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Paget's Disease of the Breast
Paget's disease of the breast is a rare form of breast cancer that almost exclusively occurs in women. However, rare cases have been recorded in which men have been affected. The condition was originally reported in 1874 by Sir James Paget, an English surgeon, who also described an unrelated skeletal condition known as Paget's disease of the bone. It is essential to note that these disorders are distinct disease entities that are medically unrelated.
Paget's disease of the breast is characterized by inflammatory, "eczema-like" changes of the nipple that may extend to involve the areola, which is the circular, darkened (pigmented) region of skin surrounding the nipple. Initial findings often include itching (pruritus), scaling, and crusting of and/or discharge from the nipple. In those with Paget's disease of the breast, distinctive tumor cells (known as Paget cells) are present within the outermost layer of skin (epidermis) of the nipple. In addition, the condition is often associated with an underlying malignancy (i.e., cancer) of the milk ducts (ductal carcinoma). The malignancy may be confined to cells lining the milk ducts (carcinoma in situ) or may have invaded surrounding tissue (infiltrating carcinoma). (The milk ducts [lactiferous ducts] are the channels that carry milk secreted by lobes of the breast to the nipple.) Paget's disease of the breast is thought to represent approximately two to four percent of breast cancers.
This is an abstract of a report from the National Organization for Rare Disorders, Inc. ® (NORD). A copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html
The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.
It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.
For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org
Last Updated: 4/1/2008
Copyright 1990, 2000 National Organization for Rare Disorders, Inc.
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