Genetic review of ectodermal dysplasia

Genodermatoses are congenital skin disorder often grouped into three categories: chromosomal, single gene, and polygenetic. Several of these disorders are isolated and also has oral phenomenon, called oral genodermatoses. Among these Ectodermal dysplasia (EDs) is a large group of an inherited disorders represented by a primary defect in hair, teeth, nails or function of sweat gland, in collaboration to another abnormality in an ectodermal derived tissue e.g. ears, eyes, lips, mucous membranes of an oral cavity or nose, central nervous system. The diverse forms of ectodermal dysplasia are caused by the mutation or deletion of specific genes located on different chromosomes. The signs and symptoms differ markedly among the different forms of the condition and rely on the structures that are affected. Presently there are about 150 different forms of ectodermal dysplasias. The commonest forms are Hypohidrotic (anhidrotic) Ectodermal Dysplasia and Hidrotic Ectodermal Dysplasia. The frequency of the different ectodermal dysplasias is highly variable in a given population. There is no particular treatment, only disease management is accessible.