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What is Paget disease of the breast?
Paget disease (PDB) is a form of breast cancer that can present as a rash or eczema-like lesion on the nipple, areola, or breast. It is most commonly associated with underlying ductal carcinoma in situ of the breast. Invasive breast cancer has been noted to be present in some cases as well.
Who is at risk?
The risk factors for PDB are ...
- Over the age of 50 (although cases have been noted in patients in their 20s)
- Personal history of breast cancer or atypical changes
- Family history of breast or ovarian cancer
- Dense breast tissue on mammogram
- History of radiation exposure
- BRCA1 and BRCA 2 gene mutations
- Hormone replacement therapy post menopause
- Excess alcohol intake
How does it present?
I suspect that Paget’s disease may be underdiagnosed due to its presentation. It can appear as eczema-like patches or plaques on the breast. It is estimated to account for about 1 to 4% of breast cancer cases. However, it does not always have associated mammographic changes.
In fact, studies reveal that 50% of cases may have a mammographic change. MRI can be helpful to detect changes associated with malignancy, however, there are cases where the MRI has also been negative.
The cases I have diagnosed have most often presented as eczema-like changes. Many times patients have already tried a topical steroid and noted that there is little response. This is an important indication to biopsy the affected skin. A punch biopsy of the affected skin performed in-office can provide the key information needed for a diagnosis.
As a side note, I have found that it can be difficult for some patients to understand the need for a biopsy in these cases. Since it is possible to not have an underlying palpable mass or nodule and also possible to have a normal mammogram, I have run into circumstances where a patient simply does not believe that there could be a problem. Taking the time to discuss and educate patients is so important as this is not a common way for breast cancer to present.
How is it treated?
If a biopsy reveals evidence of Paget disease of the breast, then the next steps would be similar to if a breast biopsy for a mass came back as positive. Staging the extent of disease and determining treatment interventions follow the same algorithm as for other breast cancers.
Due to the fact that this type of presentation is often associated with ductal carcinoma in situ which is an early presentation of breast cancer, it is ideal that more women recognize this disease by its subtle skin findings.