saulsbury FT. Epidemiology of Henoch-Schonlein purpura. Cleve Clin J Med . 2002;69(Suppl 2):SII87-SII89.



Last reviewed October 2007 by Ross Zeltser, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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