Immunofluorescence microscopy of epidermodermal junction components in serial cryosections from the perilesional skin of a patient with generalized atrophic benign epidermolysis bullosa (GABEB) showed broken line‐like staining of both BPAG2 (180‐kDa bullous pemphigoid antigen) and uncein (antigen of 19‐DEJ‐l monoclonal antibody), whereas integrin α6 and laminin 5 were continuously expressed along the basement membrane zone. Immunoelectron microscopy revealed a mosaic distribution of the BPAG2/uncein positive and negative cells. BPAG2, a candidate protein of GABEB, probably has a close connection with uncein, an anchoring filament component.