ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
Genetic syndromes affecting the skin
LD27.0 — Ectodermal dysplasia syndromes
LD27.02 — Hypohidrotic ectodermal dysplasia
ICD-11 LD27.02 — Hypohidrotic ectodermal dysplasia
Hypohidrotic ectodermal dysplasia is a genetic disorder of ectoderm development characterised 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.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: