Ectodermal dysplasia: A retrospective evaluation of the clinical findings of forty-four cases in the 0-16 years age

Aim: The aim of this study was to review the craniofacial anomaly results of children diagnosed with ectodermal dysplasia (ED) and to identify the oral requirements of ED cases. Methodology: The data of this study were obtained by taking clinical examinations and radiographs on forty-four (44) children (22 females and 22 males), 0-16 years aged, who were admitted to the Dicle University, Faculty of Dentistry due to dental problems and were diagnosed with ED. The number of affected siblings was ascertained, and systemic findings were evaluated. Malformations in the hair, nails, nose, skin, lips, and teeth were clinically examined. Results: The most common clinical findings were sparse hair, dry skin, sweating problems, respiratory difficulty, saddle nose, a history of fever, hearing loss, and deformation in the nails. In the intraoral and radiological examinations, findings were evaluated of conical teeth, protuberant lips, prosthetic rehabilitation, impacted teeth, and abnormal root resorption. The rates of ED in the siblings of the ED cases were determined as 39.2% in the siblings of male cases and 37.9% in the siblings of female cases. Conclusion: The frequency rates of the anomalies seen in ED obtained in this study can be considered important as a guide for further studies of individuals with ED. When multiple missing teeth and conical teeth are encountered, the dental practitioner should investigate whether or not there are other symptoms of ED, and it must not be forgotten that the dentist may be the first step in the diagnosis of this genetic irregularity.