エナメル質形成不全を伴った先天性表皮水疱症

概要

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A 20-year-old student with epidermolysis bullosa was referred for the examination of teeth and oral mucosa in June, 1983.<BR>During the physical examination, extensive erosion and blistering were recognized over the limbs and arms. The fingernails and toenails were absent, but scarring and milia were not seen. By the oral examination, enamel hypoplasia of all teeth was seen. Panoramic radiography showed eight impacted teeth. Biopsies were performed on the gingiva and skin of blistering and clinically intacted areas. Histological findings showed junctional blister formation.<BR>Upon ultrastructual examination of the gingiva and skin, the number of hemidesmosomes was found to be less than in normal individuals.<BR>Diagnosis was made as the Disentis type of epidermolysis bullosa hereditaria with junctional blistering. Our report represents a second case in Japan and the first case in the dental province.<BR>We think that it is vital to have a thorough understanding of the oral manifestations of epidermolysis bullosa.
社団法人日本口腔外科学会の論文