Henoch-Schonlein purpura

DEFINITION

Henoch-Schonlein purpura (HEN-awk SHURN-line PUR-pu-ruh) is a disorder that causes inflammation and bleeding in the small blood vessels in your skin, joints, intestines and kidneys.

The most striking feature of Henoch-Schonlein purura is a purplish rash, typically on the lower legs and buttocks. Henoch-Schonlein purpura can also cause abdominal pain and aching joints. Rarely serious kidney damage can occur.

Although Henoch-Schonlein purpura can affect anyone, it’s most common in children between the ages of 2 and 6. Henoch-Schonlein purpura usually improves on its own. Medical care is generally needed if the disorder affects the kidneys.

CAUSES

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.

Nearly half the people who have Henoch-Schonlein purpura developed the disease after an upper respiratory infection, such as a cold. Infectious triggers may include chickenpox, strep throat, measles and hepatitis. Other triggers may include certain medications, food, insect bites or exposure to cold weather.

SYMPTOMS

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. Many children with Henoch-Schonlein purpura develop gastrointestinal symptoms, such as abdominal pain, nausea, vomiting or bloody stools. These symptoms sometimes occur before the rash appears.
• Kidney involvement. Henoch-Schonlein purpura can also affect the kidneys. In most cases, this shows up as protein or blood in the urine, 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 as soon as possible if you or your child develops the distinctive rash associated with Henoch-Schonlein purpura.