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Epidermolysis bullosa acquisita in Adult
See also in: External and Internal Eye
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Epidermolysis bullosa acquisita in Adult

See also in: External and Internal Eye
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Contributors: Christine S. Ahn MD, FAAD, William W. Huang MD, MPH, FAAD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Epidermolysis bullosa acquisita (EBA) is a rare, chronic, acquired autoimmune blistering disease of the skin and mucous membranes. Characterized clinically by the development of subepidermal tense vesicles and bullae, EBA typically occurs in adults, although it can occur in individuals of any age. Patients demonstrate autoantibodies directed against type VII collagen, a major component of the skin's basement membrane.

There are several clinical forms of EBA, as defined by the International Bullous Diseases Group in 2018:
  • Classical / mechanobullous form: a noninflammatory form that affects trauma-prone sites and extensor skin surfaces 
  • Nonclassical / nonmechanobullous bullous pemphigoid (BP)-like form: an inflammatory form with features characteristic of BP mixed with atypical lesions for BP, such as skin fragility and milia, that is characterized by a generalized eruption of vesicles and bullae
  • Mucous membrane form: a mucous membrane pemphigoid-like form, which predominantly affects mucous membranes (mouth, pharynx, conjunctiva, genitalia)
  • Brunsting-Perry-type form: a chronic recurrent blistering dermatosis of the head and neck that presents with scarring alopecia
  • IgA form: a form that may resemble linear IgA bullous dermatosis and presents with linear IgA deposits in the basement membrane
EBA can also occur in different clinical settings; there are reports of EBA occurring as a paraneoplastic phenomenon, in association with psoriasis vulgaris, and in the setting of inflammatory bowel disease or other autoimmune diseases, particularly systemic lupus erythematosus (SLE).

EBA typically has a slow onset and a chronic clinical course. Scarring may produce secondary dysfunction, particularly of the hands and fingers. Other sequelae include hair and nail loss, esophageal stenosis, periodontal disease, malnutrition, and blindness.

Related topic: Epidermolysis bullosa simplex

Codes

ICD10CM:
L12.30 – Acquired epidermolysis bullosa, unspecified

SNOMEDCT:
2772003 – Epidermolysis bullosa acquisita

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Last Reviewed: 09/27/2018
Last Updated: 10/05/2018
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Epidermolysis bullosa acquisita in Adult
See also in: External and Internal Eye
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Epidermolysis bullosa acquisita (Classical Mechanobullous Form) : Extensor distribution, Scarring alopecia, Hands, Skin erosions, Knees
Clinical image of Epidermolysis bullosa acquisita
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