About Extramammary Paget’s disease (EMPD)
What is extramammary Paget’s disease (EMPD)?
Extramammary Paget’s disease (EMPD) is a rare skin cancer that can appear around the genitals, anus, perineum, groin or arm pit areas in women and men. In the rarest of rare cases it can appear elsewhere in the body. EMPD has similarities to Paget’s breast cancer.
Since EMPD is so rare, it is often difficult to find a physician that is familiar with the disease. Further, EMPD symptoms often mimic other conditions slowing diagnosis.
Aggressive surgical intervention is often necessary although other treatments are also used. EMPD begins as an in situ carcinoma in the epidermis and may progress to invade the dermis, increasing the chances of underlying malignancies.
The most common symptoms include a rash or red, scaly, or crusty areas around the genital or anal area. These areas often itch or burn and sometimes bleed. Men often say the symptoms feel like "Jock Itch". Occasionally, patients have no obvious symptoms or only have a slight increased sensitivity in the region. EMPD generally affects postmenopausal women and men over the age of 50, with most cases occurring in individuals over 60. However, EMPD has been diagnosed in people as young as 29 years old.
EMPD is typically slow growing – but not always – and can take years to become noticeable and even longer to diagnose. Left unchecked, EMPD cells are capable of progressing to invasive carcinoma and metastasizing internally. This is why it's critical to have any unusual symptoms in the genital, anal or armpit regions checked by a physician. Due to its rarity, no clear diagnostic or standardized EMPD treatment guidelines have been developed.
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 anus 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, eyelid, scalp, nose and external auditory canal. EMPD can occur in any of the apocrine-rich sweat glands.
When surgery is used it’s important to determine the edges and extent of the cancer. Scouting biopsies are often used to help understand where the EMPD may be located.
A private support group for EMPD patients, family members, and doctors who have treated EMPD is available. If you or a family member has been diagnosed with EMPD and would like to talk with others that have been treated for EMPD, contact Steve Schroeder.
Since the complexity of EMPD varies, a cross-disciplinary approach with EMPD care, involving several physicians, is not unusual in the United States. Depending on where the EMPD is located, physicians treating EMPD frequently include: dermatologist, Mohs surgeon, plastic surgeon, general surgeon, colorectal surgeon, medical oncologist, reconstructive urologist surgeon and gynecologic oncologists.
In defined areas, Mohs microscopic surgery can often be helpful in treatment. If EMPD covers a large area on a male, a reconstructive urologist surgeon may also be a key player on a treatment team. In larger areas, sometimes skin grafts are used. Women with EMPD commonly use a gynecologic oncologist as part their team.
Biopsies are the traditional way to determine the extent of EMPD. However, 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. Other treatments may include radiotherapy, CO2 laser, topical creams or photodynamic therapy (PDT).
Due to the rareness of EMPD, and lack of clinical trials, chemotherapy is used infrequently to treat EMPD. However, this is starting to change as doctors are now able to perfrom next-generation genome sequencing to look for mutations in tumor cells. Once a known cancer mutation is found, it can help doctors determine if any known pharmaceutical remedies or immunotherapies should be considered in the treatment.
“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!” – Steve Schroeder (EMPD Patient)
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. Many scientists believe Paget's disease of bone is related to a viral infection.
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. For more information on EMPD, review the research, doctors, news, and Mohs surgery, skin grafts, non- surgical treatments, confocal imaging and gallery sections of this website.