ICD-11 classes
14 Diseases of the skin
Skin disorders involving specific cutaneous structures
Disorders of the epidermis and epidermal appendages
Disorders of eccrine sweat glands or sweating
EE01 — Hypohidrosis
EE01.1 — Hypohidrosis due to genetic abnormalities of eccrine gland...
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:

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