Yotsumoto S, Setoyama M, Hisadome H, Tashiro M, Murata F
Department of Dermatology, Faculty of Medicine, Kagoshima University, Japan.
Dermatology. 1993;186(3):201-4. doi: 10.1159/000247346.
We report a patient with the Cockayne and Touraine type epidermolysis bullosa dystrophica domains. A 6-year-old Japanese female developed blisters and erosions on the extremities 3 months after birth. Immunohistology showed a linear binding pattern of the monoclonal antibody against type VII collagen (LH:2) on the epidermal basement membrane. By means of electron microscopy and morphometric analysis, it became apparent that the anchoring fibrils were rudimentary in structure and reduced in number.
我们报告了一名患有科凯恩和图赖讷型营养不良性大疱性表皮松解症区域的患者。一名6岁日本女性在出生后3个月时四肢出现水疱和糜烂。免疫组织学显示，针对VII型胶原的单克隆抗体（LH:2）在表皮基底膜上呈线性结合模式。通过电子显微镜和形态计量学分析，明显可见锚定原纤维结构发育不全且数量减少。
