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Henoch-Schonlein purpura is a type of vasculitis, a group of disorders that cause blood vessel inflammation. In Henoch-Schonlein purpura, this inflammation causes bleeding in the small blood vessels (capillaries) in your skin, joints, intestines and kidneys. The main symptom is a purplish rash, mainly on the lower legs and buttocks. Henoch-Schonlein purpura also often causes abdominal pain and aching joints, and in some people, kidney problems.

Although Henoch-Schonlein purpura can affect anyone, it's most common in children and young adults. Henoch-Schonlein purpura usually improves on its own, but if your child's kidneys are affected he or she will need medical care as well as long-term follow-up to prevent more serious problems.

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

• Rash (purpura). Reddish-purple spots, which look like bruises, are the most distinctive and universal symptom of Henoch-Schonlein purpura. The rash develops mainly on the buttocks, legs and feet, but it can also appear on the arms, face and trunk and may be worse in areas of pressure, such as the sock line and waistline.
• Abdominal pain. More than half of children with Henoch-Schonlein purpura develop severe abdominal pain. Other gastrointestinal symptoms may include nausea, vomiting and bloody stools.
• Swollen, sore joints (arthritis). People with Henoch-Schonlein purpura often have painful, swollen joints — mainly in the knees and ankles. These symptoms subside when the disease clears and leave no lasting damage.

Kidney problems
Sometimes, kidney problems can be a serious complication of the disease. In that case, the primary signs of kidney problems are blood and protein in the urine. In some cases, urinary blood (hematuria) may be visible only under a microscope.

When to see a doctor
In some cases, Henoch-Schonlein purpura causes serious problems of the bowel or kidneys. See your doctor or your child's pediatrician as soon as possible if you notice the distinctive rash and other symptoms.

In Henoch-Schonlein purpura, some of the body's small blood vessels become inflamed, which can cause bleeding in the skin, joints, abdomen and kidneys. Why this initial inflammation develops isn't clear, although it may be the result of an overzealous immune system responding inappropriately to certain triggers.

Some of these triggers include:

• Viral and bacterial infections, such as strep throat and parvovirus infection — nearly half the children with Henoch-Schonlein purpura develop it after an upper respiratory infection
• Certain medicines, including some types of antibiotics and antihistamines
• Insect bites
• Some vaccinations, including those for measles, typhoid, yellow fever and cholera
• Cold weather
• Certain chemicals

• Age. The disease affects children and young adults, with the majority of cases occurring in children 2 to 11 years of age.
• Sex. Henoch-Schonlein purpura is nearly twice as common in boys as it is in girls.
• Race. White and Asian children are more likely to develop Henoch-Schonlein purpura than black children are.
• Illness. Having an upper respiratory infection or other bacterial or viral illness increases a child's risk.
• Season. Henoch-Schonlein purpura strikes mainly in autumn and spring, and almost never in summer.

For most people, symptoms of Henoch-Schonlein purpura improve in a few weeks, leaving no lasting problems. Recurrences are fairly common, however. Children who have severe symptoms appear more likely to have a recurrence, but repeat bouts are usually milder than the initial episode.

In rare cases, Henoch-Schonlein purpura can cause a bowel obstruction (intussusception) that reduces blood flow to the intestinal tract and leads to inflammation of other organs, including the pancreas.

Kidney damage: A serious complication
The most serious complication of Henoch-Schonlein purpura is kidney damage, which can cause blood in the urine, swelling and high blood pressure. Most children with kidney problems recover fully, but in a small percentage of cases, Henoch-Schonlein purpura leads to end-stage kidney disease. In that case, dialysis or a kidney transplant may be needed.

The long-term outcome for people with Henoch-Schonlein purpura appears to depend on whether they develop kidney problems and how severe those problems are.

Because appointments can be brief and there's often a lot of ground to cover, it's a good idea to be well prepared for your appointments. Here's some information to help you get ready for your appointment and what to expect from your doctor.

What you can do

• Write down all symptoms and how long they've been present — even those that seem unrelated to the rash. When all three major indicators of Henoch-Schonlein purpura — rash, joint pain and abdominal pain — are present, the diagnosis isn't difficult. If the only symptom is rash, however, diagnosis can be challenging.
• Make a list of all medications, including vitamins, herbs and over-the-counter drugs, that you or your child is taking. Even better, take the original bottles and a written list of the dosages and directions.
• Write down questions that you want to ask your doctor. Don't be afraid to ask questions or to speak up when you don't understand something your doctor says. Start with the problems that concern you most. If you run out of time, ask to speak with a nurse or physician's assistant or leave a message for your doctor.

If you have symptoms of Henoch-Schonlein purpura, questions you may want to ask include:

• What might be causing these symptoms?
• What tests are needed to confirm the diagnosis?
• Is this condition temporary or chronic?
• How will I know if there is kidney damage? What if it turns up later on?
• How is Henoch-Schonlein purpura treated?
• What are the side effects of treatment?
• Do you have any literature on this condition? Is there a Web site you can recommend where I can learn more?

What to expect from your doctor
Your doctor is likely to ask a number of questions, such as:

• When did these symptoms first start?
• Did they come on suddenly or gradually?
• What did the rash look like when it first started?
• Is the rash painful? Does it itch?
• Were you or your child sick before the rash started?
• Do you or your child have other symptoms, such as stomach pain or joint aches?
• What medications do you take?

No single laboratory test can confirm Henoch-Schonlein purpura, but certain tests can help rule out other diseases and make a diagnosis of Henoch-Schonlein seem likely.

In addition to 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 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. If there is any doubt about the diagnosis, your doctor may check the number of platelets — the cells that help blood clot — in the blood.
• 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 the urine, as well as elevated levels of certain proteins, can help your doctor determine the extent to which the kidneys are affected by the disease.
• Skin biopsy. If other tests are inconclusive, your doctor may take a small sample of skin that's sent to a lab and examined under a microscope.
• 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.

Henoch-Schonlein purpura usually improves on its own within four to six weeks with no lasting ill effects. Treatment is usually aimed at relieving discomfort and preventing complications. Most often, this means bed rest, plenty of fluids and, occasionally, nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin, others).

In certain circumstances, children and young adults with Henoch-Schonlein purpura may require hospitalization. This is likely if they have:

• Trouble staying well hydrated
• Severe abdominal pain or gastrointestinal bleeding
• Joint symptoms that make movement difficult
• Serious kidney problems

Corticosteroids
The use of powerful corticosteroids in treating or preventing complications of Henoch-Schonlein purpura is controversial. Because these drugs can have serious side effects and their usefulness isn't clear, be sure to discuss the risks and benefits with your doctor or pediatrician.

Children and adults with Henoch-Schonlein purpura should be kept comfortable while the disease runs its course. Rest and NSAIDs as needed to relieve joint discomfort and swelling may help. These drugs can cause stomach upset and even ulcers, so follow package directions carefully and take them with food or milk and for just a brief time.

Be sure to take your child for follow-up urine tests for as long as your doctor recommends. This helps alert your doctor to any serious kidney complications.