Epidermolysis bullosa

Epidermolysis bullosa

Chris Schach

Author Bio -

 

Key Points
*Unusual condition resulting in blisters on the skin
*Condition more prevalent in middle-aged to older people
*This disorder is one of several somewhat similar conditions in which the body’s immune system targets certain proteins in the skin resulting in damage and blister formation 

Epidermolysis bullosa acquisita (EBA) is a very rare disease in which tense blisters appear at various possible skin sites, often where trauma is likely. EBA is not inherited and usually presents in adult life.

EBA blisters tend to be localized to areas that are easily injured such as the hands, feet, knees, elbows, and buttocks. Sometimes blisters form in the mouth, nose and eyes. EBA may mimic other inflammatory blistering diseases.

EBA usually occurs in the fourth and fifth decade of life. Males and females of all races can be affected. EBA is an autoimmune blistering disease, in which cells of the immune system target and damage certain skin proteins, causing blistering. Some patients with EBA have been reported to have other immue disorders such as Crohn's disease, systemic Lupus erythematosus, Amyloidosis, multiple myeloma and lymphoma.

EBA has a few variants. In more inflammatory forms, there will be generalized redness, itching, and plaque formation.

Differential Diagnosis (Other conditions with similar appearance)

Bullous pemphigoid
Cicatrical Pemphigoid
Linear IgA dermatosis
Lupus erythematosus, Bullous
Porphyria cutanea tarda

Diagnosis
Key Points
*Diagnosis is based on appropriate clinical findings in conjunction with skin biopsy including immunofluorescence.

A battery of tests should be performed to diagnose EBA, which might include skin biopsy of a blister, direct immunofluorescence, and possibly indirect immunofluorescence.

Treatment
Key Points
*The primary aim of treatment is protecting the skin and preventing blister formation, promote healing and prevent complications.
*There are several medications that may be effective.

The primary aim in the treatment of EBA is to protect the skin and stop blister formation, promote healing and prevent complications. Because EBA is an autoimmune disease, it is often necessary to use immunosuppressive medications.  These might include Azathioprine, Dapsone, Colchicine, corticosteroids, Mycophenolate mofetil, and intravenous Immunoglobulin.  EBA is a chronic disorder for most patients, so prolonged treatment is often required.

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