The rash or other changes in the nipple can indicate a cancer in the breast ducts, many times located under the nipple, which has then extended itself onto the surface of the nipple.
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Sometimes this sign of breast cancer indicates a small ductal carcinoma in situ (DCIS), which is a very early breast cancer that has not yet left the duct. Other times, Paget's disease of the nipple may indicate an invasive cancer somewhere else in the breast. In some cases, a woman who shows signs of Paget's disease of the nipple will additionally have an abnormal mammogram or have lump in her breast.
Before we move on I want to clarify that Paget's disease of the nipple is not the same as Paget's disease of the bone, which is a severe bone disease. Sir James Paget, a British surgeon and physiologist, discovered both conditions which were first documented by him, but they are completely unrelated diseases. Paget's disease of the nipple can also affect men, although it is rare.
Paget's disease of the nipple is often first noticed when physical signs of the disease appear. Signs of Paget's disease usually only occur on one nipple and can include persistent crustiness, scaliness, or redness of the nipple, itching or burning of the nipple and surrounding areola and bleeding or oozing from the nipple and areola.
Paget's disease can often be confused with other skin conditions, such as breast eczema. Breast Eczema is a highly treatable condition which can be characterized by red, itchy patches or weeping blisters around the nipple which reoccur, but clear up with proper treatment. Paget's disease does not clear up with routine treatment for eczema or infection and usually only affects one nipple.
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A mammogram is the next step to check for cancer in the actual breast. Sometimes any underlying abnormal breast mass will not be present on a mammogram. A clean mammogram combined with an abnormal nipple finding requires further investigation.
A biopsy of the nipple tissue will need to be performed. Usually this consists of a "punch biopsy" that removes a small amount of tissue to check for cancer. If the mammogram indicates other areas of concern within the breast, biopsies of those areas should be performed.
If Paget's disease is caught early while it is still confined to the nipple and underlying breast ducts, the patient typically has an excellent prognosis. However, if Paget's disease of the nipple is associated with an invasive breast cancer or if the cancer has spread out of the breast to other areas of the body (metastatic disease), the survival rate can be lower.
Treatment of Paget's disease of the nipple involves surgery, radiation treatment and Chemotherapy or drug therapy (such as tamoxifen). Like other types of breast cancer, the location of the cancer will determine which type of surgery is done - a lumpectomy or mastectomy. Radiation therapy usually follows a lumpectomy.
A recent development in surgical treatment involves removing only the nipple and areola (sometimes followed by radiation therapy) in patients whose Paget's disease has no other underlying breast cancer, thus allowing the woman to keep her breast. Following treatment, an artificial nipple can be recreated using skin grafts and tattooing.
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