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Henoch-Schönlein Purpura

Pronunciations

Henoch-Schönlein purpura (allergic purpura) is a disease in which blood vessels in the skin, joints, digestive tract, or kidneys become inflamed and may leak.

Henoch-Schönlein purpura, an uncommon disease, affects mainly young children, but it can affect older children and adults. The disease is believed to result from an autoimmune reaction, in which the body attacks its own tissues. Usually, Henoch-Schönlein purpura develops after a respiratory tract infection, but it can develop after an immunization or an insect bite or be caused by an allergic reaction to drugs or food. The rate at which the disease develops and its duration vary.

Symptoms and Diagnosis

The disease may begin with the appearance of small, bluish purple spots (purpura)—most often on the feet, legs, arms, and buttocks-as blood leaks from vessels in the skin. Over several days, the purpura may become raised and hard; crops of new purpura may break out for several weeks after the first one appears. Swollen, achy joints are common, usually accompanied by fever. Bleeding in the digestive tract may cause abdominal cramps and pain. Blood in the urine (hematuria) may develop. Most people recover completely within a month, but symptoms may recur several times. Bleeding in the kidneys may cause kidney damage.

The diagnosis is based on the symptoms. Sometimes a sample of affected skin is removed and examined under a microscope (biopsy) to confirm the diagnosis.

Treatment

A drug that may be causing an allergic reaction is discontinued immediately. Corticosteroids (for example, prednisone Some Trade Names
DELTASONE
METICORTEN
) may help relieve swelling, joint pain, and abdominal pain, but they do not prevent or reverse kidney damage. Drugs that reduce the activity of the immune system (immunosuppressive drugs), including azathioprine Some Trade Names
IMURAN
or cyclophosphamide Some Trade Names
CYTOXAN
, are sometimes used if kidney damage develops, but it is not known if they are helpful.

Last full review/revision May 2006 by Joel L. Moake, MD

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