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Diseases and Conditions
Henoch-Schonlein purpura
From MayoClinic.com
Special to CNN.com

Introduction

Henoch-Schonlein purpura affects the small blood vessels of your skin, joints, intestines and kidneys, causing them to become inflamed and swollen (vasculitis).

Blood vessel inflammation can cause bleeding into your skin. For people with Henoch-Schonlein purpura, the bleeding causes a purplish skin rash (purpura) that can last up to several weeks.

Although Henoch-Schonlein purpura can affect anyone, it's most common in children ages 2 to 11, with 5 to 7 being the ages at which it's most likely to occur. It's the most common vasculitis that occurs in children. Boys are more likely to develop the condition than girls are, and it occurs less frequently in blacks than it does in whites or Asians.

In most cases, Henoch-Schonlein purpura improves on its own within four to six weeks. Medications may ease discomfort from some of the signs and symptoms of Henoch-Schonlein purpura.

Signs and symptoms

There are three primary signs and symptoms of Henoch-Schonlein purpura, although not everyone with the disease develops all three. They are:

  • Rash (purpura). Reddish-purple spots, which may look like bruises or be raised, occur in almost all cases. The rash usually appears on your child's buttocks, around elbows, and on legs and feet, although it may show up on the face and on other parts of your child's skin.
  • Swollen, sore joints (arthritis). Knees and ankles are most commonly affected. Joint symptoms affect 60 percent to 80 percent of those with Henoch-Schonlein purpura. The arthritis resolves with no permanent damage when the disease clears up.
  • Abdominal pain. About half of people with Henoch-Schonlein purpura develop gastrointestinal signs and symptoms, usually about eight days after the appearance of the rash. The most common is abdominal pain, which may be severe. Other gastrointestinal signs and symptoms may include nausea, vomiting and bloody stools or urine.

These signs and symptoms may be accompanied by fever.

Causes

Although the cause of Henoch-Schonlein purpura isn't known, experts believe it to be most often triggered by an upper respiratory infection, such as a common cold. The blood vessel inflammation (vasculitis) may result from an overzealous immune system response to the triggering agent. Although the triggering agent may be contagious, Henoch-Schonlein purpura itself isn't.

Besides a common cold, other possible triggers include:

  • Viral and bacterial infections, such as strep throat and parvovirus infection
  • Certain medicines, such as some types of antibiotics and antihistamines
  • Insect bites
  • Certain vaccinations, such as for measles

When to seek medical advice

Henoch-Schonlein purpura can cause inflammation of the blood vessels in any organ, including the intestines and kidneys. Rarely, serious problems of the bowel or kidneys can develop. So see a doctor if you or your child has the signs and symptoms of the disease, such as the characteristic rash, joint pain and swelling, and abdominal pain.

Screening and diagnosis

If you or your child has all three major indicators of Henoch-Schonlein purpura — rash, joint pain and abdominal pain — the diagnosis shouldn't be difficult. Without all three indicators, diagnosis can be challenging.

There isn't a single laboratory test that will confirm the diagnosis, but there are tests that can help rule out other diseases and lend support to a diagnosis of Henoch-Schonlein purpura. Besides conducting a physical exam and taking a medical history, your doctor may request several tests, including:

  • Blood tests. An elevated level of a special type of protein called immunoglobulin A (IgA) may suggest Henoch-Schonlein purpura, but it isn't conclusive. Another indication may be an elevated erythrocyte sedimentation rate — commonly referred to as sed rate. By measuring how quickly red blood cells fall to the bottom of a tube of blood in an hour, this test may indicate the level of inflammation in the body. Blood test results also may rule out conditions other than Henoch-Schonlein purpura, such as an inflamed pancreas (acute pancreatitis) or another type of vasculitis.
  • Urine tests. These evaluate kidney function to determine whether the disease is affecting the kidneys. It can take several months after the appearance of the rash for the kidneys to be affected, so your doctor may want to repeat the urine tests monthly for up to six months. Blood in your child's urine, as well as elevated levels of certain proteins, can help your doctor determine the extent to which your child's kidneys are affected by the disease.
  • Imaging studies. Your doctor may request a computerized tomography (CT) scan to rule out other causes of abdominal pain, such as appendicitis, a tear in the bowel (perforation) or bowel obstruction.

Complications

For most people with Henoch-Schonlein purpura, the signs and symptoms improve in a few weeks leaving no lasting problems. Up to one-third of those who get the condition will get it at least once again. Children who have a more severe form of the disease appear to be more likely to have a recurrence, but recurrences typically are milder than the initial disease.

Rarely, the disease damages kidneys. The disease affects most children's kidneys only mildly. However, some children and most adults who develop Henoch-Schonlein purpura experience more serious kidney involvement, leading to the kidneys' inability to remove waste from the blood. In extreme cases Henoch-Schonlein purpura can lead to kidney failure.

Also rare, if Henoch-Schonlein purpura affects the bowel, it can cause an obstruction (intussusception) that may require surgery to correct.

Treatment

There's no treatment for Henoch-Schonlein purpura, which typically improves on its own within four to six weeks. If your doctor determines that the cause is a reaction to a medicine, then stopping the medicine will clear up the condition. Until the disease clears up, some medications may help ease discomfort:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs). NSAIDs, such as ibuprofen (Advil, Motrin, others), can be effective in easing joint pain and swelling. They also can cause stomach upset, including ulcers, so follow package directions carefully and have your child take them with food or milk and only for as long as necessary.
  • Corticosteroids. Your doctor may prescribe a powerful anti-inflammatory corticosteroid, such as prednisone, to help relieve severe abdominal pain. The abdominal pain usually subsides within 72 hours, so your child shouldn't have to take corticosteroids very long. However, the drugs can have serious side effects, such as elevated blood sugar, so be sure your child takes them only as directed.

Self-care

If your child has Henoch-Schonlein purpura, try to keep him or her comfortable while the disease runs its course. Rest and NSAIDs as needed to relieve joint discomfort and swelling may help. Be sure to take your child for follow-up urine tests for as long as your doctor recommends.

November 03, 2006

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