Paget’s disease of the nipple – what lies beneath

Dr Lucienne Van Schalkwyk educates us on the signs and symptoms of Paget’s disease of the nipple and the treatment thereof.

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Paget’s disease of the nipple is a fairly uncommon way for breast cancer to present. The condition is named after Sir James Paget, who first described what appeared to be eczema of the nipple associated with breast cancer in 1874.

Paget’s disease is caused by breast cancer cells growing into the outer layer of the skin of the nipple, known as the epidermis. The most common theory for the origin of Paget’s disease is that these cancer cells originate within the branch-like milk ducts in the breast, grow along the ducts towards the nipple, and finally out into the skin of the nipple itself.

Signs and symptoms

Initially, Paget’s disease can be quite subtle. The nipple may itch or burn and a change in colour or small area of peeling skin may become visible in the centre of the nipple. A discharge may be present, which may dry on the nipple, forming a crust or scab.

As the disease progresses, it resembles eczema; it may be red and weepy (wet), or a dry, crusty rash. If left untreated, the area will become bigger, spreading beyond the nipple onto the surrounding areola, and even the normal skin of the breast. In advanced cases, Paget’s disease can ulcerate, leaving an open wound on the breast.

What lies beneath

The cancerous cells may be isolated to the nipple itself or it might be a bit like the tip of the iceberg, where what we see is only a small portion of what is actually going on. We shouldn’t be so focused on what we can see on the skin, that we forget about what might be hidden inside the breast.

Paget’s may be accompanied by ductal carcinoma in situ (DCIS), a pre-invasive form of breast cancer, or an invasive breast cancer deeper within the breast.

As healthcare providers, we examine the breast carefully for any signs of disease beyond what is seen on the nipple, any lumps in the breast or any enlarged lymph nodes under the arm.

Thorough investigation

It’s very important to obtain a mammogram and ultrasound of the breasts, and some breast physicians supplement this with an MRI scan of the breast, which can sometimes identify abnormalities in the breast that can’t be seen on mammogram.

If anything concerning is found, it can be investigated further by a core needle biopsy. A small piece of the skin of the nipple should be removed as a biopsy to confirm the presence of Paget’s disease, which can be done under local anaesthesia.

Treatment

In the past, Paget’s disease was managed by removing the entire breast (mastectomy). Mastectomy may still be the best option where Paget’s disease of the nipple is present along with extensive cancer in the breast. Thanks to research, we know now that Paget’s disease that is only present in the area of the nipple can also be treated successfully with a lumpectomy, followed by radiation.

A lumpectomy for Paget’s disease includes removal of the nipple and the areola, which is an area that is cosmetically important to the appearance of the breast. It’s crucial to involve a reconstructive surgeon in the surgery, so that a good cosmetic outcome can be achieved. The nipple that has been removed can be reconstructed and tattooed at a later stage. If DCIS or an invasive cancer is present along with Paget’s disease, further treatment by an oncologist (such as chemotherapy) may be necessary, depending on the characteristics of the tumour.

It can be quite difficult to distinguish between Paget’s disease and other conditions,such as forms of eczema/dermatitis (including contact dermatitis), certain types of skin cancer (squamous cell carcinoma or melanoma) and non-cancerous conditions such as nipple adenosis.

Unfortunately, many patients with Paget’s disease, assuming that the rash is only eczema, treat the condition at home and delay seeking medical care until it’s quite advanced. If you’re in any way concerned about a change in your nipple, visit your healthcare team early on for further examination.

We may have to biopsy the nipple to obtain answers but if the biopsy confirms Paget’s disease, appropriate treatment can be started at an early stage; and if the biopsy rules out Paget’s disease, you have obtained peace of mind.

MEET THE EXPERT – Dr Lucienne van Schalkwyk

Dr Lucienne van Schalkwyk is a general surgeon with a special interest in breast surgery. Her practice manages the entire spectrum of benign and malignant breast disease, and her specific interests include breast-conserving surgery and oncoplastic surgical techniques.