Ectodermal dysplasia: important role of complex dental care in its interdisciplinary management

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ABSTRACT

Aim

Despite the fact that ectodermal dysplasia (ED) is a rare disease, it is often seen in a tertiary clinic. ED affects ectodermal tissues such as skin, hair, teeth, nails, and sweat glands. Patients usually have sparse light hair, deformed nails, and dry skin. They suffer from dental abnormalities such as oligodontia (absence of 6 or more teeth) or complete anodontia; salivation can also be affected. The absence of teeth can be the overriding problem for both patients and their parents, and lead to substantial social ostracisation. This study aims to summarise the facts about the disease, especially dental treatment options based on data drawn from a representative Czech cohort.

Material and methods

The present article summarises the facts about ectodermal dysplasia (ED) in a cohort of 13 patients, where the following were evaluated: clinical manifestations of ED, pathogenic variants detected in selected candidate genes and dental treatment options from child removable dentures to fixed crowns and implants insertion. Three cases are described in detail and demonstrate approaches for different age groups.

Results

Our study highlights the need for awareness of the early signs of ED in dental and medical genetic practice. The ideal dental treatment plan includes interim removable dentures at a young age, orthodontic treatment in children and adolescents, and fixed restoration, including implants at a later date. With correct dental treatment, normal development of the patient’s jaw, as well as their self-esteem, is markedly improved.

Conclusion

Early diagnosis and active cooperation between the geneticist and dentist will facilitate cooperation with parents and patients and assure secondary prevention. It is preferable that the geneticist understands dental treatment options and can discuss these with patients/parents.

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