By Steve Schroeder
It all started with a small purple pimple on my scrotum. I was pretty sure it was an ingrown hair, so it was easy to ignore. Then it turned into a dime-sized rash that looked and felt a lot like jock itch. I wasn’t overly concerned.
When I finally showed the rash to my family doctor, nine months later, she concurred with my self-diagnosis and suggested trying a different antifungal cream. When the rash didn’t go away I went back to my doctor and she invited one of her colleagues to also take a look. They both agreed it was jock itch but I just needed a stronger treatment. By this time the dime-sized rash had grown to the size of a quarter.
After a year, when the rash remained, I decided to ask my dermatologist to take a look. (As someone with a past history of malignant melanoma – diagnosed more than two decades ago – I visit a dermatologist annually.) The dermatologist continued down the same path of treating a fungal problem. On the second visit he decided I must have an allergic reaction from either shampoo, soap or fabric softener. After five more months of chasing the problem, I requested a biopsy.
A few days later, I was shocked to learn that I had extramammary Paget’s disease with invasion (Apocrine Carcinomas) of the scrotum. As I learned the news from my dermatologist over the phone, I had difficulty understanding what he was saying. I was in shock.
Searching the internet for extramammary Paget’s disease (EMPD) cancer treatment was little help. Much of what I found was limited, contradictory and confusing. Many cancer and urology websites didn’t even mention it. Finding EMPD experts, including EMPD doctors and patients that could share their knowledge and experiences with me was impossible to find. Unlike most cancers, hospitals and physicians don’t even list EMPD on their website. Of course that makes sense because EMPD is rare but it sure makes it difficult for the handful of patients that do want to find EMPD care. Fortunately, through networking, I was able to find an urologist that actually had treated a few EMPD patients previously.
Since I was having a difficult time finding EMPD information, I created a web page in hopes that others might find me. That idea went bust until The Washington Post did a story about my EMPD challenges, along with a Facebook Live interview. My single page EMPD website went down for days from the heavy traffic. From that experience men and women with EMPD, from all around the world, started to reach out. Many of them had EMPD, some suspected they did and others were writing on the behalf of family members.
I quickly set up a private Facebook support group and we now have over 60 EMPD patients that can connect with each other. This group is likely the largest group of EMPD patients outside of China and because of its size several US physicians have used its resource pool of EMPD patients for research studies.
Since setting up the initial Facebook support group, a Twitter account and a far more robust EMPD website have been created. This new website was designed to withstand any level of traffic. Several people have found the website while searching for answers to their ailments. They then went to their doctor and found out that they did indeed have EMPD.
What is extramammary Paget’s disease (EMPD)?
Extramammary Paget’s disease (EMPD) is an incredibly rare skin cancer. EMPD in women characteristically appears in the vulva or anus but can extend to the labia, mons pubis, vagina and thighs. EMPD in men usually starts around the genital or anus areas but EMPD can appear in the scrotum, penis, groin, legs and perianal areas.
EMPD patients typically indicate one or more of these symptoms: redness, rash, itching, burning sensation, soreness, scaliness, crustiness, weeping, inflammation, irritation, inflamed hair follicle or pimple, bleeding lesions or subtle skin change. Since these symptoms are similar to other ailments, EMPD is often misdiagnosed as: jock itch (tinea cruris), chronic dermatitis, non-resolving eczema, intertrigo, psoriasis, moniliasis, fungal infections and Bowen’s disease.
EMPD is usually slow-growing and most often affects postmenopausal women and men over the age of 50, with most cases occurring in individuals over 60. A biopsy, along with special staining of the tissue sample, can determine if a person has EMPD. Sometimes EMPD can point to underlying malignancies.
Mammary Paget’s disease (MPD) and extramammary Paget’s disease (EMPD) have similarities. The main difference between the cancers is where they are found, the breast versus the genital or anal regions.
EMPD treatment does not fit squarely within just one medical specialty and this can make treatment challenging. Often EMPD patents must see several different doctors for their overall care including: dermatologists, pathologists, Mohs microscopic surgeons, reconstructive urologist surgeons, plastic surgeons, oncologists, gynecologic oncologists and colorectal surgeons.
Surgery is the most common treatment for EMPD although other treatments, such as off-label topical creams, CO2 laser, photodynamic therapy and radiotherapy, are sometime tried. The age of the patient and the apparent location of the cancer can be a determining factor for the type of treatment used.
EMPD often appears to be multi-centric and finding it all is not a simple matter. Scouting biopsies are frequently used to try and determine the extent of the EMPD cancer but this approach can be similar to throwing darts at a dartboard. This is not a pleasant proposition when you are dealing with the genital or anal regions.
Most EMPD patients go many years without being properly diagnosed. This is often caused by doctors not being familiar with the rare cancer and in turn not taking a simple biopsy. Patients that are embarrassed to broach the subject with family or physicians can also delay diagnosis.
New treatments impacting EMPD
Over the past two and half years I have had five EMPD surgeries including a combination of Mohs and wide local excision surgeries requiring extensive skin grafts. With the help of the EMPD support group, the myEMPD.com website and my talented physicians, I have learned about a number of cutting edge technologies that can help EMPD patients.
A few doctors in the United States are using in vivo reflectance confocal microscopy (RCM) to help determine the extent of EMPD with “virtual biopsies”. RCM is non-invasive and allows real-time viewing of cells. In my case, physicians using RCM at Memorial Sloan Kettering Cancer Center, in New York, were able to detect EMPD cancer that was not found during traditional exams. Those findings lead to additional EMPD surgeries.
Due to the rareness of EMPD, and lack of clinical trials, chemotherapy has been used infrequently to treat EMPD. However, this may change as a few hospitals are now performing next-generation genome sequencing to look for mutations in EMPD tumors. Once a known cancer mutation is found, it can help doctors determine if any known drugs should be considered in the treatment plan.
Undergoing somatic mutation testing earlier this year, my results showed that my EMPD had several gene alterations, with two likely cancer causing. However, those genes do not currently have any FDA recognized drug therapies that have been used on other cancers that share similar gene mutations. I plan to keep a close eye on this as the data gained with next-generation sequencing is growing rapidly.
Knowledge and support is the key
My cancer buddies – as I call them – have given me insight, suggestions and ideas as I have sought out treatments and support. I can’t imagine not having the support of others while trying to battle this rare cancer. The EMPD support group includes men and women with EMPD, from all around the world, as well as their families and physicians. However, due to the age of many EMPD patients using technology such as Facebook and Twitter has been challenging for some and thus some EMPD patients remain isolated.
While it took awhile to find them, the excellent EMPD treatment that I received from physicians and hospitals so far has come from:
- Thomas Jefferson University Hospital, Philadelphia, PA
- Memorial Sloan Kettering Cancer Center, New York, NY
- University of North Carolina Health Care, Chapel Hill, NC
- Harborview Medical Center at the University of Washington Medical Center, Seattle, WA
Steve Schroeder may be contacted at myEMPD@gmail.com. Other resources include:
- myEMPD website: https://www.myempd.com
- Facebook: https://www.facebook.com/myEMPD
- Twitter: https://twitter.com/MyEMPD
- Washington Post — Doctors thought he just had jock itch. Then it spread: http://wapo.st/2eNSN36
- Washington Post — Facebook Live Video (26 minutes): https://www.facebook.com/washingtonpost/videos/10154303527997293/
© 2017, Linda Girgis MD. All rights reserved.