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Henoch-Schonlein purpuraBy Mayo Clinic staff
Original Article: http://www.mayoclinic.com/health/henoch-schonlein-purpura/DS00838
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Henoch-Schonlein purpura (HEH-nawk SHURN-line PUR-puh-ruh) 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, typically 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 the kidneys are affected, medical care is generally needed, as well as long-term follow-up to prevent more-serious problems.
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There are four main characteristics of Henoch-Schonlein purpura, although not everyone with the disease develops all four. They include:
- Rash (purpura). Reddish-purple spots, which look like bruises, are the most distinctive and universal sign 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.
- Swollen, sore joints (arthritis). People with Henoch-Schonlein purpura often have painful, swollen joints — mainly in the knees and ankles. Joint pain sometimes precedes the classical rash by one or two days. These symptoms subside when the disease clears and leave no lasting damage.
- Gastrointestinal symptoms. More than half the children with Henoch-Schonlein purpura develop gastrointestinal symptoms, such as abdominal pain, nausea, vomiting or bloody stools. These symptoms usually develop within eight days of developing the classic rash.
- Kidney involvement. About 20 to 50 percent of people with Henoch-Schonlein purpura have some degree of kidney involvement. In most cases, this manifests itself as protein or blood in the urine, or both, which you may not even know is there unless you have a urine test done. Usually this goes away once the illness passes, but in a few cases, kidney disease may develop and even persist.
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 may include:
- Viral and bacterial infections, such as strep throat and parvovirus infection — nearly half the children with Henoch-Schonlein purpura develop the disease 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 primarily children and young adults, with the majority of cases occurring in children between 4 and 6 years of age.
- Sex. Henoch-Schonlein purpura is slightly more common in boys than 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, winter and spring, and rarely 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.
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 very small percentage of cases, Henoch-Schonlein purpura leads to end-stage kidney disease. In that case, dialysis or a kidney transplant may be needed. Adults are at greater risk than children of developing end-stage kidney disease.
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.
In rare cases, Henoch-Schonlein purpura can cause a kind of bowel obstruction (intussusception) that reduces blood flow to the intestinal tract and leads to inflammation of other organs, including the pancreas.
Women who've had Henoch-Schonlein purpura during childhood may be at increased risk of high blood pressure during pregnancy. If you're pregnant and have a history of Henoch-Schonlein purpura, be sure to tell your doctor about it so that you can be monitored appropriately.
Preparing for your appointment
You'll likely see your family doctor or your child's pediatrician for this condition, although you may be referred to a kidney specialist (nephrologist) if kidney complications develop. Here's some information that may 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.
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 website 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?
Tests and diagnosis
A diagnosis is fairly easy to make when the classic rash, joint pain and gastrointestinal symptoms are present. If only one or two are present, diagnosis can be more of a challenge.
Although no single laboratory test can confirm Henoch-Schonlein purpura, 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.
- 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 there are doubts about the rash or if other tests are inconclusive, your doctor may take a small sample of skin to be sent to a lab and examined under a microscope for the presence of IgA in the blood vessels.
- Kidney biopsy. A kidney biopsy is a more invasive procedure, but your doctor may request it if there are signs and symptoms of severe kidney involvement, such as high blood pressure or sudden onset of kidney failure, especially if the tests above are not conclusive. Results of the kidney biopsy can help your doctor decide on appropriate treatment.
- Imaging studies. Your doctor may request an abdominal ultrasound to rule out other causes of abdominal pain, such as appendicitis or a tear in the bowel (perforation), or to check for possible complications, such as bowel obstruction.
Treatments and drugs
Henoch-Schonlein purpura usually improves on its own within a few weeks — usually no more than eight 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
- Taking acetaminophen (Tylenol, others) or nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen (Advil, Motrin, others)
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.
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
- Extensive ulceration of the skin
- Serious kidney problems, which may be treated with immunosuppressive drugs
The use of powerful corticosteroids in treating or preventing complications of Henoch-Schonlein purpura is controversial. It's most often used to treat severe gastrointestinal symptoms. 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.
Lifestyle and home remedies
Children and adults with Henoch-Schonlein purpura should be kept comfortable while the disease runs its course. Rest and pain relievers as needed to relieve joint discomfort and swelling may help. NSAIDs can cause stomach upset and even ulcers, so follow package directions carefully and take NSAIDs with food or milk and for just a brief time.
In some children with abdominal pain, eating can trigger further pain. But most children are able to eat a simple, bland diet.
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- Henoch-Schonlein purpura. Vasculitis Foundation. http://www.vasculitisfoundation.org/HenochSchonleinpurpura. Accessed Sept. 7, 2010.
- Henoch-Schonlein Purpura. National Kidney and Urologic Diseases Clearinghouse. http://kidney.niddk.nih.gov/kudiseases/pubs/HSP. Accessed Sept. 7, 2010.
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