Paget’s Disease of the Breast: Causes, Symptoms, Diagnosis and Treatment

Paget's Disease of the Breast: Causes, Symptoms, Diagnosis and Treatment
Paget's Disease of the Breast: Causes, Symptoms, Diagnosis and Treatment

Paget’s disease (also known as Paget’s disease of the nipple and mammary Paget disease) is a rare form of breast cancer that affects the nipple and the areola area. This relatively rare disease affects women over the age of 62 and men over the age of 69.

The condition accounts for 1 to 4.3% of all breast cancers and was first described by Sir James Paget, an English surgeon in 1874. Jacobaeus (1904) was the most significant research of Paget’s disease of the breast, in which the author has identified the disease as an intraductal cancer. (It is important to mention that several other diseases are named after Sir James Paget, including Paget’s disease of bone and extramammary Paget’s disease, which includes Paget’s disease of the vulva and Paget’s disease of the penis. These diseases are not the same as Paget’s disease of the breast.) In this article only Paget’s disease of the breast is discussed.

What Causes Paget’s Disease?

Today, there are two theories of occurrence of Paget’s disease.

  1. Experts suppose that about 95% of such abnormalities that found out in patients after diagnosing with cancer, which is localized in the “milk” organ. Atypical cells travel through the milk ducts to the surface of the breast, and abnormal formations develop there. Most often this disease is associated with intraductal carcinoma or ductal carcinoma in situ.
  2. Another theory is believed that healthy skin cells regenerate in atypical cells spontaneously. This process refers to injury of nipples, the presence of cancer diseases of other organs, scleroderma, pemphigus.

What are the Symptoms of Paget’s Disease of the Breast?

The symptoms of Paget disease of the breast are often mistaken for those of some benign skin conditions, such as dermatitis or eczema. This disease is characterized by certain signs. For example:

  • Symptoms of Paget’s Disease of the BreastFlaky or scaly skin on the nipple
  • Crusty, oozing or hardened skin resembling eczema on the nipple, areola or both
  • Itching
  • Redness
  • A tingling or burning sensation
  • Straw-colored or bloody nipple discharge
  • A flattened or turned-in (inverted) nipple
  • A lump in the breast
  • Thickening skin on the breast

Signs and symptoms usually occur in one breast only. The disease typically starts in the nipple and may spread to the areola and other areas of the breast.

How is Paget’s Disease of the Breast diagnosed?

Diagnosis of the disease includes several steps. Among them:

  • physical exam and palpation of the lesion;
  • biopsy, the material is taken from the affected area;
  • mammography;
  • ultrasound;

palpationSkin scraping is helpful to make the correct diagnosis, but the accuracy of this method depends on the skill of the cytopathologist.

Currently, the reactions with carcinoembryonic antigen (CEA), mucin and protein c-erbB-2 are used to verify diagnosis. Immunohistochemical studies with the inclusion of cytokeratin  of epithelial membrane antigen (EMA) and oncoprotein c-erbB-2 make the process easier. However, the negative response is not sufficient to exclude this disease, so an open biopsy is needed to make the final diagnosis.

The similarity of the external manifestations of this type with skin disorders, lack of general practice and necessary knowledge, suspicion of a cancerous nature of such abnormalities in healthy young women often lead to the wrong diagnosis.

How is Paget’s Disease of the Breast Treated?

nippleThis disease is treated with surgery. An invasive form of ductal carcinoma is required a mastectomy. The breast itself and the surrounding lymph nodes are removed. When non-invasive type takes place a simple mastectomy is used. Only the affected areas are removed.

Sector resection of the organ with removal of the nipple, areola and some parts of metastasis is used when the slightest abnormal formations take place.

In order to avoid the recurrence of the disease or metastasis a hormone therapy and chemotherapy is used after surgery.

After treatment, most patients have the added benefit of a reconstructive mammaplasty.


The risk of death from breast cancer depends, firstly, on the stage of the disease, secondly, on its biological aggressiveness.

The system of prognostic factors is not static – as studies of the biological characteristics of tumors are conducted there are new criteria that influence on the individual course of the disease.

All currently known factors that have an impact on the prognosis of the common types of breast cancer play an important role in Paget’s disease of the breast as well.

Here are the most important ones:

  • the structure of the breastclinical stage;
  • age;
  • the amount of affected lymph nodes;
  • The presence of adverse morphological criteria;
  • Multicentre (especially in combination with invasive lobular carcinoma);
  • the degree of malignancy;
  • overexpression of c-erb 2neu;
  • And DNA.

Prevention of Paget’s Disease of the Breast

The main preventive measures are regular breast self-examination to find out abnormalities and changes in the nipple and areola.

If you find any abnormal formations, redness, itching, flaky skin on the nipple, discharge from it you should go to the doctor.






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