2E64.1

ICD-11 MMS 2026-01

Extramammary Paget disease of skin

Description

An intraepithelial adenocarcinoma of apocrine gland-bearing skin and mucous membrane. Clinically it presents as sharply demarcated erythematous plaques most commonly affecting the vulva in women and perianal skin in men.

Index terms

  • Extramammary Paget disease of skin
  • EMPD - [extramammary Paget disease]
  • Extramammary Paget disease of penis
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  • Extramammary Paget disease of anus
  • Extramammary Paget disease of anogenital skin

Exclusions from above levels

  • 2E63 – Melanoma in situ neoplasms

Foundation children elsewhere

Sibling codes

  • 2E64.0 – Intraepidermal squamous cell carcinoma
  • 2E64.2 – Carcinoma in situ of anal margin or perianal skin
  • 2E64.Y – Other specified carcinoma in situ of skin
  • 2E64.Z – Carcinoma in situ of skin, unspecified

Foundation entity

Extramammary Paget disease of skin – http://id.who.int/icd/entity/1796624917

Allowed postcoordination axes

Part of: 2E64: Carcinoma in situ of skin › 02: Neoplasms