Paget's Disease of the Nipple

Original article by Tom Leach | Last updated on 28/5/2014
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Introduction

A malignant disease which has an eczematous appearance, involving the nipple. It is commonly associated with an underlying ductal carcinoma in situ. It is associated with 2% of all cases of breast cancer.
 

Presentation

  • An erythematous ‘eczema-like’ rash, usually unilateral.
  • Itchy, inflamed nipple
  • Burning sensation
  • Discharge from the affect area
    • May also be discharge from the nipple related to the underlying cancer
  • Inverted nipple
 

Pathology

It is caused by the presence of Paget’s cells in the epidermis of the nipple. These are large cells derived from the original carcinoma – even though no direct connection may be seen. These cells are themselves malignant.
 

Investigations and treatment

Histology may confirm the diagnosis, but as the condition is indicative of underlying carcinoma, further treatment is as for breast cancer.
 
Extramammary Paget’s disease (EMPD)
A rare condition that may affect the vulva or penis. The local pathology is the same as for Paget’s disease of the nipple (large Paget’s cells resulting in an eczema like appearance), but EMPD is not normally associated with underlying breast malignancy, and instead is associated with malignancy of other local glandular tissue, such as the urethra or rectum.
  • In the penis it is extremely rare
  • Primary cases do exist – and are treated with local excision
 
Differentiating Paget’s and Eczema
  • Paget’s typically starts at the nipple and works outwards
  • Eczema starts at the periphery of the areolar and works inwards