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Epidermolysis Bullosa Acquisita

This page covers the following:

What is Epidermolysis Bullosa Acquisita?

Epidermolysis bullosa acquisita (EBA) is an autoimmune disease, caused by a disturbance in your immune system. The patient's antibodies (autoantibodies) go awry and attack the binding systems that enable the epidermis and dermis (for the skin) and/or the epithelium and chorion (for mucous membranes) to stick together. It is this that causes blisters.


Do not confuse epidermolysis bullosa acquisita (EBA) with hereditary epidermolysis bullosa (EBH). EBA is an autoimmune disease, EBH a genetic disease that affects children from birth. These two diseases are very different, even though they both form blisters: they are treated differently.


Epidermolysis bullosa acquisita can affect people of any age, ethnic origin and gender. The disease frequently affects young adults. It is also the most frequent autoimmune bullous disease in black-skinned people.  It is an extremely rare disease (less than 1 new case per million inhabitants per year in Western Europe).


The disease is not contagious, and it is not hereditary.

 

What are the symptoms of EBA?

There are several forms of epidermolysis bullosa acquisita with different symptoms:

  • Classic form

  • Brunsting Perry's form: an exceptional form manifested by extensive scalp erosions

  • Bullous pemphigoid-like form

  • Mucous membrane pemphigoid-like form

  • Form resembling linear IgA dermatosis


In the classic form, lesions are most often found on areas where the skin rubs hands, feet, knees and elbows. Your nails may also be affected.


Blisters are generally tight and surrounded by normal skin. They are sometimes very small (vesicles). Scars on mucous membranes are more problematic, which is why a rapid diagnosis is essential. The lesions evolve, forming scars (or fibrosis) that mark the skin, sometimes permanently. In epidermolysis bullosa acquisita, these scars are often prominent.

 

How will I be treated?

Since this disease can affect several mucous membranes, its management requires the involvement of several medical specialists. The choice of medication depends on the nature of the disease.

 

Where can I get more information?

We would recommend that you contact DEBRA, the charity for epidermolysis. There is also further information on DermnetNZ.

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