Epidermolysis bullosa acquisita (EBA) is a rare autoimmune disorder that causes the skin to form tense blisters in response to minor injury. This is an acquired autoimmune disease and the initiating event that leads to disease is unknown. This means that the immune system attacks healthy cells by mistake. In EBA, the body mistakenly attacks collagen, a type of protein in the skin that helps to keep the skin intact. Epidermolysis bullosa is a genetic form of the disease that occurs through inheritance of specific genes, unlike EBA, which occurs sporadically in people with no history of the condition in their families.
EBA usually presents in adulthood, most commonly on the hands, feet, knees, elbows, and buttocks. It can also affect the mouth, nose, and eyes. Males, females and individuals of all races can be affected. Some affected people have other medical problems such as Crohn's disease, systemic lupus erythematosus, amyloidosis, and multiple myeloma.
Symptoms usually occur in the fourth decade of life and may include widespread, tense, blood- or pus-filled blisters with generalize redness and itching. The blisters may heal with significant scarring so treatment is aimed to protect the skin and stop blister formation, promote healing, and prevent complications such as infection of ruptured blisters.
EBA is a chronic inflammatory disease with periods of partial remissions and exacerbations. If treated and cared for properly, patients can expect to live a normal life. Due to the rarity of EBA, there is insufficient research regarding the best treatment. However, because EBA is considered an autoimmune disease, it is reasonable to use immunosuppressive agents such as azathioprine with systemic steroids to reduce inflammation and modify the body’s exaggerated response. In patients with involvement of the eyes and mouth, it may be beneficial to seek consultation with an eye doctor and dentist. In addition, hard foods or acidic foods such as orange juice should be avoided.
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