Figure 2.

Different clinical forms of EBA. All patients were documented at the first visit in a center for auto-immune bullous disease. (Left) Patient with a classical/mechanobullous form of EBA: lesions are preferably localized to the extensor skin surfaces and trauma-prone sites, i.e., dorsal hands knees elbows and ankles. Tense or flaccid bullous lesions are surrounded by non-inflamed skin; erosions are covered or not by crusts; one erosion with angular contours had been induced by adhesive plaster; old lesions have healed with milium formation and/or are atrophic papery scar. (Middle) Patient with a BP-like form of EBA, with little blistering: urticarial plaques with small or large bullous lesions as in BP, but location of lesions on extensor areas of limbs, hands and scalp, and scars and extensor areas of the face (not shown) and limbs (atypical for BP). (Right) Patient with a BP-like form of EBA, with extensive blistering: bullous lesions and erosions on erythematous skin in flexural areas of limbs (tight and arm) as in BP but also bullous lesions and erosions on normal skin and involvement of extensor area of the limbs and scalp, atypical for a BP involvement of the scalp (not shown) and both flexural and extensor areas of limbs extremities with bullous lesions and erosions on erythematous but also normal skin. The tongue and the lips are the most frequent sites of mucosal lesions in all EBA variants. Other mucosal lesions (not shown) are possible regardless of the variant of EBA The involvement of nasal and buccal mucous membrane are visible in all EBA variants.