A case of epidermolysis bullosa hereditaria--dominant dystrophic type of Cockayne and Touraine.
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.
Basement Membrane↗