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Epidermolysis bullosa acquisita

David T Woodley, MD
Mei Chen, PhD
Gene Kim, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD


Epidermolysis bullosa acquisita (EBA) is a rare, sporadic, subepithelial, mucocutaneous blistering disease that usually develops in adulthood. EBA is classically described as a mechanobullous disorder characterized by skin fragility, noninflammatory tense bullae, milia, and scarring. Alternatively, EBA can present as an inflammatory bullous eruption reminiscent of bullous pemphigoid or other subepithelial autoimmune blistering diseases.

The pathogenesis of EBA involves the production of antibodies against type VII collagen, a major component of anchoring fibrils in the basement membrane zones of skin and mucosa (figure 1). The immune-mediated disruption of the anchoring fibrils likely contributes to cleavage within the basement membrane zone and clinical blistering.

The pathogenesis, clinical manifestations, and management of EBA will be discussed here. Hereditary epidermolysis bullosa is reviewed separately. (See "Epidemiology, pathogenesis, classification, and clinical features of epidermolysis bullosa" and "Diagnosis of epidermolysis bullosa" and "Overview of the management of epidermolysis bullosa".)


Although the name "epidermolysis bullosa acquisita" arose from the original view of EBA as a disorder reminiscent of recessive dystrophic epidermolysis bullosa (RDEB), EBA and epidermolysis bullosa are distinct diseases. RDEB is a hereditary disease that results from mutations in the COL7A1 gene that encodes type VII collagen. In contrast to EBA, RDEB usually first presents in infancy. (See "Diagnosis of epidermolysis bullosa".)


EBA is a rare disorder of uncertain incidence. Although pediatric cases have been reported, adults are most commonly affected [1-5]. Increased risks for EBA based upon gender, ethnicity, or geographic location have not been definitively established.

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Literature review current through: Nov 2017. | This topic last updated: Sep 28, 2017.
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