Medicines for Paget's disease of the nipple

What it is

Paget's disease of the nipple (also called Paget's disease of the breast) is a rare condition that causes changes to the skin of the nipple and the area around it (the areola), and — importantly — is often a sign of an underlying breast cancer. It is a different condition from Paget's disease of bone (which shares the name but is unrelated). In Paget's disease of the nipple, cancer cells are present in the skin of the nipple, and in most cases there is an associated breast cancer within the breast (which may be an early, non-invasive cancer, or an invasive one), though occasionally the changes are confined to the nipple. Because of this association, Paget's disease of the nipple is important to recognise and have checked. The changes it causes affect the nipple and areola, and can include: a red, scaly, crusty, or flaky rash on the nipple and surrounding skin, which can look like eczema or dermatitis; itching, tingling, burning, or soreness of the nipple; the nipple becoming flattened or pulled in (inverted); a discharge from the nipple; and, sometimes, a lump in the breast. A key point is that these changes usually affect one nipple, and are persistent — and, importantly, because the rash can look like eczema, it can be mistaken for a simple skin condition and be treated as such, which can delay the diagnosis. So a persistent, eczema-like rash or change of the nipple — particularly if it affects one nipple, does not clear up, or does not respond to treatment for eczema — should always be checked promptly, and assessed properly (including for breast cancer), rather than assumed to be a simple skin problem. Paget's disease of the nipple is diagnosed by examination and tests (such as a biopsy of the nipple skin, and breast imaging such as a mammogram, to assess for an underlying cancer). Treatment is directed at the condition and any underlying breast cancer, and, as with breast cancer generally, the outlook is better when it is found early — which is why prompt checking of nipple changes matters. The key messages are that Paget's disease of the nipple is a rare skin change of the nipple that can be a sign of an underlying breast cancer, and that a persistent, eczema-like rash or change of the nipple should always be checked promptly.

How it is treated

Paget's disease of the nipple is assessed and treated by specialist breast teams, with the focus on diagnosing it and any underlying breast cancer and treating accordingly; because it can be a sign of breast cancer, prompt assessment of nipple changes is essential. Because the skin changes of Paget's disease of the nipple can look like eczema, and can be mistaken for a simple skin condition, an important message is that a persistent, eczema-like rash or change affecting the nipple — particularly one nipple, that does not clear up or does not respond to treatment for eczema — should be checked promptly and assessed properly, rather than assumed to be a simple skin problem; if a nipple rash treated as eczema does not settle, it should be reassessed. Assessment is by a breast specialist, and follows the principles of breast assessment: examination of the breasts and nipples, breast imaging (such as a mammogram and/or ultrasound) to look for any underlying breast cancer, and a biopsy — including a biopsy of the affected nipple skin (which shows the characteristic cells and confirms Paget's disease of the nipple), and biopsy of any lump or abnormal area found in the breast. This determines whether there is an underlying breast cancer and its type and extent, which guides treatment. Treatment is directed at Paget's disease of the nipple and any underlying breast cancer, and is planned by the specialist breast team according to the findings, following the principles of breast cancer treatment: it usually involves surgery — which may be surgery to remove the affected area (including the nipple and areola and surrounding tissue) or, depending on the extent and any underlying cancer, removal of more of the breast (mastectomy), sometimes with assessment of the lymph nodes — and may also include other treatments used for breast cancer (such as radiotherapy, and, depending on the type and stage of any underlying cancer, hormone therapy, chemotherapy, or targeted treatments), tailored to the individual. As with breast cancer generally, the outlook is better when the condition is found and treated early, which is why prompt checking of nipple changes is so important. Support through and after treatment, and follow-up, are part of care. The key and reassuring messages are that Paget's disease of the nipple, although rare and often associated with an underlying breast cancer, is assessed and treated by specialist breast teams (with surgery and, where relevant, other breast cancer treatments), that outcomes are better with early diagnosis, and — most importantly — that a persistent, eczema-like rash or change of the nipple should always be checked promptly, rather than assumed to be a simple skin condition; so prompt assessment of nipple changes, proper diagnosis, and specialist treatment are the keys.