Gorlin syndrome

ICD-11 code : LD2D.4;

Preferred Label : Gorlin syndrome;

ICD-11 definition : Gorlin syndrome, also known as naevoid basal cell carcinoma syndrome (NBCCS), is a hereditary condition characterized by a wide range of developmental abnormalities (odontogenic keratocysts of the jaws, hyperkeratosis of palms and soles, skeletal abnormalities, intracranial ectopic calcifications, and facial dysmorphism) and a predisposition to neoplasms (multiple basal cell carcinomas, medulloblastoma).;

ICD-11 synonym : Naevoid basal cell carcinoma syndrome;

ICD-11 inclusion : Naevoid basal cell carcinoma syndrome;

Details

Origin ID

1012745138;

Automatic exact mappings (from CISMeF team)
- Basal cell nevus syndrome [MedDRA LLT]
- Basal cell nevus syndrome [PASCAL descriptor]
- Basal cell nevus syndrome 1 [OMIM Phenotype]
- Gorlin syndrome [ORDO Disease]
- Gorlin syndrome (disorder) [SNOMED CT concept]
- Nevoid Basal Cell Carcinoma Syndrome [NCIt concept]
- basal cell nevus syndrome [MeSH Descriptor]
- basal cell nevus syndrome [MeSH concept]
- gorlin syndrome [SNOMED Notion]
- nevoid basal cell carcinoma syndrome [DO Concept]
ICD-10 Mapping
- Phakomatoses, not elsewhere classified [ICD-10 category]

Keyword's position in hierarchy(ies) :

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