Rare diseases with indication for dental implant therapy: Ectodermal dysplasia

Ectodermal dysplasia describes a heterogeneous group of hereditary congenital malformations with developmental dystrophies of ectodermal structures that can manifest with oligodontia. The aim of this study was to analyze the oral health-related quality of life (OHRQoL) of patients with ectodermal dysplasia who had not yet received implant therapy and to depict the various phenotypes in this paper. To achieve this, patients with ectodermal dysplasia and multiple dental agenesis or anodontia seen in the consultation hours for "rare diseases with oral involvement" were asked about their OHRQoL using the Oral Health Impact Profile-14 (OHIP-14) questionnaire. A total of 12 patients from 11 families without genetic relationship participated in the study. The mean value of the OHIP-14 total score was 25.5. All patients had tooth agenesis; on average, 22.67 teeth were congenitally missing. Patients with ectodermal dysplasia without masticatory function and implant rehabilitation showed high OHIP-14 scores; in these cases, implant placement might contribute to higher OHRQoL. Ectodermal dysplasia must always be considered as a differential diagnosis in cases of oligodontia even if there are no external features.