Define paget disease of the breast.
a rare type of breast cancer that affects the lactiferous ducts and the skin of the nipple and areola
Describe the pathogenesis.
Not fully understood
The following two hypotheses have been postulated: [14]
Migratory/epidermotropic theory: neoplastic ductal epithelial cells from an underlying DCIS or IDC move through the lactiferous ducts and invade the surrounding epidermis of the nipple.
In situ malignant transformation of existing cells into Paget cells, meaning that the condition is a carcinoma in situ, without preexisting DCIS/IDC.
List the clinical features.
Erythematous, scaly, or vesicular rash affecting the nipple and areola
Pruritus; burning sensation
Nipple retraction
Ulceration that causes blood-tinged nipple discharge
Which diagnostics should be carried out?
Punch/wedge or surface biopsy of nipple tissue: Paget cells confirm disease.
Imaging to look for underlying DCIS/IDC
What is a DD?
mamillary eczema
While it may also manifest as an eczematous rash with poorly defined margins, mamillary eczema shows no signs of infiltration, unlike Paget disease. However, Paget disease is often misdiagnosed as mamillary eczema.
Describe the treatment.
Breast-conserving surgery with subsequent adjuvant whole-breast radiation
Mastectomy
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