Epidermolysis Bullosa Simplex (Weber-Cockayne) and Dystrophica: Light and Electron Microscopic Studies

• Published in: Skin research Vol. 31; no. 1; pp. 80 - 86
• Main Authors: KATO, Haruhisa, TSUJI, Takuo, HAMADA, Toshio
• Format: Journal Article
• Language: English
• Published: Meeting of Osaka Dermatological Association 1989
• Subjects: anchoring fibril; epidermolysis bullosa dystrophica; epidermolysis bullosa simplex (Weber-Cockayne); ultrastructural studies
• ISSN: 0018-1390; 1884-541X
• DOI: 10.11340/skinresearch1959.31.80

Three cases of epidermolyis bullosa, two of dystrophic type and one of simplex type (Weber-Cockayne), are reported. An electron microscopic examinasion of a bullous lesion in one patient with dystrophic type showed bulla formation in the uppermost dermis just below the basal lamina. Damaged or rudimentary anchoring fibrils were seen along the basal lamina. Electron microscopic findings of scar lesion in another patient revealed decrease in number of anchoring fibris. In the case of epidermolysis bullosa simplex (Weber-Cockayne), a positive family history of the disease was seen. Light microscopic findings revealed intraepidermal vesiculation and frank blister formation especially in the basal layer. Electron microscopic examinations showed cytolysis of suprabasal spinous cells and basal cells together with lysis of desmosomes and clumping of tonofilaments. It is supposed that an absence or attenuation of normal anchoring fibrils caused blisters in dystrophic epidermolysis bullosa. In the simplex case, activation of cytolytic enzymes by warming the skin or mechanical trauma might play a role in blister formation. In addition, clumping of tonofilaments following an abnormal alteration of cytoskeleton may cause blister formation.