enamel dysplasia with hamartomatous atypical follicular hyperplasia

Enamel dysplasia with hamartomatous atypical follicular hyperplasia is 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.

It has been proposed to name this syndrome Enamel Dysplasia with Hamartomatous atypical Follicular Hyperplasia (EDHFH) syndrome.

References

 Enamel dysplasia with hamartomatous atypical follicular hyperplasia: review of the literature and report of a case. Feller L, Wood NH, Anagnostopoulos C, Bouckaert M, Raubenheimer EJ, Kramer B, Lemmer J. SADJ. 2008 Mar;63(2):096-7, 100-1. Review. PMID: 18561808