Objective: Ectodermal dysplasia (ED) is a rare hereditary disease that affects ectoderm-derived tissues such as skin, nails, hair, sweat-glands and teeth. In this study it is aimed to evaluate systemic and dental findings of patients with ED who applied to our faculty hospital; clinically and radiologically.

Material and Method: We retrospectively evaluated 49 cases of ED who were admitted to Dicle University Faculty of Dentistry with complaint of lack of teeth between 2006-2017. During clinical evaluation, hair, nails, skin, teeth, nose, sweat glands and similar malformations were examined. On radiological examination, dental germs, jawbone and tooth roots were examined using conventional radiography and conical beam computed tomography.

Results: A total of 49 patients consisting of 25 female, (51.1%) and 24 male patients (48.9%) with the mean age of 11,9±4,6 years were evaluated in our study. Oral findings of patients with ED in the study revealed; 100% (n =49) absence of the tooth, 100% (n =49) mandibular protrusion, 75.51% (n =37) conical tooth and 6.12% (n =3) root shape anomalies. In the clinical examination, the sweating problem was determined as 77.55% (n =38), hair-bristle anomaly 95.91% (n =47) and abnormal fingers and nails 83.67% (n =41).

Conclusion: As the main findings of ED are in the oral and maxillofacial region, aesthetic and chewing problems arise. For this reason, ED has a special place in dentistry requiring multidisciplinary treatment. Coordinated study of medical doctors and dentists is very important for increasing the quality of life in these patients.