Linear IgA Disease
This page covers the following:
What is Linear IgA disease?
Linear IgA disease is an autoimmune disease, as those described elsewhere, where the immune system attacks structures in your skin layers - your body produces antibodies against its own mucous membranes or skin which prevent the binding systems between the first two layers of skin and mucous membranes from functioning. When these structures are damaged, skin blisters may form.
There are different types of autoantibodies, and in this disease, the autoantibodies are of the IgA type and are deposited under the outer layer of the skin (the epidermis) in a line (linear). It is a very rare blistering condition of the skin in adults. A similar condition affecting children is known as Chronic Bullous Disease of Childhood (CBDC).
It is not contagious, and is not hereditary.
It can affect people of all ages, ethnic origins and genders, particularly in the drug-induced form. It is the most common autoimmune bullous disease of children.
What are the symptoms of Linear IgA disease?
Symptoms range from mild itching to a severe burning sensation. You will probably experience an itchy rash with small blisters, often arranged in clusters or rings. The blisters can come up all over your body and limbs, or just affect a few localised areas. The blisters may show the ‘string of pearls sign’ which describes blisters on the rim of red patches. Blisters may heal to leave temporarily discoloured areas of skin, particularly if you have darker skin. Most affected adults have blisters or ulcers in the mouth, eyes or the genital region. It does not affect your internal organs.
In children, the rash often occurs in the genital region and on the face especially around the mouth.
How will I be treated?
In forms triggered by drugs, then, stopping the medication implicated and applying cortisone creams are generally sufficient.
Treatment choice depends on the severity of the disease. Mild disease can be treated with topical steroids alone.
How long will it last?
In drug-induced forms, evolution is usually positive after discontinuation of the drug in question.
Children's forms usually heal easily with simple treatment. In adults, your disease usually progresses in successive attacks. It can be severe in the absence of treatment.
Can it be cured?
Treatment suppresses linear IgA disease but does not cure it. Skin lesions tend to heal without leaving permanent scars. However, ulcers in the eyes, mouth or genital region can leave scars or cause permanent damage.
Where can I find out more?
The British Association of Dermatologists have produced a patient information leaflet. DermNet NZ have a useful page. The Primary Care Dermatology Society also have a dedicated page.
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