Extramammary Paget's disease

Last Updated: 2023-07-07

Author(s): Anzengruber F., Navarini A.

ICD11: 2E64.1

Paget carcinoma, adenocarcinoma apocrinocellulare epidermotropicum.

Special form of Paget's disease in which regions with apocrine sweat glands are affected rather than the nipple. Alternatively, the prostate, cervix, bladder and rectum can also be affected.

  • Women : men = 3-4 : 1 (Europe)
  • Men > women (Japan)
  • Average age: 70 y.

As with Paget's disease of the nipple, extramammary Paget's disease is an adenocarcinoma, but it is not due to a breast carcinoma.

Sharply demarcated, irregular, erythematous, possibly pruritic or dull plaque, partly accentuated at the edges, with tongue-shaped extensions.

  • Clinical picture
  • Biopsy
    • In the centre of the lesion

Adnexal adenocarcinoma (25% of cases)

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