ICD-11 definition : Hypohidrotic ectodermal dysplasia is a genetic disorder of ectoderm development characterized
by malformation of ectodermal structures such as skin, hair, teeth and sweat glands.
It comprises three clinically almost indistinguishable subtypes with impaired sweating
as the key symptom: Christ-Siemens-Touraine syndrome (X-linked), autosomal recessive
and autosomal dominant hypohidrotic ectodermal dysplasia, as well as a fourth rare
subtype with immunodeficiency as the key symptom.;
Hypohidrotic ectodermal dysplasia is a genetic disorder of ectoderm development characterized
by malformation of ectodermal structures such as skin, hair, teeth and sweat glands.
It comprises three clinically almost indistinguishable subtypes with impaired sweating
as the key symptom: Christ-Siemens-Touraine syndrome (X-linked), autosomal recessive
and autosomal dominant hypohidrotic ectodermal dysplasia, as well as a fourth rare
subtype with immunodeficiency as the key symptom.