Extramammary Paget disease (EMPD) is the rarer non-breast form of Paget disease of the nipple. It is considered a form of adenocarcinoma of the apocrine glandular tissue. In men, the penis and scrotum are most frequently involved, and in women the vulva. Nodal and distant organ metastatic disease is seen.
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Epidemiology
It is rarer than Paget disease of the nipple, accounting for less than 10% cases found. The commonest affected site when data from both sexes is combined is the vulva, with penoscrotal disease being much less common.
Associations
- GI malignancy
- GU malignancy
Clinical presentation
- solitary or multiple dry, reddened, elevated skin lesions with indolent growth
- locations include penoscrotal, anorectal and vulval
- lesions may ulcerate
- pruritus (may occur before any visible skin abnormality)
- initially the lesions are often misdiagnosed as benign disease e.g. fungal infections
Complications
- metastases to nodes, lung and bone
Treatment and prognosis
Due to the rarity of the condition, optimal treatment has not been adequately researched. Nevertheless it is clear that best results are seen with surgical resection of local disease. Radiotherapy and chemotherapy are also both used 1.
History and etymology
This condition is named after Sir James Paget (1814-1899) British surgeon and pathologist 2.
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