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Henoch-Schonlein Purpura(Anaphylactoid Purpura; HSP; Vascular Purpura)

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

Definition

Henoch-Schonlein purpura (HSP) is inflammation of the blood vessels in the skin and other body organs. When it involves the skin, it causes a telltale rash. The rash looks like bruising or small dots in the skin, referred to as purpura.

Causes

HSP is caused by an abnormal reaction of the immune system. Normally, the immune system marks and attacks foreign items like viruses and bacteria. However, with HSP, the immune system attacks the blood vessels. It is not clear why the immune system attacks the body.

The change in the immune system may be triggered by:

  • Bacterial or viral infections
  • Certain medications
  • Recent exposure to certain vaccines
  • Infection by insect bites

HSP occurs most often after a respiratory infection. HSP is not contagious.

Risk Factors

HSP is most common in children aged 2 to 11 years old, but it can occur at any age. Factors that increase your risk of HSP include:

  • Recent upper respiratory illness, such as a cold
  • Recent exposure to vaccines, chemicals, cold weather, or insect bites
Symptoms

Symptoms may last for 4 to 6 weeks and may include:

  • Skin rash:
    • Reddish-purple spots that can be felt and are not itchy
    • Often appears on the buttocks or legs, may appear on the elbows
    • Red spots of various sizes
    • Bruising, usually below the waist
  • Pain in the joints, especially knees and ankles
  • Abdominal pain
  • Blood 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. A physical exam will be done.

Your bodily fluids, tissues, and waste may be tested. This can be done with:

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

Skin Biopsy

Skin proceedure

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Treatment

HSP usually gets better on its own. Your doctor may prescribe medications if symptoms or complications are causing problems. Medications may include:

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

There are no guidelines to prevent 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:

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

Henoch-Schonlein purpura. National Institute of Diabetes and Digestive and Kidney Disease website. Updated September 7, 2012. Available at: http://familydoctor.org/familydoctor/en/diseases-conditions/henoch-schonlein-purpura.html. Accessed November 13, 2012.

Henoch-Schonlein purpura. National Institute of Health Office of Rare Disease Research website. Available at: http://rarediseases.info.nih.gov/GARD/Condition/8204/HenochSchonlein_purpura.aspx/Print. Accessed August 6, 2013.

Kraft D, McKee D, et al. Henoch-Schonlein purpura: a review. Am Fam Physician. 1998 Aug 1;58(2):405-408. Available at: http://www.aafp.org/afp/1998/0801/p405.html. Accessed November 13, 2012.

Ronkainen J, Koskimies O, 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 August 2013 by Michael Woods, 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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