Paget’s Disease of the Nipple: Symptoms, Diagnosis, and Treatment in Beverly Hills

Paget’s disease of the nipple, also called mammary Paget’s disease, is a rare form of breast cancer that affects the skin of the nipple and often extends to the areola, the darker area of skin surrounding the nipple.

It accounts for approximately 1% to 3% of all breast cancer diagnoses. Although uncommon, it is important to recognize because it is frequently associated with another type of breast cancer within the breast tissue. In many cases, cancer cells from an underlying tumor travel through the milk ducts and reach the surface of the nipple, where they cause visible skin changes. These underlying cancers may include ductal carcinoma in situ (DCIS), a noninvasive form of breast cancer, or invasive breast cancer.

Paget’s disease typically begins on the nipple itself and may extend outward to the areola. Changes affecting only other areas of the breast, without involving the nipple, are less characteristic of this condition.

Paget’s disease can resemble common skin conditions, particularly eczema and dermatitis. Symptoms often develop gradually and typically affect one breast.

Possible signs and symptoms include:

• Redness or discoloration of the nipple
• Flaking, scaling, or crusting of the nipple skin
• Thickened or hardened skin around the nipple
• Persistent itching, burning, or tingling
• Nipple discharge, which may appear yellowish or bloody
• Flattening or inversion of the nipple
• Changes that extend from the nipple to the surrounding areola

Some patients experience itching, burning, or discomfort, while others have no noticeable sensation at all. The presence or absence of these symptoms does not determine whether Paget’s disease is present. Additionally, Paget’s disease does not typically behave like a temporary skin irritation that appears and disappears from one day to the next. Persistent or recurring changes, particularly those involving one nipple, should be evaluated by breast specialist.

No. Many more common, noncancerous conditions can cause changes to the nipple and surrounding skin.

These include:

• Eczema: A common inflammatory skin condition that can cause dryness, redness, itching, and scaling.
• Psoriasis: A condition that causes skin cells to build up, resulting in thickened, scaly patches.
• Contact dermatitis: An inflammatory reaction caused by exposure to irritants or allergens, including soaps, detergents, fabrics, or topical products.
• Fungal infections: Certain infections, including yeast infections, can cause redness, irritation, and scaling of the skin.
• Hyperkeratosis: A buildup or thickening of the outer layer of skin that can affect the nipple.

These conditions are significantly more common than Paget’s disease and can sometimes look very similar. If a nipple rash persists for several weeks, repeatedly returns, or does not respond as expected to treatment, further evaluation may be appropriate. A biopsy can help establish exactly what is causing the skin changes.

Diagnosing Paget’s disease involves evaluating the affected skin and determining whether there are any underlying abnormalities within the breast.

The evaluation may include:

Clinical Breast Examination

A breast specialist will examine the nipple, areola, and surrounding breast tissue for visible changes, thickening, or lumps. They may also ask when the symptoms began, whether they have changed over time, and whether previous treatments have helped.

Breast Imaging

Diagnostic imaging may be recommended to look for an underlying breast abnormality. Depending on the individual findings, this may include:

• Diagnostic mammography
• Breast ultrasound
• Breast MRI

Nipple Skin Biopsy

A skin biopsy is the most definitive way to diagnose Paget’s disease. One commonly used approach is a punch biopsy, in which the affected area is cleaned and numbed with local anesthesia before a small circular instrument is used to remove a tiny sample of skin. The tissue is then examined under a microscope to determine whether cancer cells are present. This is generally a straightforward, well-tolerated procedure performed in an outpatient setting. If imaging identifies a separate abnormality or mass within the breast, that area may also need to be sampled independently to determine whether an underlying breast cancer is present and assess the extent of the disease.

For patients with pure Paget’s disease, meaning stage 0 cancer cells (DCIS) associated with visible changes to the nipple skin but no invasive cancer, the prognosis is generally very favorable, with cure rates comparable to those of DCIS and approaching 99%. Treatment typically involves local management through surgical removal of the affected tissue. Chemotherapy is not generally beneficial in these cases. The extent of surgery depends on where the cancer cells are located and whether any additional disease is present. Options may include removal of the nipple alone, the nipple and surrounding areola, or a mastectomy, depending on the clinical findings and the patient's aesthetic considerations and personal preferences. Radiation therapy or anti-hormone therapy may also be recommended following surgery, depending on the individual diagnosis.

1. Can Paget’s disease affect both nipples?

Paget’s disease almost always affects only one breast. While synchronous (simultaneous) bilateral Paget’s disease affecting both nipples has been reported in rare medical literature, it is extremely rare.

2. Can Paget’s disease be mistaken for eczema?

Yes. Paget’s disease can cause redness, itching, scaling, and crusting that closely resemble eczema or dermatitis. As the conditions can look similar, persistent or recurring changes involving the nipple should be evaluated rather than assumed to be a skin condition. A biopsy can help distinguish between them.

3. Does Paget’s disease require a mastectomy?

Not necessarily. Some patients may be candidates for breast-conserving surgery, which involves removing the affected nipple and areola along with the necessary underlying tissue. Radiation therapy may also be recommended. Mastectomy is another option depending on the extent of disease, associated breast cancer, and individual treatment preferences. The appropriate approach is determined after a complete evaluation.

Changes to the nipple should not be ignored, but they do not automatically indicate cancer. If you notice persistent or unusual changes, a clinical evaluation can help determine the cause and whether further testing is needed.