| >> Patient Information and Leaflets
Please click here to download a printable version
Linear IgA disease
What are the aims of this leaflet?
This leaflet has been written to help you understand more about linear IgA disease. It tells you what it is, what causes it, what can be done about it, and where you can find out more about it.
What is linear IgA disease?
Linear IgA disease is a very rare blistering eruption of the skin. One type affects adults, usually over the age of 60; and another (also known as chronic bullous disease of childhood) affects children, usually under the age of five.
The term ‘linear IgA disease’ comes from the fact that a line of immunoglobulin of type A (IgA) can be seen when a small sample of skin (a biopsy) is prepared specially and examined under the microscope. This line of IgA lies just under the outer layer of the skin (the epidermis).
What causes linear IgA disease?
Infections and antibiotics sometimes trigger linear IgA disease. The IgA lying in the skin is made by the body’s immune system and seems to be directed against structures anchoring the outer layer of the skin to the deeper tissues (i.e. it is an autoimmune disease). When these structures are damaged, the skin blisters more easily than it should. Linear IgA disease is not infectious.
Is it hereditary?
No.
What are the symptoms of linear IgA disease?
These range from mild itching to a severe burning sensation.
What does it look like?
Patients have an itchy rash with small blisters, often arranged in rings. The blisters can come up all over the body and limbs, or just affect a few smaller patches. The ‘string of pearls sign’ is the presence of blistering lying around the rim of wheal-like red patches. In children, the rash usually occurs in the genital region. Blisters and ulcers can affect the mouth and eyes, but the internal organs remain clear.
How will it be diagnosed?
The diagnosis can be difficult, as linear IgA disease can mimic several other disorders. Confirmation of the diagnosis requires a skin biopsy in which a line of IgA under the epidermis can be shown up by a special test (direct immunofluorescence).
Can it be cured?
Treatment suppresses linear IgA disease but does not switch it off. However the disease does tend to go away by itself, taking 3 to 6 years to do so.
How can it be treated?
Most patients with linear IgA disease do well with special tablets such as dapsone, sulphonamides or steroids. Most will need to go on seeing a dermatologist as well as their own GP. |
- In adults, the tablet used most often is dapsone. This has some potential side effects, so treatment will not usually start until the diagnosis has been confirmed by a skin biopsy. All patients on dapsone get some decrease in haemoglobin (the oxygen-carrying part of red blood cells), which is usually dose-related and can be monitored with blood tests. However a few patients on dapsone get a rapid fall in their blood counts. For this reason, weekly blood tests are performed to begin with, and any unusual symptoms (e.g. a sore throat, dizziness or faintness) should be reported to your doctor. Dapsone also causes headaches in some patients.
- In children, the tablet used most often is one called sulphapyridine. This is generally safe, but can cause some side effects like those of dapsone.
- Many patients will get better slowly over several years and so need less treatment.
What can I do? |
- Ask to see an eye specialist if you are getting problems with your eyes – for example, redness and a sore gritty feeling.
- Cover your blisters and raw areas with sterile dressings
| Where can I get more information about linear IgA disease?
Web links to detailed leaflets:
www.thedoctorsdoctor.com/diseases/linear_iga_disease
www.dermnetnz.org/dna.bullous.pemphigoid/liniga.html
www.emedicine.com/derm/topic240.htm
The British Association of Dermatologists has also issued a related patient information leaflet on dapsone.
(While every effort has been made to ensure that the information given in this leaflet is accurate, not every treatment will be suitable or effective for every person. Your own doctor will be able to advise in greater detail)
BRITISH ASSOCIATION OF DERMATOLOGISTS
PATIENT INFORMATION LEAFLET
PRODUCED SEPTEMBER 2004
|
