Anus & perianal area


Paget disease

Last author update: 8 March 2021
Last staff update: 21 April 2021

Copyright: 2002-2022,, Inc.

PubMed Search: Paget disease anus "free full text"[sb]

Raul S. Gonzalez, M.D.
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Cite this page: Gonzalez RS. Paget disease. website. Accessed December 2nd, 2022.
Definition / general
  • Intraepithelial tumor of apocrine or possibly eccrine gland origin
Essential features
  • Intraepidermal neoplastic cells, sometimes linked to anal malignancy
  • More common in older patients
  • Neoplastic cells are positive for CK7 and mucicarmine
  • Primary extramammary anal Paget disease is rare
  • Men and women are equally affected
  • Most often occurs in the fifth to seventh decade
  • Typically indolent but often recurs
  • About 15% of patients with anal Paget have secondary disease (associated with an underlying malignancy)
  • Arises anywhere between the dentate line and the perianal skin
Clinical images

Images hosted on other servers:
Extensive perianal Paget disease, perineum

Extensive perianal Paget disease, perineum

Early response

Early response

Notable telangiectasia and fibrosis

Notable telangiectasia and fibrosis

Gross description
  • Erythematous, ulcerated or eczematous plaques or patches
Microscopic (histologic) description
  • Large, pale staining to clear intraepidermal neoplastic cells containing abundant mucin, arranged singly or occasionally in nests or gland-like formations
  • Cells are more numerous basally
  • Microscopic disease may extend beyond grossly visible changes
  • Associated changes include squamous hyperplasia, fibroepithelioma-like hyperplasia and papillomatous hyperplasia (Am J Surg Pathol 2000;24:543)
Microscopic (histologic) images

Contributed by Raul S. Gonzalez, M.D.
Paget disease Paget disease

Paget disease

Images hosted on other servers:

Secondary anal Paget disease
(first image) with associated

Positive stains
Negative stains
Sample pathology report
  • Anus, biopsy:
    • Paget disease (see comment)
    • Comment: The cells are positive for CK7 by immunohistochemistry, confirming the diagnosis. Anal Paget disease sometimes arises in the setting of an invasive anal malignancy. Further clinical workup may be indicated.
Differential diagnosis
Board review style question #1

Which of the following is true about this anal lesion?

  1. It always occurs alongside adenocarcinoma
  2. It arises secondary to treatment for malignancy
  3. It is positive for CK7 and mucicarmine
  4. It only occurs in men
Board review style answer #1
C. Anal Paget disease is positive for CK7 and mucicarmine

Comment Here

Reference: Anal Paget disease
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