Ectodermal dysplasia syndromes

Definition

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.

6 subordinate codes

• LD27.00Incontinentia pigmenti
• LD27.01Cronkhite-Canada syndrome
• LD27.02Hypohidrotic ectodermal dysplasia
• LD27.03Hidrotic ectodermal dysplasia, Clouston type
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified

Nearby in Syndromes with skin or mucosal anomalies as a major feature

• LD27.1Xeroderma pigmentosum
• LD27.2Syndromic ichthyosis
• LD27.3Genetic syndromes with hypertrichosis
• LD27.4Genetic syndromes affecting nails
• LD27.5Genetic hamartoneoplastic syndromes affecting the skin
• LD27.6Genetic lipodystrophy
• 4B4YOther specified diseases of the immune system
• 4B4ZDiseases of the immune system, unspecified