Learn about Extramammary Paget’s disease (EMPD)
What is extramammary Paget’s disease (EMPD)?
Extramammary Paget’s disease (EMPD) is a rare intraepithelial adenocarcinoma (skin cancer) that can appear around the genitals, anus, perineum, groin or axillae (armpit) areas in women and men. In the rarest of rare cases it can appear elsewhere in the body. EMPD has cellular similarities to Paget’s breast cancer.
Extramammary Paget’s disease (EMPD) often appears as a scaly, itchy or red area within the genital, anal or armpit regions. The cancer is extremely rare, generally slow growing, and is often misdiagnosed. The prognosis and recovery from EMPD is often good, especially if it is found and treated early. However, some cases of EMPD can be more aggressive, invading the dermis and subcutaneous tissue.
It is often difficult to find a physician that is familiar with EMPD. Further, EMPD symptoms often mimic other conditions, which can slow diagnosis.
The most common EMPD symptoms include a rash or red, scaly, or crusty area around the genital or anal area. These spots often itch or burn and sometimes bleed. EMPD patients often remark that their symptoms feel like "Jock Itch". Occasionally, patients have no obvious symptoms or only have a slight increased sensitivity in the region. EMPD generally affects women and men over the age of 40, with the average age for EMPD diagnosis being 66. In spite of that, EMPD has been diagnosed in people as young as 17 years old.
The vulva is the most common location for EMPD with it being involved in 65% of EMPD cases. Roughly 20% of EMPD diagnoses involve the perianal area followed by the scrotum and penis accounting for 14%. EMPD occurrences outside the genital or anal areas comprise less than 1% of all EMPD cases.
EMPD begins as a cancer in the epidermis and if left untreated may progress to invade the dermis, increasing the chances of underlying malignancies. Research varies but medical reviews indicate 10 to 30% of those that are diagnosed with (primary) EMPD may also develop a (secondary) cancer. This secondary cancer may occur before, simultaneously, or after an EMPD diagnosis. Secondary EMPD can range from other skin cancers to internal malignancies.
EMPD is typically slow growing and can take years to become noticeable and even longer to diagnose. The prognosis for a patient with primary EMPD is generally favorable. Such a prognosis can be impacted by how early a diagnosis is made, the treatment received, the age of the patient, and the location, depth, and overall amount of EMPD. The prognosis is best when EMPD is only located in the epidermal (top layer of the skin) and the surrounding area is surgically removed, and cancer-free margins are found.
Typically extramammary Paget’s disease (EMPD) will appear in the genital, anal or arm pit areas in women and men. However, in the rarest of rare cases it can appear elsewhere such as the buttocks, thigh, legs, arms, eyelid, scalp, nose, temple and external auditory canal. In this photo EMPD appears on the arm. When EMPD affects nonapocrine bearing skin, it is called ectopic extramammary Paget’s disease.
EMPD in women typically appears in the vulva or anus but can extend to the labia, mons pubis, vagina and thighs. EMPD in men generally starts around the genital or anal areas but EMPD can appear in the scrotum, penis, groin, legs and perianal regions. In extremely rare cases, EMPD has also been diagnosed on the axillae (armpits), buttock, thigh, arm, eyelid, scalp, nose, temple and external auditory canal. EMPD typically appears near apocrine-rich sweat glands.
Due to its rarity, no clear diagnostic or standardized EMPD treatment guidelines have been developed. Since the complexity of EMPD varies, a cross-disciplinary approach with EMPD care, involving several physicians and specialities, is not unusual in many countries. Depending on where the EMPD is located, physicians treating EMPD frequently include: dermatologist, Mohs surgeon, plastic surgeon, general surgeon, colorectal surgeon, medical oncologist, genitourinary oncologist, reconstructive urologist surgeon and gynecologic oncologists.
Surgical interventions, such as Mohs micrographic surgery (MMS) or wide local excision (WLE), are the most common treatments for EMPD although other approaches are also used. Other treatments include: topical creams, photodynamic therapy (PDT), carbon dioxide (CO2) laser, cavitational ultrasonic surgical aspiration (CUSA), radiation therapy, chemotherapy, immunotherapy, or hormone therapies. Disease management may also include a combination of treatments. Regardless of the treatment choice, reoccurrence of EMPD is common.
Scouting or mapping biopsies are often used to help understand the extent of the EMPD and where the EMPD may be located. Some doctors now use in vivo reflectance confocal microscopy (RCM) which can also help determine the extent of EMPD with digital "virtual biopsies". Even though RCM can help locate EMPD, traditional biopsies continue to be used as the gold standard in determining EMPD. Traditional biopsies require immunohistochemical staining and the review by a knowledgeable pathologist.
““If you suspect you might have extramammary Paget’s disease (EMPD), I encourage you to see a doctor. Don’t put it off and don’t be embarrassed!” ”
Mammary Paget's disease (MPD) also known as mammary Paget’s disease of the breast and Paget's disease of the nipple and extramammary Paget’s disease (EMPD) have similarities. The main difference between the cancers is where they are found in the body, the areola or nipple versus the genital or anal region. The origins of these cancers are not fully understood.
Paget's disease of bone is not related to EMPD or MPD cancers. The cause of Paget's disease of bone is unknown but scientists suspect environmental and genetic factors.
This website is dedicated to assisting those that have been diagnosed with extramammary Paget's disease (EMPD), those who have EMPD symptoms, and family members of those with EMPD. This information is not intended as a substitute for professional medical care. If you suspect you may have extramammary Paget’s disease (EMPD) you are encouraged to visit your doctor. Always follow your healthcare professional’s instructions.
additional EMPD information
Extramammary Paget’s disease (EMPD) symptoms, treatment, and overview information may be found here:
The content presented on this website, encompassing both textual material and visual media, serves educational and informational purposes aimed at aiding individuals affected by extramammary Paget’s disease (EMPD) and their families in comprehending the complexities associated with EMPD. It is imperative to note that the website is not designed to serve as a replacement for professional medical advice, diagnosis, or treatment. Furthermore, the website does not advocate for or endorse any specific treatments, tests, physicians, procedures, or products. Should you suspect that you are experiencing symptoms of EMPD, we strongly advise you to seek guidance from your healthcare provider.