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Extramammary Paget's disease (EPD) is a neoplasm of the anogenital and axillary skin, histologically and clinically similar to Paget's disease of the breast, and often represents an intraepiderrnal extension of a primary adenocarcinoma of underlying apocrine glands or of the lower gastrointestinal, urinary, or female genital tracts.


Causes:

The histogenesis of EPD is not uniform. Paget cells in the epidermis may occur as an in situ upward extension of an in situ adenocarcinoma in deeper glands (25%). Alternatively, EPD may have a multifocal primary origin in the epidermis and its related appendages. The primary tumor and paget cells are usually mucus-secreting. Primary tumors in the anorectum can arise within the rectal mucosa or intramuscular glands.


Symptoms:

At clinical examination, EMPD may appear as chronic intertrigo or presumed tinea cruris. EMPD may appear eczematous, and it has usually been present for a long time before biopsy is performed to confirm the diagnosis.

Initially, only slight erythema, crusting, and increased maceration may be noted.

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