Introduction :
Paget's disease of the breast is a rare form of breast cancer in which the first clinical finding is usually a scaling of the nipple skin. It may also be associated with nipple redness and burning. The diagnosis of this condition is typically made with a skin biopsy, which shows cells typical of this condition (Paget's cells). The "good news" about Paget's disease is that it is usually a "curable cancer". However, most cases of scaling and burning of the nipple are due to benign conditions, which can be easily confused with Paget's disease.

Making the Diagnosis :
The major goal of this section is to help women (and their physicians) detect the early nipple changes associated with Paget's disease, and to help differentiate them from benign conditions. Making the diagnosis of Paget's disease is actually quite easy. We 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 takes a few minutes to perform under local anesthesia, and is painless. A small amount of skin and underlying tissues is removed and sent to the pathologist. In our experience with hundreds of cases, it has proved to be 100% accurate.

The first step in evaluating any patient with a change in the skin of the nipple is to obtain a careful history and perform a careful breast examination, followed by a diagnostic mammogram. When performing a diagnostic mammogram, the mammographer is informed about the possibility of Paget's disease. Special films look for fine calcifications behind the nipple. If the mammogram is negative and our clinical suspicion is low, we usually suggest that the patient place a cortisone cream on the nipple for two weeks.

If the condition has not responded completely to conservative treatment in two weeks, we proceed to a skin punch biopsy (A word of caution: We have seen cases of Paget's disease in which the mammogram was negative. The patient seemed to respond to treatment, but nipple changes recurred again in a few months. Therefore, if a biopsy is not done, careful follow-up is recommended). Our rule of thumb: if in doubt, do a skin biopsy.

Our Approach to benign conditions of the nipple :
There are a variety of conditions that can mimic Paget's disease, such as atopic dermatitis or nipple eczema, or contact dermatitis. A detailed description of these conditions is beyond the scope of this discussion.

In our experience, most of the women referred to us with a possible Paget's disease have similar changes in both nipples. However, we have never seen (or heard) of a case of Paget's disease in both breasts at the same time. Most such patients are referred to a dermatologist to evaluate the underlying cause of the nipple change. However, we would still insist that these patients have a baseline mammogram. If there are any reasons for concern about the possibility of Paget's disease diagnosis, we would not hesitate to do a skin punch biopsy.

Most of our patients who do have a skin punch biopsy end up with a benign result. These patients simply require reassurance that there is no cancer present, and follow-up observation is all that is indicated. Sometimes a moisturizing cream is helpful to control any nipple dryness. Patients with persistent symptoms following a negative biopsy are also referred to a dermatologist for treatment.

Treatment of Paget's disease :
In the past, the standard treatment for Paget's disease was a mastectomy. Currently, our treatment of choice is to save the breast. We remove the nipple (and any surrounding breast tissue) that contains breast cancer. If we are able to get clear surgical margins, we then irradiate the affected breast. Our results with this approach have been excellent. In some cases, there will be more extensive involvement of the breast tissue. In these cases, we recommend a mastectomy, usually followed by immediate reconstruction.

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