Genetic hamartoneoplastic syndromes affecting the skin

Description

A heterogeneous group of inherited diseases characterised by the presence of multiple hamartomata and associated with an increased risk of malignancy.

Index terms

Genetic hamartoneoplastic syndromes affecting the skin
Legius syndrome
NF1-like syndrome

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NFLS - [Neurofibromatosis 1-like syndrome] (MIM 611431)
Neurofibromatosis 1-like syndrome
Bazex-Dupré-Christol syndrome
Follicular atrophoderma and basal cell carcinoma syndrome [Bazex]
Birt-Hogg-Dubé syndrome
Fibrofolliculomas with trichodiscomas and acrochordons

Exclusions from above levels

http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Foundation children elsewhere

LD2D.1 – Neurofibromatoses
LD2D.2 – Tuberous sclerosis
LD2D.3 – Gardner syndrome
LD2D.4 – Gorlin syndrome

Sibling codes

LD27.0 – Ectodermal dysplasia syndromes
LD27.1 – Xeroderma pigmentosum
LD27.2 – Syndromic ichthyosis
LD27.3 – Genetic syndromes with hypertrichosis
LD27.4 – Genetic syndromes affecting nails
LD27.6 – Genetic lipodystrophy
LD27.Y – Other specified syndromes with skin or mucosal anomalies as a major feature
LD27.Z – Syndromes with skin or mucosal anomalies as a major feature, unspecified

Foundation entity

Genetic hamartoneoplastic syndromes affecting the skin – http://id.who.int/icd/entity/2064431086

Part of: LD27: Syndromes with skin or mucosal anomalies as a major feature › 20: Developmental anomalies