About Vasculitis

Henoch Schönlein Purpura

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What is Henoch-Schönlein Purpura?

Henoch-Schönlein Purpura (HSP) is a systemic vasculitis which can affect the skin, joints, bowel and kidneys. If it affects the kidneys it is usually called Henoch-Schönlein Nephritis or Vasculitic IgA Nephropathy.

Who are affected?

HSP mostly affects children, but can affect adults. Sometimes it follows a throat or chest infection. It affects boys and girls equally. Half the children affected are under the age of five. Kidney involvement is more likely to be severe in older children and adults.

Sometimes HSP is occasionally also called Berger's disease but this should not be confused with Buerger's disease which is a different type of vasculitis

What is the aetiology (cause)?

The cause of HSP is not yet known.

What are the symptoms?

Skin rash in HSPSymptoms occur over days or several weeks. A widespread rash is common mainly on the backs of the legs, buttocks, trunk and back. The rash is caused by inflammation in the small blood vessels of the skin which can cause bleeding into the skin. It is often felt as small bumps (palpable) and is a reddish/purple colour (purpura). In severe cases it can cause large areas of raised purple patches and skin ulcers. Other common symptoms are painful joints, stomach and abdominal pain and sometimes bleeding from the bowels. If the kidneys are involved there will be blood and protein in the urine.

 Blisters pre-diagnosis in HSP T Skin rash in HSP

Making a diagnosis

As with other types of vasculitis there is no single specific test. The diagnosis depends on the doctor recognising the pattern of symptoms and findings on examination. Blood tests may show evidence of inflammation. There may be a raised level of immunoglobulin A (IgA) (a type of antibody) in the blood. If the skin or kidneys are affected then a biopsy may show immunoglobulin A antibodies present in the tissue. A positive biopsy helps confirm the diagnosis but a negative biopsy does not rule out the diagnosis.

Treatment

No specific treatment is needed for most cases of HSP with the symptoms resolving spontaneously over time. Anti-inflammatory drugs (such as ibuprofen) are often prescribed for the joint pains. There is some evidence that steroid treatment may lead to more rapid improvement of bowel symptoms in children. If the kidney is involved and there is inflammation in the kidney then treatment with steroids or other immunosuppressants may be needed to prevent damage to the kidneys.

Drugs and Side effects

For information on the main drugs prescribed for Henoch Schönlein Purpura see:

For information on other drugs used in the treatment of vasculitis see Glossary of drugs and side effects.

Prognosis

Although relapses are quite common they are mostly mild and self-limiting. Usually the disease stops re-occurring as patients get older.

Key Points

  • Children are mostly affected by HSP
  • The disease may be present for months or years before a diagnosis is made
  • Treatment depends on the severity of the disease
  • The disease commonly relapses after the initial treatment

Related Vasculitis Articles

Further reading

Useful links

Our Useful Vasculitis Links page contains contact details for organisations offering help and support for patients with Henoch-Schönlein Purpura and other vasculitis diseases.

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