Extramammary Paget's disease
|Extramammary Paget's disease|
|Classification and external resources|
|ICD-10||C44 (ILDS C44.L75)|
Extramammary Paget’s disease (EMPD), also extramammary Paget disease, is a rare, slow-growing, usually noninvasive 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.
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. Isolated Paget's disease of the penis is extremely rare.
Signs and symptoms
Signs and symptoms are skin lesions, 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.
It is important to exclude that the lesion is associated with another cancer.
Extramammary Paget's disease is usually seen in isolation and is associated with an underlying invasive malignancy about 12% of the time. It is associated with an underlying adnexal malignancy about 24% of the time. Paget's disease of the breast is almost always associated with an underlying invasive malignancy, i.e. breast cancer (e.g. mammary ductal carcinoma).
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.
- Ekwueme, Kingsley C; Zakhour, Hani D; Parr, Nigel J (1 January 2009). "Extramammary Paget's disease of the penis: a case report and review of the literature". Journal of Medical Case Reports 3 (1): 4. doi:10.1186/1752-1947-3-4.
- Chanda, JJ. "Extramammary Paget's disease: prognosis and relationship to internal malignancy.". Journal of American Academy of Dermatology.
- Marques-Costa, JC; Cuzzi, T; Carneiro, S; Parish, LC; Ramos-e-Silva, M (May–Jun 2012). "Paget's disease of the breast.". Skinmed 10 (3): 160–5. PMID 22779098.
- Definition at cancer.org