Abstract
Amelogenesis imperfecta is known as a hereditar y disease which is confined only to the enamel, while the other hard tissues are normal in quantity and quality. In this article, we examined two sisters,13 years old and 11 years o l d, as well as their mother, all of whom were affected with amelogenesis imperfecta. In addition to the routine dental examination, in vivo observation of the detailed surface structures of the affected teeth was performed using a scanning electron micrscope (SEM) by means of the replica technique. The follow i ng results were obtained: 1) The disease is restricted to all permanent teeth, and the appearance of the enamel surface showed, what is called, welliger Schmelz.2) Based on the clinical findings and SEM findings, this case could be classified as the hypoplastic type according to Witkop and Rao.3) The mode of inheritance in this case is the autosomal dominant or sex-linked dominant type judging from their pedigree.4) For the observation of the surface structure of enamel in vivo, the replica technique was shown as the most reliable method to observe the detailed structure of tooth in vivo, and exhibited an excellent reproducibility.
