Intrafamilial Phenotypic Variability and Dental Management of Ectodermal Dysplasia in Three Siblings: A Case Report

Ectodermal dysplasia (ED) is a heterogeneous group of inherited disorders affecting ectoderm-derived structures, with dental anomalies representing a major clinical concern. This case report describes three siblings from a Tunisian family presenting marked intrafamilial phenotypic variability of ED. Clinical and radiographic findings revealed variable patterns of oligodontia (ranging from two to eight missing teeth), peg-shaped teeth, taurodontism, and craniofacial skeletal discrepancies. Cephalometric analysis demonstrated altered dentoalveolar development, whereas the adolescent patient reported significant psychosocial distress. Management included minimally invasive composite reshaping in two patients and a removable partial denture in the youngest child to restore function and vertical dimension, combined with orthodontic referral and longitudinal growth monitoring over a 2-year follow-up period. Despite a shared genetic background, the severity and clinical expression varied considerably, requiring individualized therapeutic strategies. This report emphasizes that early, growth-adapted, multidisciplinary management is essential in ED and that conservative and interim prosthetic approaches can significantly improve functional, aesthetic, and psychosocial outcomes during childhood.