Mammary Paget disease

Author: Vanessa Ngan, Staff Writer, 2003.


What is mammary Paget disease?

Mammary Paget disease is an uncommon skin cancer characterised by a chronic eczema-like rash of the nipple and adjacent areolar skin. It is also called Paget disease of the nipple.

Mammary Paget disease is associated with an underlying cancer, either in situ adenocarcinoma of the breast or a more widespread infiltrating cancer, although this is sometimes difficult to detect on clinical examination or by mammogram.

A similar condition that involves the skin of female and male genitalia is known as extramammary Paget disease.

Who gets mammary Paget disease?

Mammary Paget disease is most commonly diagnosed between 50 and 60 years of age. It is much more common in women than in men. 

What are the clinical features of mammary Paget disease?

Most patients with mammary Paget disease complain of an itchy, burning rash on and around the nipple area. Sometimes the skin may be broken and sore from itching. Other symptoms include:

  • Redness
  • Swelling
  • Oozing nipple discharge
  • Scaling
  • Bloody nipple discharge
  • Ulceration
  • Inversion of the nipple

Scaly, crusty and thickened plaques often form on the nipple, which then spreads to the surrounding area. Usually only one nipple is affected although rare cases of involvement of both nipples has been seen.

Paget disease

How is mammary Paget disease diagnosed?

Although it may be suspected clinically, skin biopsy of the lesion is performed for accurate diagnosis of mammary Paget disease. Under the microscope, the presence of Paget cells along with other histological findings confirms diagnosis. Skin biopsy may also determine whether there is underlying cancer, although some breast carcinomas are not seen because they are situated more deeply in the breast tissue. A mammogram may be performed to accurately locate the underlying cancer prior to breast biopsy.

What is the differential diagnosis for mammary Paget disease?

The differential diagnosis for mammary Paget disease includes:

  • Nipple eczema (this is much more common than mammary Paget disease, especially in adolescents and young adults)
  • Nipple papillomatosis
  • Naevoid hyperkeratosis of the nipple

What is the treatment of mammary Paget disease?

Mastectomy (removal of the breast) is often necessary treatment for mammary Paget disease. Alternatively, it can be widely locally excised along with samples of tissue taken from nearby lymph nodes in the armpit. Sometimes conservative treatment such as partial nipple excision, wedge excision, cone excision, radiotherapy, or a combination of these may be used in women with less advanced stages of the disease. However, recurrence is common in these cases.

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Related information

 

References

  • Book: Textbook of Dermatology. Ed Rook A, Wilkinson DS, Ebling FJB, Champion RH, Burton JL. Fourth edition. Blackwell Scientific Publications.

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