EMPD Explanations



Extramammary Paget's disease

Extramammary Paget’s disease (EMPD), also extramammary Paget disease, is a rare, slow-growing, usually noninvasive intraepithelial (in the skinadenocarcinomaoutside the mammary gland and includes Paget's disease of the vulva and the extremely rare Paget's disease of the penis.[1]

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[2]

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.[1]

British Assoc. of Dermatologists

British Assoc. of Dermatologists


What are the aims of this leaflet?

This leaflet has been written to help you understand more about extra-mammary Paget’s disease. It tells you what it is, what causes it, what can be done about it, and where you can find out more about it.

What is extra-mammary Paget’s disease?

Extra-mammary Paget’s disease (EMPD) is a rare, slow-growing disease that is usually due to a pre-invasive type of skin cancer. Usually it is confined to the skin, but in approximately 20% of the cases it can be associated with an invasive cancer more deeply. It typically looks similar to a patch of eczema. It usually affects skin in the genital area and around the anus of both males and females. It is commonest in people aged between 50-60 years. It can be primary, when its origin is in the skin, or secondary, when it comes from other adjacent regions internally like urethra, cervix, bladder or bowel. Paget’s disease, in contrast, refers to the same type of changes affecting the breast or nipple. There is no relation to another disease called Paget’s disease of the bone. 

DermNet New Zealand

DermNet New Zealand

Extramammary Paget disease

Extramammary Paget disease is an uncommon cancer characterised by a chronic eczema-like rash of the skin around the anogenital regions of males and females. Under the microscope extramammary Paget disease looks very similar to the more common type of mammary Paget disease that occurs on the breast.

Who gets extramammary Paget disease?

Extramammary Paget disease most commonly occurs in the vulva of women aged between 50–60 years. It can also affect males of similar age.