LD2D.1

ICD-11 MMS 2026-01

Neurofibromatoses

Description

The neurofibromatoses (NF) are related genetic disorders which affect bone, soft tissue, skin and nervous system. In NF type 1 neurofibromas develop in the skin and elsewhere: these can cause problems as a result of their visibility in the skin, compression of vital internal structures or from malignant degeneration. Neuromas of the acoustic nerve are the predominant problem in NF type 2.

Exclusions from above levels

  • 2E83.Z – Enchondromatosis
  • 4A01.31 – Ataxia-telangiectasia
  • 8C21.1 – familial dysautonomia [Riley-Day]
Show allHide
  • LA90.00 – Rendu-Osler-Weber disease
  • LD23 – Sturge-Weber syndrome
  • LD26.60 – Angio-osteohypertrophic syndrome
  • LD2C – Proteus syndrome
  • LD2F.1Y – Maffucci syndrome
  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Child codes

  • LD2D.10 – Neurofibromatosis type 1
  • LD2D.11 – Neurofibromatosis type 2
  • LD2D.12 – Neurofibromatosis type 3
  • LD2D.1Y – Other specified neurofibromatoses
  • LD2D.1Z – Neurofibromatosis, unspecified

Sibling codes

  • LD2D.0 – Peutz-Jeghers syndrome
  • LD2D.2 – Tuberous sclerosis
  • LD2D.3 – Gardner syndrome
  • LD2D.4 – Gorlin syndrome
  • LD2D.Y – Other specified phakomatoses or hamartoneoplastic syndromes
  • LD2D.Z – Phakomatoses or hamartoneoplastic syndromes, unspecified

Foundation entity

Neurofibromatoses – http://id.who.int/icd/entity/1697803519

Part of: LD2D: Phakomatoses or hamartoneoplastic syndromes › 20: Developmental anomalies