Rare condition presents as a roughening, reddening, and slight ulceration of the nipple; the vast majority of Paget’s (97%) will also have an ****underlying neoplasm, either in situ or invasive disease

Aetiology

• Risk factors as for breast carcinoma

Pathophysiology

• Microscopically there is involvement of the epidermis by malignant ductal carcinoma cells
• The mechanism behind Paget’s disease is currently unknown, however it is hypothesised that either malignant cells migrate from the ducts to the nipple surface or the cells of the nipple themselves become malignant

Clinical presentation

• Usually presents as a dry, scaly, eczematous lesion
• Can also present as an erythematous weeping lesion on the surface of the nipple and the areola
• There is often an underlying palpable mass or a radiological abnormality

Investigations

• Histologic tissue biopsy (incisional or punch biopsy)

Management

• Surgery first line - the type of surgery depends on how advanced the underlying breast cancer is, but in all cases the nipple and areola will need to be removed
• In the cases associated with an underlying malignancy, radiotherapy may also be necessary