| Extramammary Paget's disease | |
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| Classification and external resources | |
Micrograph of extramammary Paget's disease. H&E stain. |
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| ICD-10 | C44 (ILDS C44.L75) |
| ICD-O: | M8542/3 |
| MeSH | D010145 |
Extramammary Paget’s disease (EMPD), also Extramammary Paget disease, is a rare, slow-growing, usually non-invasive intraepithelial (in the skin) adenocarcinoma outside of the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis.[1] Extramammary Paget’s disease involves primarily the epidermis but occasionally extends into the underlying dermis.[1] It has a predilection for apocrine gland bearing areas, mostly the perineum, vulva, axilla, scrotum, and penis.[1]
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Extramammary Paget's disease is usually seen in isolation and is not usually associated with an underlying invasive malignancy. This is unlike Paget's disease of the breast, which is almost always associated with an underlying invasive malignancy, i.e. breast cancer (e.g. mammary ductal carcinoma).
Signs and symptoms are a skin lesion often mistaken as 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.
Paget's disease of the vulva, a rare disease, 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 postmenopausal.
Paget's disease of the penis may also be primary or secondary and is even rarer than genital Paget’s disease in women. At least one case has been misdiagnosed as Bowen's disease.[1] Isolated Paget's disease of the penis is extremely rare.[1]
It is important to exclude that the lesion is associated with another cancer. Primary disease is usually treated by surgical excision.
James Paget described Paget's 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.
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