Paget's disease of the breast is a rare form of breast cancer, accounting for less than 5 percent of all breast cancers. Paget's disease of the breast starts in the breast ducts and extends to the skin of the nipple and to the dark circle of skin (areola) around the nipple. Paget's disease of the breast isn't related to Paget's disease of the bone, a metabolic bone disease.

Paget's disease of the breast occurs most often in women over age 50. Most women with Paget's disease of the breast have underlying infiltrating ductal breast cancer.

• Paget's disease of the nipple also occurs at other sites, most often in the groin or perianal area (extramammary Paget's disease)
• The bladder, anus, and rectum are the most common sites
• Extramammary Paget's disease is a rare intraepithelial adenocarcinoma of apocrine gland–bearing sites

Symptoms

• Flaky or scaly skin on your nipple
• Crusty, oozing or hardened skin resembling eczema, on the nipple, areola or both
• Redness
• Itching
• A burning sensation
• Straw-colored or bloody nipple discharge
• A flattened or inverted nipple
• Affected areas beyond the nipple and areola
• A distinct lump underneath the nipple and areola
• Skin and nipple changes usually in one breast only
• Fluctuating skin changes early on, making it appear as if your skin is healing on its own

Risk factors

• Older age > 50 yrs
• A personal history of breast cancer
• Family history of breast cancer
• Genetic predisposition. Defects in one of several genes, especially BRCA1 or BRCA2
• Radiation exposure
• Excess weight
• Exposure to estrogen- Taking estrogen, such as for hormone replacement therapy, also increases the risk of breast cancer for some women.

Tests and diagnosis

Clinical breast exam and physical exam

Mammography — an X-ray exam of your breast tissue

Breast biopsy

Treatment

• Simple mastectomy or lumpectomy
• Ablation of overlying cutaneous involvement, either surgically or by CO2 laser ablation
• Adjuvant therapy- anti-cancer drugs or hormone therapy to prevent a recurrence of the breast caner.