Paget’s Disease of the Breast

What is Paget’s disease?

Paget’s disease of breast, also known as Paget’s disease of nipple, is a malignant disease affecting the breast and on the breast it simulates the symptoms of eczema. This condition is unrelated to Paget’s disease of the bone. The breast disease actually starts from the nipple area and gradually spreads to other part of the breast tissue.

This condition more commonly affects elderly women over 50 years of age and in many cases there is also underlying cancer of the breast ducts (in situ or invasive ductal cell carcinoma). The most common presenting symptoms includes appearance of scaly flaky scales on the nipple along with crusting and oozing from the nipple. Surgery is the standard treatment for Paget’s disease of breast.

Breast Symptoms

Paget’s disease presents with the following symptoms :

  • Appearance of flakes or scales on the skin of the nipple. The nipple and overlying skin gradually become red, hard, crusty, thick and there is often associated oozing of discharge from the nipple. The discharge may be straw colored or bloody.
  • Sometimes there may be frank ulceration on the nipple.
  • The affected nipple becomes flattened or inverted.
  • A lump may sometimes be felt in the breast tissue
  • Some patients complain of a burning sensation in the nipple.

Usually one side of the breast is affected but in rare instances both the breasts may be affected. The severity of the symptoms may improve with time which can be misleading in that patients think the condition has resolved. However, the disease continues to progress in the underlying tissue.

Causes of Paget’s Disease

The exact cause is not known but currently it is accepted that Paget’s disease of the breast is associated with an underlying cancer of the breast tissue. Cancer is where some cells become abnormally shaped and grow excessively. It invades healthy breast tissue thereby destroying it. Cancerous cells can also spread to other sites in the body. Most cancers are due to damage of the genetic material in cells. This genetic damage is permanent and leads to uncontrollable cell growth. Breast cancer is one of the most common cancers in women.

In Paget’s disease of breast, the cancerous cells from the underlying affected breast tissue reach the nipple through the ducts running to the surface. In the United Sates, about 1% to 4% of breast cancer patients suffer from Paget’s disease of the breast. The disease occurs almost exclusively in females and very rarely in males.

Risk factors

Common risk factors include :

  • Being older.
  • Prior history of breast cancer.
  • Certain breast diseases like atypical hyperplasia
  • Family history of breast cancer.
  • Exposure to radiation.
  • Being overweight.
  • Having dense breast tissue.
  • Using hormone replacement therapy after menopause.

Treatment and Surgery

Surgery is the main treatment for Paget’s disease of breast. The two main surgical options are :

  1. Simple mastectomy where the entire breast tissue is removed.
  2. Lumpectomy where only the diseased portion including the nipple and areola are removed keeping the rest of breast intact.

In some patients additional therapy such as anti-cancer drugs, radiation or hormone therapy may also be necessary.

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