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IgA vasculitis

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Introduction

IgA vasculitis (va-skyu-lai-tuhs), also known as Henoch-Schönlein purpura (hee-nok shon-line per-per-a) or HSP, is a type of inflammation of the small blood vessels known as vasculitis. It commonly affects the skin and kidneys. This condition often causes a rash on both legs and is sometimes associated with joint and tummy pain. It can occasionally cause kidney issues in the long term. It is the most common form of systemic vasculitis in children.

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Who gets it?

It affects 2 per 10 000 children and mainly affects school aged children but

can occasionally affect toddlers and adults. It occurs more commonly in boys than girls. 

What causes it?

It is not completely understood what causes IgA vasculitis, however it commonly occurs after a viral upper respiratory infection. It is likely due to a combination of genetic, environmental and immunologic factors which makes some children develop the disease where others do not. It cannot be passed on from one child to another.

What does it look and feel like?

Looks like… 

  • It initially can look like multiple red flat or raised skin patches which can turn into the the raised purple spots that may look like bruises.

  • The rash usually affects a child's ankles, lower legs and buttocks. However, in some cases, can affect their arms and body.

  • Rarely there can be raw areas on the skin (ulcers) and bleeding.

  • There maybe blood in urine (wee) or faeces (poo).

Feels like… 

  • There may be lumps on the skin. 

  • The skin can be itchy or painful.  

  • Your child may have sore or swollen joints, most commonly hips, knees and ankles. 

  • They may complain of tummy pain, nausea or vomiting.

What tests might be needed?

Your child's doctor will need to organise some tests to confirm the diagnosis and to find out what other organs this condition might be affecting.

  • Blood tests (blood count, blood clotting test, kidney function and liver function)

  • Urine test

  • Skin biopsy - a sample of the skin rash is taken to look under the microscope

  • Abdominal ultrasound or x-ray

What treatments are available?

What can I do?

Your child's symptoms will usually settle by themselves. Encourage your child to:

  • Avoid tight clothing

  • Rest 

  • Elevate legs 

Medical therapies 

  • Cream steroids can be given to treat the rash.

  • Pain relief, e.g. paracetamol, may be used to help relieve joint or abdominal pain.

  • Steroids by mouth, e.g. prednisone, may be used in some cases.

  • Rarely, other immune suppressive medications may be used in severe cases

What happens after treatment?

All children who have had IgA vasculitis must have close follow up with their doctor. In most children, symptoms will settle within 1 month. Occassionally your child may have another episode within the first 12 months. If there is permanent damage to the kidneys, they might need ongoing testing and follow-up by a nephrologist (kidney specialist).

What support is available?

Your GP might do some tests or refer your child to the emergency department for tests. They might also refer you to a dermatologist, paediatrician or nephrologist (kidney specialist.) All children will require a clear follow-up plan over the next 6-12 months.

Authors/Reviewers:

Author: Dr Chelsea Jones
Paediatric Reviewer: Dr Mayuri Sivagnanam
Editor in Chief: Dr Tevi Wain

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