Paget's disease of the breast

By Dr Arusha Campbell-Chambers

Many persons are not aware that changes in the skin of the nipple and areola (the dark area surrounding the nipple) can be associated with cancer in that breast.

Paget's disease of the breast is a rare form of cancer which involves the nipple, areola and an underlying 'intraduct' or 'invasive' breast cancer. The condition was named after the British doctor, Sir James Paget, who discovered the relationship between skin changes in the nipple and breast cancer in 1874.

This disease mostly affects women, though men can also develop it. About one to four per cent of cases of breast cancer involve Paget's disease of the breast. Individuals have been diagnosed from puberty to the late 80s, however, it is most commonly detected in people in their 50s.

The exact cause of the condition is not fully understood. It is widely believed that cancer cells from a tumour inside the breast travel through the milk ducts to the nipple and areola. It usually affects only one breast.

Resembles eczema

The symptoms and signs of this condition resemble those of conditions like eczema or dermatitis of the breast. These include tingling, itching, burning, redness, flaking, skin thinning, ulceration, flattening or sinking (inversion) of the nipple and a bloody or yellowish nipple discharge. One or more lumps may also be detected in that breast. Associated lumps in the underarm may also be present if the cancer has spread to the lymph glands.

Like an orange peel

Other forms of breast cancer can also cause these changes in the skin. In particular, some forms of breast cancer may cause the skin's texture to resemble an orange peel. It should be emphasised that most cases of rashes of the breast are not associated with cancer but are more commonly caused by benign conditions like eczema.

A biopsy of the nipple is usually done to diagnose Paget's disease of the breast. A mammogram and/or breast ultrasound and/or magnetic resonance imaging (MRI) scan may be requested to look for underlying tumours. Other investigations may also be ordered to help determine how far the cancer has spread in the body.

Mastectomy is done

The standard treatment for Paget's disease of the breast is mastectomy (surgical removal of the breast) with or without the removal of lymph glands in the underarm on that side of the chest. In some cases, where no underlying lump is felt nor seen on the mammogram, the nipple and areola may be removed while leaving the rest of the breast in place, followed by radiation of the whole breast.

Chemotherapy and/or hormonal therapy may also be recommended depending on how far the cancer cells have spread and other factors relating to the types of cancer cells present.

The prognosis for the patient depends on whether or not a tumour is detected, how deep the cancer cells have spread, the types of cancer cells and how far it has spread in the body.

Early detection of the cancer improves the outcome so individuals should not ignore skin changes which may indicate an underlying breast cancer.

Dr Arusha Campbell-Chambers is a dermatologist and founder of Dermatology Solutions Skin Clinics & Medi-Spas; email: yourhealth@gleanerjm.com.