Introduction
Paget’s disease of the breast (aka Paget’s disease of the nipple) is a form of breast cancer which is malignant and which has an eczematous appearance, involving the nipple. It is commonly associated with an underlying ductal carcinoma in situ. It is associated with 2% of all cases of breast cancer.
It should NOT be confused with Paget Disease of the Bone which is a completely unrelated disease. Generally the term “Paget’s disease” refers to that of the bone.
Presentation
- An erythematous ‘eczema-like’ rash, usually unilateral.
- Itchy, inflamed nipple
- Burning sensation
- Discharge from the affect area
- May also be discharge from the nipple related to the underlying cancer
- Inverted nipple
Pathology
It is caused by the presence of Paget’s cells in the epidermis of the nipple. These are large cells derived from the original carcinoma – even though no direct connection may be seen. These cells are themselves malignant.
Investigations and treatment
Histology may confirm the diagnosis, but as the condition is indicative of underlying carcinoma, further treatment is as for breast cancer.
Extramammary Paget’s disease (EMPD)
A rare condition that may affect the vulva or penis. The local pathology is the same as for Paget’s disease of the nipple (large Paget’s cells resulting in an eczema like appearance), but EMPD is not normally associated with underlying breast malignancy, and instead is associated with malignancy of other local glandular tissue, such as the urethra or rectum.
- In the penis it is extremely rare
- Primary cases do exist – and are treated with local excision
Differentiating Paget’s and Eczema
- Paget’s typically starts at the nipple and works outwards
- Eczema starts at the periphery of the areolar and works inwards
References
- Murtagh’s General Practice. 6th Ed. (2015) John Murtagh, Jill Rosenblatt
- Oxford Handbook of General Practice. 3rd Ed. (2010) Simon, C., Everitt, H., van Drop, F.
- Beers, MH., Porter RS., Jones, TV., Kaplan JL., Berkwits, M. The Merck Manual of Diagnosis and Therapy