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

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

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 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 medicationfor significant abdominal pain or kidney disease Antibioticsto 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 Physicianswebsite. 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 Medicinewebsite. 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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