Paget Disease of the Breast
- 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
- Transformation theory (not favored)
- 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
- 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).
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Domino, Frank J., et al., editors. "Paget Disease of the Breast." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2020. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast.
Paget Disease of the Breast. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2020. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast. Accessed June 3, 2023.
Paget Disease of the Breast. (2020). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (27th ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/116430/all/Paget_Disease_of_the_Breast
Paget Disease of the Breast [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2020. [cited 2023 June 03]. 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 - Domino,Frank J, ED - Baldor,Robert A, ED - Golding,Jeremy, ED - Stephens,Mark B, 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 -