• Henoch-Schonlein Purpura

    (Anaphylactoid Purpura; HSP; Vascular Purpura)

    Definition

    Henoch-Schonlein purpura (HSP) is swelling 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.”
    People of all ages may develop HSP, but it is most common in children.

    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 attack the blood vessels. It is not clear why the immune system attacks the body.
    The change in the immune system It 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 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 are not itchy
      • Often appears on the buttocks or legs, may appear on the elbows
    • 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. A physical exam will be done. Tests may include:
    • 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 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

    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://kidney.niddk.nih.gov/kudiseases/pubs/HSP/ . Accessed November 13, 2012.

    Henoch-Schonlein purpura. American Academy of Family Physicians website. 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%5Fpurpura.aspx/Print . Accessed November 13, 2012.

    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.

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