Ectodermal dysplasia syndromes

Description

Ectodermal dysplasias (EDs) are a heterogeneous group of syndromes characterised by developmental dystrophies of ectodermal structures, such as hypohidrosis, hypotrichosis, onychodysplasia and hypodontia or anodontia. More than 160 clinically and genetically distinct hereditary ectodermal dysplasia syndromes have been catalogued.

Exclusions from above levels

http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Child codes

LD27.00 – Incontinentia pigmenti
LD27.01 – Cronkhite-Canada syndrome
LD27.02 – Hypohidrotic ectodermal dysplasia
LD27.03 – Hidrotic ectodermal dysplasia, Clouston type
LD27.0Y – Other specified ectodermal dysplasia syndromes
LD27.0Z – Ectodermal dysplasia syndrome, unspecified

Foundation children elsewhere

LD24.80 – Langer-Giedion syndrome
LD25.00 – Oral-facial-digital syndrome

Sibling codes

LD27.1 – Xeroderma pigmentosum
LD27.2 – Syndromic ichthyosis
LD27.3 – Genetic syndromes with hypertrichosis
LD27.4 – Genetic syndromes affecting nails
LD27.5 – Genetic hamartoneoplastic syndromes affecting the skin
LD27.6 – Genetic lipodystrophy
LD27.Y – Other specified syndromes with skin or mucosal anomalies as a major feature
LD27.Z – Syndromes with skin or mucosal anomalies as a major feature, unspecified

Foundation entity

Ectodermal dysplasia syndromes – http://id.who.int/icd/entity/1156567558

Part of: LD27: Syndromes with skin or mucosal anomalies as a major feature › 20: Developmental anomalies