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INFORMATION FOR PATIENTS
Epidermolysis bullosa acquisita or EBA is an autoimmune bullous disease caused by autoantibodies that are directed against a substance called type VII collagen, which forms the anchoring fibrils of the dermoepidermal junction or its equivalent (the junction of epithelium and lamina propria) in the mucous membranes that line the mouth, the conjunctiva of the eye, the genital organs, the anus, the nose, the throat and the oesophagus. These antibodies bind to the anchoring fibrils and this causes the epidermis at the surface of the skin to detach from the underlying dermis, or the same process to occur in the equivalent tissues in the mucous membranes (epithelium and lamina propria). The result of this is a “bulla”, more commonly known as a “blister”, which contains a clear serum, and this is followed by “erosion”, commonly known as a “wound” when the top of the blister detaches.
EBA is the least common of all autoimmune bullous diseases. It affects 0.22 - 0.26 new patients per million inhabitants in Western Europe on a yearly basis, and these are twice as likely to be females than males. This disease is not inherited or contagious and this means that there is no reason for family members to be worried about developing it.
The diagnosis can be made by taking a blood sample and a tissue sample called a biopsy, which will indicate whether the autoantibodies against type VII collagen are present in the blood, skin or mucous membranes. These examinations are only carried out in highly specialised laboratories.
This disease is very difficult to treat. Firstly, treatment will consist of an attempt to stop the body producing the autoantibodies against type VII collagen that cause the disease. The choice of medicines will depend on how severe the disease is at the time of diagnosis. This will be assessed through a very thorough clinical examination which will involve a number of different specialist doctors (dermatologist, stomatologist, ophthalmologist, ENT, gynaecologist etc.): the treatment is immunomodulators (dapsone, colchicine) in mild or moderate cases and immunosuppressants (ciclosporin) in more serious forms of the disease. The use of biological treatments like rituximab is currently being examined. Steroids for general administration are rarely used. Topical steroids are only used in addition to a main treatment. When the skin lesions are widespread, ointment and dressings must be applied by a nurse.
This is a chronic disease and partial or full remission takes a long time to obtain. This remission comes at the price of dystrophic scarring and milia cysts on the skin although these do fade over time. Fragile skin will persist for a long time after the blisters have disappeared and doctors will need to assess the impact of this on the patients’ professional and personal lives. The most troublesome after-effect that patients are at risk of developing is scars affecting the eyes, throat or oesophagus as this can interrupt the normal functioning of these organs. Maintenance treatment to avoid recurrence is needed, as is regular and long-term follow-up.
SYNONYMS (CLINICAL FORMS)
Classic EBA, mechanobulllous EBA, inflammatory EBA, mucous membrane EBA, IgA-mediated EBA
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