Epidermolysis bullosa acquisita
- David T Woodley, MD
David T Woodley, MD
- Professor and Chair, Department of Dermatology
- The Keck School of Medicine, University of Southern California
- Mei Chen, PhD
Mei Chen, PhD
- Professor and Director of Research - Department of Dermatology
- University of Southern California
- Gene Kim, MD
Gene Kim, MD
- Assistant Professor of Pathology and Dermatology
- University of Southern California
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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- Type VII collagen
- Pathogenic mechanism
- Genetic factors
- CLINICAL MANIFESTATIONS
- Classical (noninflammatory) EBA
- Inflammatory EBA
- Possible rare clinical presentation
- ASSOCIATED DISORDERS
- Initial evaluation
- - Histopathology
- - Direct immunofluorescence
- - Immunofluorescence on basement membrane zone-split skin
- - Additional studies
- DIFFERENTIAL DIAGNOSIS
- General measures
- Approach to treatment
- - Colchicine
- - Dapsone
- Refractory disease
- Other therapies
- SUMMARY AND RECOMMENDATIONS