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
ICD-11 LD27.0 — Ectodermal dysplasia syndromes
Ectodermal dysplasias (EDs) are a heterogeneous group of disorders 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 dysplasias have been catalogued.
The diagnosis includes nothing.
The diagnosis excludes nothing.
Diagnosis with code LD27.0 contains 7 clarifying diagnoses:
- LD27.00 — Incontinentia pigmenti
- LD27.01 — Cronkhite-Canada syndrome
- LD27.02 — Hypohidrotic ectodermal dysplasia
- LD27.03 — Hidrotic ectodermal dysplasia, Clouston type
- LD24.80 — Langer-Giedion syndrome
- LD25.00 — Oral-facial-digital syndrome
- LD27.0Y — Other specified ectodermal dysplasia syndromes
The diagnosis is coded elsewhere:
- Syndromes with skin or mucosal anomalies as a major feature #23324
- Ectodermal dysplasia syndromes #23325
The diagnosis is included in 11 other classes.
- Langer-Giedion syndrome (LD24.80)
- Oral-facial-digital syndrome (LD25.00)
- Solitary median maxillary central incisor syndrome (LA30.Y)
- Rothmund-Thomson syndrome (LD2B)
- Hallermann-Streiff-François syndrome (LD2B)
- Keratitis – ichthyosis – deafness syndrome (LD27.2)
- Papillon-Lefèvre syndrome (EC20.30)
- Cataract – hypertrichosis – intellectual deficit (LD27.3)
- Hypomelanosis of Ito (EC23.2Y)
- Ectodermal dysplasia – skin fragility syndrome (EC30)
- Dyskeratosis congenita (3A70.0)