A Case of Total Anodontia with X-linked Recessive Inheritance

Abstract

A 5 year 11 month old boy who was diagnosed as an anhidrotic ectodermal dysplasia associated with total anodontia was reffered again to our clinic with the request to re-make a full denture. Consequently, detail examination and questioning were conducted and the results added to the previously obtained data.
Then following findings were obtained
1) The patient had the 3 symptoms consisting of sparse hair, no teeth (total anodontia)and no sweat glands together with such characteristic features as sparse eyebrows, wide forehead, protrusive mental region, saddle nose and warped lips.
2) In the development of jaw and face, it was revealed that the mandible grew normally but the maxilla grew recessively. The length and width of alveolar ridge increased in length for 3 years.
3) The cousin on his mother's side had similar symptoms such as sparse hair, small number of teeth (partial anodontia) and no sweat glands, and baced on the questioning, his mother's brother was suspected of having the same symptoms. However, no such similar signs were seen in his mother, his mother's sister and her daughter.
From these results, it was logically thoght that this case was caused by X-linked recessive inheritance.