Kirtschig G, Caux F, McMillan J R, Bedane C, Aberdam D, Ortonne J P, Eady R A, Prost C
Department of Dermatology, University Hospital, Marburg, Germany.
Br J Dermatol. 1998 Jan;138(1):125-30. doi: 10.1046/j.1365-2133.1998.02038.x.
We report the case of a 72-year-old man with clinical features resembling those of non-lethal junctional epidermolysis bullosa associated with IgG autoantibodies to the beta chain of laminin-5. The patient presented with a sudden onset of blistering and severe fragility of the skin and mucous membranes resulting in atrophic scars. Electron microscopy showed that the blistering arose in the lamina lucida. Indirect immunofluorescence indicated that the autoantibodies bound to the dermal side of 1 mol/L NaCl-split skin, and both direct and indirect immunoelectron microscopy demonstrated antibody binding to the lamina densa. Postembedding immunogold electron microscopy also revealed labelling in the lamina lucida beneath the hemidesmosomes. On immunoblotting, we found the autoantibodies to comigrate with the beta chain of laminin-5. Following the nomenclature of inherited junctional epidermolysis bullosa with mutations of the laminin-5 gene, we propose the name acquired junctional epidermolysis bullosa for this newly recognized disease.
我们报告了一例72岁男性病例，其临床特征类似于与层粘连蛋白-5β链IgG自身抗体相关的非致死性交界性大疱性表皮松解症。患者突然出现水疱，皮肤和黏膜严重脆弱，导致萎缩性瘢痕。电子显微镜检查显示水疱出现在透明层。间接免疫荧光表明自身抗体与1mol/L NaCl分离皮肤的真皮侧结合，直接和间接免疫电子显微镜均显示抗体与致密层结合。包埋后免疫金电子显微镜也显示半桥粒下方透明层有标记。免疫印迹分析发现自身抗体与层粘连蛋白-5β链迁移一致。按照层粘连蛋白-5基因突变所致遗传性交界性大疱性表皮松解症的命名法，我们将这种新认识的疾病命名为获得性交界性大疱性表皮松解症。
