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LD27.0 Ectodermal dysplasia syndromes

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

code elsewhere

• 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)

sections/codes in this section (LD27.0-LD27.0)

• Incontinentia pigmenti  (LD27.00)
• Cronkhite-Canada syndrome  (LD27.01)
• Hypohidrotic ectodermal dysplasia  (LD27.02)
• Hidrotic ectodermal dysplasia, Clouston type  (LD27.03)
• Other specified ectodermal dysplasia syndromes  (LD27.0Y)

synonyms

• Ectodermal dysplasia syndromes

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