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Henoch Schonlein Purpura

Also known as: Schönlein–Henoch purpura, Anaphylactoid Purpura, Purpura Rheumatica, IgA vasculitis

Background

Henoch Schonlein purpura (HSP), also known as IgA vasculitis (IgAV) is caused by vasculitis (an inflammation of blood vessels). It is not a genetic condition, which means that it is not passed down through families. The syndrome is usually seen in children, but it may affect people of any age. It is more common in boys than in girls.

Credits

Medical text written October 1996 by Dr AP Windrow, Consultant Paediatrician, Kingston Hospital, Kingston upon Thames, UK. Last updated November 2014 by Dr David Jayne, Consultant, Addenbrooke's Hospital, Cambridge, UK.

What are the symptoms?

Symptoms of HSP include:

  • painful, occasionally swollen joints
  • purpura (red/purple skin rash, which fails to go white with pressure)
  • abdominal (tummy) pain
  • kidney inflammation (see entry Glomerulonephritis).

Generalised abdominal pain may be caused by inflammation and swelling of the wall of the intestines or, more rarely, by a twisting inversion of the lining of the large intestine (bowel), which is called an acute intussusception.

What are the causes?

HSP usually occurs after an infection, often a viral respiratory tract infection and is due to an abnormal response of the immune system. It is unclear why this occurs.

How is it diagnosed?

Examination of the skin will reveal the typical rash seen in HSP that starts in the feet and can involve the legs, trunk and arms. Joint tenderness will also be clear from a physical examination. In some cases, a skin biopsy, removal of a small piece of skin tissue for analysis, may be required. A urine sample may be tested to look for haematuria (blood) and proteinuria (protein) in the urine. Blood and protein get into the urine because of the involvement of the kidneys in HSP. Abdominal involvement may be investigated by a CT scan.

How is it treated?

The natural course of the disease in children is to settle and not return.There is no specific treatment. Most cases go away on their own without treatment. If symptoms persist, corticosteroids, such as prednisone, may be required. Steroids dampen down the response of the immune system and will improve symptoms. If acute intussusception occurs then surgery may be necessary to correct this. Rarely other treatments are required if the disease is severe or if attacks of disease keep recurring. This is more common in adolescents and adults.

Whilst kidney involvement is common, occurring in up to 60 per cent of sufferers, serious kidney damage is very uncommon. Haematuria may persist for months if regular urine testing is performed. If kidney involvement is severe or if it persists, a kidney biopsy may be performed.

Recurrent HSP may recur in association with subsequent infections. However, the chance of a full recovery is excellent.

Inheritance patterns and prenatal diagnosis

Is there support?

Henoch Schonlein Purpura Support Group

Tel: 07766 681 077
Email: hsphelp@inbox.com

This is a small contact group, established in 2003. It offers a listening ear and, where possible, linking for affected adults and families of affected children.

Group details last updated February 2013.