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Paget Disease of the Breast

Paget Disease of the Breast is a topic covered in the 5-Minute Clinical Consult.

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  • Rare disease of the nipple-areola complex (NAC) typically associated with underlying in situ or invasive carcinoma
  • Characterized by eczematous changes of the nipple, erythema, ulceration, crusting, bleeding, and/or itching
  • Divided into three categories (1)
    • Paget disease of the nipple without ductal carcinoma in situ (DCIS)
    • Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the NAC
    • Paget disease of the nipple with associated DCIS in the underlying lactiferous ducts of the NAC and associated DCIS or invasive breast cancer elsewhere in the breast at least 2 cm from the NAC
  • System(s) affected: skin/exocrine


  • 1–3% of breast cancers in females (2)
  • 0.4% of invasive female breast cancer (3)
  • Incidence of Paget disease of the breast has been decreasing since 1988, despite an increased incidence of breast cancer (4,5).
  • Median age at diagnosis = 64 years (5)
  • Extremely uncommon in males but prognosis is worse in men (6)

<1% of population

Etiology and Pathophysiology

  • Cause is unknown, but risk factors for Paget disease are similar to those for developing breast cancer in general (see below).
  • Epidermotropic theory
    • Ductal carcinoma cells migrate from underlying mammary ducts to epidermis of the nipple to become Paget cells (4,6,7).
  • Transformation theory (not favored)
    • Epidermal cells of nipple/areola transform into Paget cells that can invade the basement membrane into the dermis (4,6,7).

No known genetic pattern, although studies suggest up to 88% display HER2/neu overexpression (2)[B]

Risk Factors

  • Same risk factors apply as for noninherited breast cancers.
  • Female gender
  • Age >40 years
  • Previous breast cancer
  • Benign breast disease (atypical ductal/lobular hyperplasia, fibroadenoma, sclerosing adenosis, intraductal papilloma)
  • First-degree relative with history of breast cancer
  • Caucasian
  • Menarche <12 years of age
  • Menopause >50 years of age
  • Nulliparity or first child after age 34 years
  • History of ionizing radiation exposure
  • History of alcohol abuse
  • Hormone replacement
  • Excess weight gain

Commonly Associated Conditions

  • Largest study, using surveillance, epidemiology, and end results (SEER) data representing 1,763 women with confirmed Paget disease, reports an underlying in situ or invasive breast cancer in 87% of patients, although there is often no associated breast mass or mammographic abnormality (5).
  • The underlying carcinomas are multifocal/multicentric in 32–41% of patients (2)[B].

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Stephens, Mark B., et al., editors. "Paget Disease of the Breast." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast.
Paget Disease of the Breast. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast. Accessed March 22, 2019.
Paget Disease of the Breast. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast
Paget Disease of the Breast [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 March 22]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Paget Disease of the Breast ID - 116430 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -