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 characterised 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 develop malignant neoplasms (such as multiple basal cell carcinomas
or medulloblastoma), and benign neoplasms in the jaw, heart, or ovaries.;
ICD-11 synonym : Naevoid basal cell carcinoma syndrome;
ICD-11 inclusion : Naevoid basal cell carcinoma syndrome;
Origin ID : 1012745138;
Automatic exact mappings (from CISMeF team)
- ICD-10 Mapping
Gorlin syndrome, also known as naevoid basal cell carcinoma syndrome (NBCCS), is a
hereditary condition characterised 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 develop malignant neoplasms (such as multiple basal cell carcinomas
or medulloblastoma), and benign neoplasms in the jaw, heart, or ovaries.
