Dentin dysplasia type I. Clinical, morphologic, and genetic studies of a case.

This report documents a case of dentin dysplasia Type I in a 17-year-old boy and two members of his family. The clinical, radiographic, histologic, and ultrastructural findings indicate that this condition is distinct from other heritable defects of dentin. The entity is transmitted as an autosomal dominant trait and is characterized by teeth which have a normal color and exhibit pulpal obliteration, short roots, periapical radiolucencies, and spontaneous exfoliation. Our ultrastructural findings in agreement with those reported by Sauk and associates.