Orphanet: Extramammary Paget disease

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Extramammary Paget disease

Disease definition

A rare skin tumor characterized by predominantly intraepithelial growth of an adenocarcinoma which may either arise primarily in the skin (primary extramammary Paget disease) or result from intraepithelial spread of a visceral carcinoma (secondary extramammary Paget disease). The lesion is typically located in the anogenital region, presenting as a scaly, oozing, pruritic or painful erythematous plaque often resembling eczema. It may exhibit an invasive component with a significant risk of lymph node metastasis.


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: Unknown
  • Inheritance: -
  • Age of onset: Adult, Elderly
  • ICD-10: C44.5
  • ICD-11: 2E64.1
  • OMIM: 167300
  • UMLS: C0030186
  • MeSH: D010145
  • GARD: 4192
  • MedDRA: 10033366  10068223
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