Paget's disease of the breast is described as an uclerated lesion that begins at the nipple then spreads out into the areola. This ulceration may vary in its character, and may be described as eczematous, vesicular, scaly, or raw, or any combination of these.
The skin lesion seen in Paget's disease of the breast varies in terms of its appearance and characteristics, but is most commonly seen as eczematous and/or pruritic.
Pruritis, or itching, is a symptom commonly reported in association with Paget's disease of the breast. Specifically, the itching tends to occur near and around the rash.
Approximately half of patients with Paget's disease of the breast are also found to have a palpable breast mass. This finding is important, as it further raises the suspicion for underlying malignancy and the presence or absence of this finding will later guide decision making regarding treatment options.
Occasionally, patients with Paget's disease of the breast may report bloody or yellow-green discharge, though this finding is not always present and therefore its absence does not exclude Paget's from the differential diagnosis.
It is important to recognize Paget's disease of the breast, because many cases have been shown to be associated with underlying malignancy related to the epithelial cells of the lactiferous ducts. This may be in the form of ductal carcinoma in-situ (DCIS) or invasive ductal carcinoma, depending on the progression of the malignancy at the time of diagnosis.
The skin findings seen in Paget's disease of the breast are thought to be a result of malignant ductal cells migrating through the lactiferous ducts to the epidermis. Another theory is that the epidermal keratinocytes within the nipple are transformed into malignant cells, representing an epidermal rather than dutal carcinoma. This theory is far less widely accepted however.
Patients with clinical findings suspicious for Paget's disease of the breast should undergo breast biopsy, ideally a punch or full-thickness wedge biopsy of the nipple.
Bilateral mammography or mammogram is necessary in order to identify any underlying mass in addition to assess for the possibility of bilateral disease. Findings on mammography and biopsy will then be important in guiding treatment, specifically regarding the choice of treatment between breast conservation therapy (IE lumpectomy plus radiation) versus mastectomy.
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