Extramammary Paget's Disease — What to watch for and what to do

If you’ve spotted a persistent, red, itchy patch in places like the vulva, scrotum, perianal area or under the armpit, don’t ignore it. That kind of patch can be a common sign of extramammary Paget's disease (EMPD). EMPD is rare, often slow-growing, and can look a lot like eczema or a fungal rash — which is why it’s routinely missed or treated with creams that only hide the problem.

How EMPD shows up

EMPD usually appears as one or more pink to red patches. The area may be scaly, crusted, moist, or bleed a little. People often report persistent itching, burning, or pain. Because it mimics other skin problems, it’s typical for symptoms to be present for months before someone thinks of a biopsy.

Common locations: the vulva in women, the scrotum or penis in men, the perianal area, and the axilla. If you see a patch that doesn’t get better after standard treatment (steroid creams, antifungals), ask for a biopsy sooner rather than later.

Triage, diagnosis and why a biopsy matters

Diagnosis starts with a skin biopsy. A dermatologist removes a small piece of skin and sends it to the lab. Pathologists use special stains to confirm Paget cells. Once EMPD is confirmed, doctors will look for any related internal cancer (for example, in the urinary tract, colon, or genital glands) depending on where the lesion sits. That often means a focused set of tests like pelvic exam, cystoscopy, colonoscopy, or imaging — your doctor will tailor tests to your symptoms and the lesion site.

Treatment choices depend on how deep or widespread the disease is. If it’s localized, surgery is the most common step. Wide local excision or Mohs micrographic surgery aims to remove the lesion with clear margins. For people who can’t have surgery, or when surgery would be disfiguring, options include topical immune therapy (imiquimod), radiation, or photodynamic therapy. If EMPD is linked to a deeper cancer, treatment follows the cancer protocol and may include more extensive surgery or systemic therapy.

One tricky fact: EMPD often comes back. Regular follow-up is necessary — usually skin checks every few months at first, then yearly if stable. Patients should also get checks for related internal cancers based on their initial workup and risk.

Practical tips: if you have a persistent patch, photograph it and note when it started and what treatments you tried. Bring a list of symptoms and any bleeding or discharge to your appointment. Don’t rely on long steroid courses without a biopsy. Seek a dermatologist experienced with uncommon skin cancers, and ask for multidisciplinary care if internal cancer is suspected.

EMPD can be managed well when found early. Spot a stubborn patch? Push for a biopsy and clear next steps — that choice makes the biggest difference.