Henoch-Schönlein purpura is inflammation mainly of small vessels, usually occurring in children.

• A rash of reddish purple bumps and spots on the lower legs is usually the first symptom, followed by joint aches, digestive upset, and kidney malfunction.
• Biopsy of the affected skin can confirm the diagnosis.
• Corticosteroids can relieve joint aches and digestive upset, but occasionally, other drugs that suppress the immune system are also needed.

Henoch-Schönlein purpura usually affects children aged 3 to 15 but can occur at any age. It may develop when the immune system responds abnormally to an infection or something else. It may be triggered by upper respiratory infections, drugs, or insect bites. Blood vessels in the intestine and kidney may become inflamed.

Symptoms

A rash of small spots that look like bruises or reddish purple bumps (purpura) appear on the arms, legs, buttocks, and top of the feet. After a few days or weeks, more spots and bumps may appear, sometimes on the face or trunk. Most children also have a fever and achy, tender, and swollen joints, including the ankles, knees, hips, wrists, and elbows.

Crampy abdominal pain, nausea, vomiting, and diarrhea are common. Stools or urine may contain blood. Rarely, the intestine slides into itself, like a collapsible telescope. This complication, called intussusception, can cause sudden stomach pain and vomiting because the intestine is blocked.

Symptoms usually resolve after about 4 weeks but often recur at least once after a few weeks. Most people recover completely. Rarely, chronic kidney failure develops.

Diagnosis

Doctors suspect the disorder when the characteristic rash occurs in children. If the diagnosis is not clear, a sample from the affected skin is taken and examined under a microscope (biopsy) to look for abnormalities that can confirm the diagnosis. Urine tests are done to check for blood and excess protein, which indicate that the kidneys are affected. Blood tests are usually done to measure kidney function.

If kidney malfunction worsens, a kidney biopsy is done. It can help doctors determine how severe the problem is and what kind of recovery can be expected.

Treatment

If a drug is contributing to the disorder, it is stopped. Otherwise, treatment focuses on relieving symptoms. It may include nonsteroidal anti-inflammatory drugs (NSAIDs) and bed rest.

Corticosteroids or other drugs, taken by mouth, may help control abdominal pain and are occasionally needed to help control severe joint pain or swelling. If the kidneys or digestive organs are severely affected, methylprednisolone (a corticosteroid), given intravenously, and cyclophosphamide (which suppresses the immune system), taken by mouth, may be used.

Last full review/revision April 2008 by Carmen E. Gota, MD