BD93.0

ICD-11 MMS 2026-01

Primary lymphoedema

Description

Lymphoedema as a result of lymphatic vessel hypoplasia

Index terms

  • Primary lymphoedema
  • Congenital lymphoedema
  • Simple primary lymphoedema
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  • Congenital primary lymphoedema
  • Hereditary lymphoedema type I
  • Milroy disease
  • Peripubertal primary lymphoedema
  • Hereditary lymphoedema type II
  • Lymphoedema praecox
  • Meige disease
  • Sporadic late-onset primary lymphoedema
  • Complex primary lymphoedema
  • Lymphoedema with atrial septal defects and facial changes
  • Lymphoedema-distichiasis syndrome
  • LDS - [lymphoedema distichiasis syndrome]
  • Hennekam lymphangiectasia-lymphoedema syndrome
  • Hennekam syndrome
  • Lymphoedema, microcephaly and chorioretinopathy syndrome
  • Deafness, lymphoedema and leukaemia syndrome
  • Emberger syndrome

Exclusions from above levels

  • MA01 – Enlarged lymph nodes
  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations and chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes

Foundation children elsewhere

Sibling codes

  • BD93.1 – Secondary lymphoedema
  • BD93.Y – Other specified forms of lymphoedema
  • BD93.Z – Lymphoedema, unspecified

Foundation entity

Primary lymphoedema – http://id.who.int/icd/entity/794588197

Allowed postcoordination axes

Part of: BD93: Lymphoedema › 11: Diseases of the circulatory system