Paget’s Disease

Paget’s disease of the breast is a rare form of breast cancer named after 19th century British doctor Sir James Paget. While Paget’s disease can apply to other parts of the human body, Paget’s disease of the breast is usually clinically observed with a scaling of the nipple skin, nipple redness or nipple burning.

Paget’s disease of the breast is present in 1-4 percent of breast cancer cases. The median age of diagnosis is 57. However, cases of Paget’s disease of the breast have been found in patients as young as their young 20s to as old as their late 80s.

It is important to note most cases of nipple skin scaling and/or burning are due to benign conditions. If an individual feels these symptoms it is important to contact a breast-dedicated physician to ensure proper diagnosis is provided.

Making a Paget’s Disease Diagnosis

The diagnosis of Paget’s disease is actually quite easy. Breastlink breast-dedicated physicians use a simple skin punch biopsy technique to remove a small piece of tissue from the area of the nipple affected by the abnormal changes. This procedure is painless and takes a few minutes to perform under local anesthesia. A small amount of skin and underlying tissues is removed and sent to a pathologist. In our experience, with hundreds of cases, it has proved to be highly accurate.

The first step to evaluate a patient with a change in the skin of the nipple is to obtain a detailed history and perform a careful breast examination, followed by a diagnostic mammogram. If Paget’s disease is diagnosed, the next step is to identify the location of the underlying breast cancer.

More than 50 percent of patients with Paget’s disease will have an abnormality on their mammogram. When performing a diagnostic mammogram, the mammogram technologist is informed about the possibility of a Paget’s disease diagnosis. Special films look for fine calcifications behind the nipple.

If the mammogram is negative, and our clinical suspicion is low, we usually suggest the patient use a cortisone cream on the nipple for two weeks. If the symptoms persist, or there is a clinical concern, additional imaging techniques such as a breast ultrasound and/or MRI can be ordered. Careful follow-up is always needed with a potential diagnosis of Paget’s disease.

Our Approach to Benign Conditions of the Nipple

There are a variety of conditions that can mimic Paget’s disease such as atopic dermatitis, nipple eczema, psoriasis, nipple adenoma or contact dermatitis. Our medical team will answer your questions about these topics in during an office visit.

In the collective experience of our breast-dedicated medical team, most patient referrals with a suspicion of a Paget’s disease diagnosis have similar changes in both nipples. Paget’s disease tends to affect only one side. Patients demonstrating bilateral nipple change are usually referred to a dermatologist who will evaluate the underlying cause of the nipple change if the mammogram is normal.

The majority of patients who have a skin punch biopsy end up with a benign result. These patients simply require reassurance there is no cancer present at the time of the office visit. Follow-up observation with the referring doctor is indicated.

Sometimes a moisturizing cream is helpful to control nipple dryness. Patients with persistent symptoms following a negative biopsy are also referred to a dermatologist for treatment.

Treatment of Paget’s Disease

The traditional, standard treatment for Paget’s disease of the breast was a mastectomy. Advancements in surgical technique and equipment have allowed modern medical practice to prefer a breast saving procedure. The affected nipple and surrounding breast tissue containing breast cancer will be removed by a Breastlink surgeon. Once we secure clear surgical margins, we irradiate the affected breast tissue.

Our results with this approach have been excellent. In rare cases, more extensive involvement of the breast tissue may be require mastectomy which is usually followed by immediate reconstruction of the breast (Reconstructive Surgery).

Pictures of patients with Paget’s disease

Pictures of patients with benign conditions of the nipple.