Paget Disease of The Breast

Introduction

• Description: Paget disease of the breast is a malignant process that involves the nipple and areola. It may also rarely involve the skin of the vulva. Paget disease is named for the 19th-century British doctor Sir James Paget, who, in 1874, noted a relationship between changes in the nipple and breast cancer.

• Prevalence: 1%–4% of breast cancers.

• Predominant Age: Menopausal and perimenopausal age. Average is 57 years.

• Genetics: No genetic pattern. There is a report of FOXA1 dysregulation in mammary and extramammary Paget disease.

Etiology and Pathogenesis

• Causes: Considered to arise in the dermoepidermal junction from multipotent cells that can differentiate into either glandular or squamous cells, although ductal migration of cells from an associated malignancy cannot be ruled out.

• Risk Factors: None known.

Signs and Symptoms

• Pruritic, red, eczematoid, flaky skin lesion, often associated with bleeding and crusting, that begins on the nipple and spreads to the areola. Flattening of the nipple is common. Pain, burning, and/or pruritus may precede the development of the clinically evident lesion by 6–8 months.

• Almost always associated with infiltrating or intraductal carcinoma in deeper parts of the breast

Diagnostic Approach