先天性大疱性表皮松解症的超微病理诊断  

The ultrapathological diagnosis of epidermolysis bullosa

作　　者：汤秀英[1] 朱学骏[2] 任雅丽[1] 王书合[1] 柴立军[1] 程明[1] 

机构地区：[1]北京大学第一医院电镜室,北京100034 [2]北京大学第一医院皮肤性病科,北京100034

出　　处：《电子显微学报》2010年第5期482-485,共4页Journal of Chinese Electron Microscopy Society

摘　　要：目的:应用电镜观察先天性大疱性表皮松解症(EB)的超微结构特点并进行分类诊断。方法:按电镜常规进行标本制备,应用透射电镜对22例EB的皮肤活检进行超微结构观察。结果:超微病理诊断单纯型(EBS)15例,交界型(JEB)1例,营养不良型(DEB)6例。EBS的表皮基底层见水疱和裂隙样结构,基底层细胞变性及空泡形成,张力丝断裂、缩短及凝聚。JEB的水泡在基底膜的透明板,半桥粒发育不良、数目减少。DEB水疱位于真皮内,锚纤维缩短、减少。结论:电镜超微病理观察对EB的分类诊断有重要的辅助诊断价值。Objective： To study the ultrapathological and diagnosis classification of epidermolysis bullosa（EB） by using electron microscopy.Methods： The skin biopsy specimens were obtained from 22 patients with EB and processed by routine thinsection electron microscopy.Results： Under electron microscopy,22 cases were diagnosis as having 15 cases of epidermolysis bullosa simplex（EBS）,1 case of junctional epidermolysis bullosa（JEB）,and 6 cases of dystrophic epidermolysis bullosa（DEB）.In EBS,the vesicles of various size and shape accompanied with degeneration and vacuolization of basal cells and brokendown and clumping of tonofilaments were seen in the basal.In JEB,the vesicles were in the lamina lucida and simi-desmosomes were poorly developed and reduced in number.In DEB,the vesicles were seen in the dermis and the anchoring fibrils appeared shortening and reducing in number.Conclusion： Electron microscopic examination is an important aid in ultrapathological diagnosis and classification of EB.

关 键 词：表皮松解症 超微病理 电镜 

分 类 号：R758.5[医药卫生—皮肤病学与性病学]