Henoch-Schönlein purpura (HSP) is a rare condition that is caused by inflammation of blood vessels (vasculitis). Blood vessels throughout the body are affected but HSP most often causes a skin rash, tummy (abdominal) pain and joint pains.
Henoch-Schönlein purpura (HSP) is not usually serious. But it's a really important condition because very occasionally it can cause serious complications, especially kidney problems.
What are the causes?
HSP develops because of an abnormal reaction of the body's defence (immune) system. It is thought that something acts as a trigger, such as an infection or a medicine. The abnormal reaction causes inflammation of the blood vessels (this is called vasculitis).
Read more about the causes of HSP.
How common is it?
HSP is not very common. Between 8 and 20 in 100,000 people will develop HSP each year. HSP mostly affects children, especially children under the age of 10 years. But HSP can also affect older children and adults. It is more common in boys than in girls.
Children under the age of 2 years tend to develop milder symptoms. Adults with HSP tend to develop more severe symptoms and are more likely to develop complications.
What are the symptoms?
There may have been an upper respiratory tract infection - for example, cough, runny nose, and a high temperature (fever) - within the few weeks before HSP develops.
The symptoms and outlook for Henoch-Schönlein purpura will depend on which parts of the body are affected. The three most common symptoms are a skin rash, tummy (abdominal) pain and joint pains.
Many people with HSP don't develop any complications. But complications may affect the kidneys, gut (bowel) or other parts of the body.
Find out more about the symptoms and complications of HSP.
How is it diagnosed?
HSP is usually suspected because of the typical symptoms. However, a number of investigations may help doctors to confirm the diagnosis and to see which parts of the body are affected. Initial tests may include blood tests and a urine dipstick test. HSP can cause high blood pressure if the kidneys are affected so you may also need blood pressure checks.
Further tests will depend on where and how badly your body is affected by HSP. These tests may include a skin biopsy, kidney biopsy and a special test on the stools (faeces) to see if there are any traces of blood in the stools, which may occur if HSP affects the gut.
Find out more about the diagnosis of HSP.
What's the treatment?
For most people, HSP will get better of its own and so no specific treatment is needed. However, there are a number of treatments that can help with the symptoms - for example, rest and painkillers. If a medicine is thought to have triggered HSP then this should be stopped.
It is important to monitor for any early signs of kidney problems if someone has HSP. If kidney problems are detected and treated early, this may reduce the chance of any long-term kidney damage. Testing the urine for any signs of kidney damage and measuring blood pressure will help to detect any signs of kidney involvement.
Other treatment will depend on whether complications develop. If the kidneys are affected then treatment may include steroids and other medicines to help reduce the abnormal reaction caused by the immune system (these medicines are called immunosuppressants). Sometimes medication to lower blood pressure is needed if high blood pressure develops because of kidney involvement.
Find out more about the treatments for HSP.
What is the outlook?
The long-term outlook (prognosis) mainly depends on whether and how badly the kidneys have been affected:
- If HSP does not affect the kidneys, most people make a full recovery within about four weeks and have no lasting problems.
- Kidney involvement is only serious in about 1 in every 10 people with HSP. The kidneys can become so badly affected that kidney failure can develop.
HSP may come back within six months of first having HSP. It is more likely to come back if the kidneys have been affected.
Further reading and references
Saulsbury FT; Henoch-Schonlein purpura. Curr Opin Rheumatol. 2010 Sep22(5):598-602.
Weiss PF, Klink AJ, Localio R, et al; Corticosteroids may improve clinical outcomes during hospitalization for Pediatrics. 2010 Oct126(4):674-81. Epub 2010 Sep 20.
Watson L, Richardson AR, Holt RC, et al; Henoch schonlein purpura--a 5-year review and proposed pathway. PLoS One. 20127(1):e29512. doi: 10.1371/journal.pone.0029512. Epub 2012 Jan 3.
Henoch-Schönlein purpura; DermNet NZ
I am going through the worst.flare up.of hsp that I have had in 18 years the rash and pain is from the shoulders to my feet and the pain is awful. I wish I didn't have this. There is no cure . It's...Steph1609
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