Genodermatosis characterized by the involvement of 2 of the following ectodermal derivates : teeth, nail, sweat glands and hair

Ectodermal dysplasias (ED) are a large group of diseases characterized by the involvement of at least two of the four ectodermal following derivates: nails, teeth, hair and sweat glands. More than 200 diseases belong to this group of diseases. Ectodermal dysplasias are divided in two groups: Pure ED involving the ectodermal derivates only and syndromic ED in which other organs might be involved such as breast (nipples and ductal growth and branching anomalies), clefts and ectrodactyly…. Syndromic ED are, in turn, divided in two groups : ED with PPK and ED with other anomalies. Anhidrotic/hypohidrotic ED remain the more frequent forms. Investigations and diagnostic evaulation are necessary for the phenotypic characterization of ED patient. Each tissue survey is related to early identification of damage due to pathology that if early detected can be treated

Incidence : 1/50 000

Prevalence : 1-9/100 000

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