Extramammary Paget's disease

Extramammary Paget’s disease (EMPD), also Extramammary Paget disease, is a usually non-invasive adenocarcinoma of the skin outside of the mammary gland and includes Paget's disease of the vulva and Paget's disease of the penis. The origin of the neoplastic cells could be apocrine glands or epithelial stem cells. Skin areas rich in apocrine glands such as the genital region are typical sites of EMPD.

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Signs and symptoms

Signs and symptoms are a skin lesion often mistaken as an eczema that may be itchy or painful. A biopsy will establish the diagnosis. The histology of the lesion is the same as for Paget's disease of the breast.

Types

Paget's disease of the vulva may be a primary lesion or associated with adenocarcinoma originating from local organs such as the Bartholin gland, the urethra, or the rectum and thus be secondary. Patients tend to be in the postmenopausal range.

Paget's disease of the penis may also be primary or secondary and is less common than genital Paget’s disease in women.

Primary disease

It is important to exclude that the lesion is associated with another cancer. Primary disease is usually treated by surgical excision.

History

James Paget had described Paget disease of the nipple in 1874. Radcliffe Crocker reported the first case of EMPD in 1889 when he described a patient with a skin lesion affecting the penis and scrotum the findings of which were identical to those described by Paget. EMPD is found in a number of locations where apocrine glands are common.

See also

• Paget's disease of the breast