Definition of Paget’s Disease of The Breast

A rare presentation of breast cancer, also called Paget’s disease of the nipple. Researchers believe Paget’s disease of the breast occurs when disordered cells from a cancer within the breast migrate to the skin surface, most likely through the milk ducts, to infiltrate the tissues of the outer breast and the nipple.

Paget’s disease of the breast is most commonly associated with an underlying invasive breast cancer or ductal cancer in situ (DCIS).

Signs and Symptoms

The symptoms of Paget’s disease of the breast may develop over months to years, and typically begin with a scaly rash that may itch or burn. The skin of the nipple and the areola (the area around the nipple) may crack and bleed.

Because skin conditions such as atopic dermatitis (also called eczema) and psoriasis commonly affect the breasts, early symptoms are often misdiagnosed as dermatologic. One subtle difference is that Paget’s disease of the breast begins in the nipple and spreads to the areola, whereas dermatologic conditions begin in the areola and extend to the nipple.

As Paget’s disease of the breast advances, the nipple may invert or there may be bloody discharge from the nipple.

Diagnosis and Treatment

The diagnostic path typically includes mammogram (x-ray of the breast) and biopsy of the cells of the nipple and underlying breast tissue. Ultrasound of the breast may reveal tumors within the breast.

Treatment begins with surgery to remove the cancer, which may be breast-conserving surgery when the cancer remains fairly localized and simple or radical mastectomy when the cancer is widespread within the breast.

Radiation therapy, hormone therapy (such as with tamoxifen) when the underlying cancer is hormone sensitive, and chemotherapy are common adjuvant (follow-up) treatments.


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