Ectodermal dysplasia - Report of 2 cases and review of literature

Ectodermal dysplasias (EDs) are a heterogeneous group of disorders characterized by developmental dystrophies of ectodermal structures. The X-linked recessive ED (Christ-Siemens-Touraine syndrome) is the most common disorder affects mostly males and is inherited through female and they become carriers. It is characterized by the triad of signs of sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth (anodontia or hypodontia) and inability to sweat due to lack of sweat glands (anhidrosis or hypohidrosis).The lack of teeth and the special appearance were reported to be major concerns. Thus an early diagnosis is important. Families suffering from this disorder should therefore be offered genetic counselling. Currently the genes and gene products are defined, hence identification of the genes and taking necessary precautions before starting a family will be beneficial. For the patients as well as the dentists, tooth agenesis and its secondary effects on growth and development of the jaws is often the most significant clinical and therapeutical problem. The course of the treatment is to restore the function and the aesthetics of the teeth, normalise the vertical dimension and support the facial soft tissues. We report two cases of ectodermal dysplasia with review in this article for better understanding of this disorder.