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 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