Enamel dysplasia with hamartomatous atypical follicular hyperplasia: review of the literature and report of a case.

This article documents an additional case of a rare syndrome, reported only in black persons in South Africa. It is characterised by hamartomatous atypical follicular hyperplasia with central odontogenic fibroma (WHO-type)-like features attached to multiple impacted teeth, and with generalized enamel dysplasia showing features of hypoplastic amelogenesis imperfecta. Other features associated with the syndrome, but not present in all reported cases, include open-bite malocclusion, gingival overgrowth, hypodontia, pulpal calcifications and aberrant root formation of the unerupted teeth. Our present case shows the additional feature of impacted supernumerary teeth. As we cannot find any reports in the literature of the occurrence of amelogenesis imperfecta-like enamel dysplasia associated with hamartomatous follicular hyperplasia showing the features of WHO-type central odontogenic fibroma, with or without the additional features mentioned above, we propose to name this syndrome Enamel Dysplasia with Hamartomatous atypical Follicular Hyperplasia (EDHFH) syndrome.