Henoch-Schonlein Purpura

1 What is Henoch-Schonlein Purpura?

Henoch-Schonlein purpura is a disorder that causing inflammation and bleeding in the small blood vessels of the skin, joints, intestines and kidneys.

The most noticeable feature of Henoch-Schonlein purpura is a purplish rash which usually occurs on the lower legs and buttocks. Henoch-Schonlein purpura can also cause abdominal pain and aching joints. Some cases can lead to kidney failure.

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

2 Symptoms

There are basically four main symptoms of Henoch-Schonlein purpura, although not every individual with the condition develops all four.

These include:

  • Reddish-purple spots, which have a similar appearance to bruises and are the most distinctive  and universal sign of the condition.
  • The rash mainly develops on the buttocks, legs and feet but can also appear on the  face, arms and trunk.
  • Individuals with Henoch-Schonlein purpura often have painful and swollen joints, mainly the knees and ankles. Sometimes joint pain preceeds the classical rash by two or more  days.These symptoms reduce after the disease clears leaving no lasting damage.
  • Many children with Henoch-Schonlein purpura develop gastrointestinal symptoms, such as abdominal pain, nausea, vomiting or bloody stool. 

These symptoms sometimes occur before the rash. Henoch-Schonlein purpura can also affect the kidneys. In most cases, this shows up as protein or blood in the urine, which cannot be determined unless a urine test done. Usually this goes away once the illness passes, but in a few cases, kidney disease may develop and even persist.

3 Causes

The exact cause of Henoch-Schonlein purpura not known.

In this condition, some of the body's small blood vessels become inflamed,  which further leads to bleeding in the skin, joints, abdomen and kidneys. There is no clear reason to why this inflammation initially develops, although it may be a result of an overzealous immune system responding inappropriately to certain triggers.

Almost 50% of individuals with Henoch-Schonlein purpura developed the disease after an upper respiratory infection, such as a cold. Infectious triggers may include certain medications, food, insect bites and exposure to cold temperatures.

4 Making a Diagnosis

The initial part of seeking a diagnosis of Henoch-Schonlein purpura will be seeing a family doctor. Your doctor can further refer you to kidney specialist or a nephrologist if any kidney complications arise.

The following information can be helpful to anyone preparing for this kind of appointment. Before the appointment it is necessary to write answers to the following questions:

  • When did the symptoms begin?
  • Did they come on suddenly or gradually?
  • Were you or was your child sick before the rash started?
  • What medications and supplements do you or your child take regularly?

Questions you may want to ask your doctor 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's 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?

The doctor is also likely to ask a number of questions, such as:

  • What did the rash look like when it first started?
  • Is the rash painful?
  • Does it itch?
  • Do you or does your child have other symptoms, such as stomach pain or joint aches?

A diagnosis of Henoch-Schonlein purpura is fairly easy to make if the classic rash, joint pain and gastrointestinal symptoms are present. In cases where these signs and symtoms are absent, the doctor may suggest one or more of the following tests. Lab tests Although no single test can confirm Henoch-Schonlein purpura, certain tests can help rule out other diseases and make a diagnosis of Henoch-Schonlein seem likely. They may include:

  • Blood tests. People who have Henoch-Schonlein purpura often have abnormal levels of a particular type of antibody in their blood.
  • Urine tests. Urine may be tested for evidence of blood and to determine if the kidneys are still working properly.
  • Biopsies. If there are doubts about the rash or if other tests are inconclusive, the doctor may take a small sample of skin to be examined under a microscope.

In cases of severe kidney involvement, the doctor may suggest a kidney biopsy to help guide treatment decisions. Imaging tests  can aslo be carried out.

5 Treatment

Henoch-Schonlein purpura usually improves on its own in a period of about a month without any specific treatment. Bed rest, plenty of fluids and over-the-counter pain relievers can aid.

The use of powerful corticosteroids  such as, prednisone in treating or preventing complications of this disease is quite controversial.  If a section of the bowel has folded upon itself or has ruptured, surgical repair will be recommended.

6 Lifestyle and Coping

Lifestyle modifications are necessary in order to cope with Henoch-Schonlein purpura.

Children and adults with mild form of Henoch-Schonlein purpura should be kept comfortable.

Bed rest, plenty of fluids and over-the-counter pain relievers may help.

7 Risks and Complications

There are several risks and complications associated with Henoch-Scholein purpura.

The following factors increase the risk of developing Henoch-Scholein purpura:

  • Age: the disease primarily affects children and adults with the majority of cases occurring in children between 2 and 6 years.
  • Sex: boys are more likely to develop this disease than girls are.
  • Race: white and asian children are more likely to develop this condition than black children are.
  • Time of the year: this disease strikes mainly in autumn, winter and spring.

Complications associated with Henoch-Schonlein purpura include:

  • Kidney damage
  • Bowel obstruction which leads to a condition known as intussusception (a condition in which a section of the bowel folds into itself).