Extramammary Paget's disease

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

Classification

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

Symptoms are not specific; most patients report itching, burning, and soreness. A small subset of patients may be asymptomatic. Presence of vulvar pain, bleeding, and tumor formation are reported to be more common in patients affected by invasive disease.