Mosaic expression of uncein and 180-kDa bullous pemphigoid antigen in generalized atrophic benign epidermolysis bullosa |
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| Authors: | Y. MATSUMURA Y. HORIGUCHI K. TODA H. FUJII S. KORE-EDA T. TACHIBANA K. OHTA H. OKAMOTO S. IMAMURA |
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| Affiliation: | Department of Dermatology, Faculty of Medicine, Kyoto University, Sakyoku, Kyoto 606, Japan;Department of Dermatology, Osaka Red Cross Hospital, Osaka, Japan |
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| Abstract: | 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. |
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