Breast malignant, males, children
In situ carcinoma
Paget disease

Topic Completed: 1 July 2017

Revised: 21 January 2019

Copyright: (c) 2002-2019,, Inc.

PubMed Search: Paget disease [title] breast
Page views in 2018: 22,637
Page views in 2019 to date: 6,441
Cite this page: Sanders MAG Paget disease. website. Accessed March 21st, 2019.
Definition / general
  • In situ carcinoma involving the nipple epidermis
Essential features
  • Clinically presents as a nipple rash
  • Usually diagnosed on skin punch or shave biopsy of the nipple
  • Paget cells originate from DCIS in lactiferous ducts
  • Majority of cases are associated with DCIS or invasive carcinoma deeper in the breast
  • "Secondary" Paget disease occurs when invasive carcinoma arising deep in the breast directly invades the skin; the invasive carcinoma is usually large in size
  • Paget disease with dermal invasion occurs when Paget cells cross the basement membrane of the epidermis; the invasive component is usually small in size (Arch Pathol Lab Med 2013;137:72)
  • Present in 1 - 4% of all patients with breast carcinoma
  • Affects men and women; age range is 27 to 88 (mean 54 - 63 years)
Case reports
  • Nipple areolar complex is removed by either mastectomy or central lumpectomy with or without sentinel lymph node biopsy
  • Treatment, including surgical and adjuvant therapy, as well as prognosis, depend on underlying DCIS or invasive carcinoma
Clinical images

Contributed by Mark R. Wick, M.D.

Breast skin

Images hosted on other servers:

Eczematoid lesion

Gross description
  • Nipple skin with scaling crust, erythema and exudate resembling chronic eczema
  • May have ulceration
Gross images

Contributed by Dr. Emily Reisenbichler

Paget disease of the nipple

Microscopic (histologic) description
  • Single cells or clusters of cells spread throughout the epidermis
  • Cells have abundant pale cytoplasm, irregular large nucleus with prominent nucleoli
  • Underlying dermis with chronic inflammation
  • Epidermis with hyperkeratosis and possibly ulceration
  • Florid cases can show gland formation
Microscopic (histologic) images

Scroll to see all images:

Contributed by Emily Reisenbichler, M.D.

Paget disease of the nipple, low and high power

Contributed by Mary Ann Gimenez Sanders, M.D, Ph.D.

Secondary Paget disease

Lactiferous duct of the nipple with DCIS

Epidermal hyperkeratosis

Epidermal ulcer

Florid Paget disease

Paget cells with pale cytoplasm

Paget disease with dermal invasion

HER2 positive Paget disease

AFIP images

Large atypical cells in
epidermis have round
nuclei and prominent

Involvement of terminal portion of lactiferous duct


Involvement of entire thickness
of epidermis, with pseudoepitheliomatous
hyperplasia and hyperkeratosis with involved
area but not in normal appearing area

Contributed by Dr. Semir Vranic - Paget with underlying DCIS





Underlying DCIS (left to right): H&E, ER alpha, CK8, HER2


Contributed by Mark R. Wick, M.D.

Breast skin





Images hosted on other servers:

Large atypical cells in epidermis

With hyperkeratosis and dermal inflammation

With underlying comedo DCIS

Derived from LCIS

CK7+, E-cadherin negative


Positive stains
Negative stains

Differential diagnosis
Sample report
  • Diagnosis: ductal carcinoma in situ involving the nipple epidermis as Paget disease
Board review question #1
Which immunohistochemical stain is most likely to be positive in Paget disease?

  1. CK5 / 6
  2. CK20
  3. ER
  4. HER2
  5. PR
Board review answer #1
D. HER2: the majority of Paget disease cases are HER2 positive and therefore HER2 can be used to confirm the diagnosis. In the absence of invasive carcinoma, HER2 positivity does not influence treatment since Paget disease is an in situ carcinoma. Paget disease is negative for CK5 / 6 and CK20. ER and PR show variable expression.
Back to top