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Paget's Disease of the Breast

Abstract

You are reading a NORD Rare Disease Report Abstract. NORD’s full collection of reports on over 1200 rare diseases is available to subscribers (click here for details). We are now also offering two full rare disease reports per day to visitors on our Web site.

NORD is very grateful to Vijayakrishna K. Gadi, MD, PhD, Assistant Professor, University of Washington Medical Center; Assistant Member, Fred Hutchinson Cancer Research Center, for assistance in the preparation of this report.

Synonyms of Paget's Disease of the Breast

  • mammary Paget's disease
  • Paget's disease of the nipple
  • Paget's disease of the nipple and areola

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
Paget's disease of the breast is a rare form of breast cancer that almost exclusively occurs in women. However, rare cases have been reported in men. 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 individuals 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, when viewed under a microscope. Most women with this disorder have an underlying cancer (malignancy) affecting the milk ducts (ductal carcinoma). The milk ducts are the channels that carry milk secreted by lobes of the breast to the nipple. The exact cause of Paget's disease of the breast is not fully understood.

Introduction
Paget's disease of the breast 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 entities that are medically unrelated.

Paget's Disease of the Breast Resources

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