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Introduction

Paget disease of the breast (PDB), also known as mammary Paget disease, is a rare form of breast cancer that presents as a unilateral erythematous outbreak involving the skin of the nipple and areola.  It is often associated with underlying ductal carcinoma in situ (DCIS) or invasive breast carcinoma.

Pathophysiology

The exact pathophysiology of PDB is poorly understood.  Malignant cells from underlying breast adenocarcinoma (DCIS or invasive carcinoma) are thought to migrate within the ductal system and lactiferous sinuses into the areola and spread outward, causing the characteristic eczema-like outbreak.

Risk factors

The strongest risk factors for PDB include:

  • Age:  >50, although it can occur at any age
  • Gender (occurs predominantly in women but can also affect men)
  • Genetics:  inherited cancer syndromes (eg,

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