Dentin dysplasia, type II: a rare autosomal dominant disorder

Dentin dysplasia, Type II, is a rare autosomal dominant disorder. The primary teeth are amber and translucent and the pulp chambers are obliterated. The permanent teeth have a normal to brown-gray coloration and a thistle-tube pulp configuration with multiple true denticles. To date, only five families with this disorder have been reported. This article presents two additional families. Light and scanning electron microscopy of an affected primary incisor showed the dentin, including the mantle layer, to be highly disorganized throughout. Possible pathogenic events associated with the phenotype are discussed.