Rare Cancer: Gynecologic Extramammary Paget's Disease

@bijou68
I waa recently dx with vulvar empd at Univ of Chgo after 8 months being treated with Lichen Sclerosus. They used every "gold standard" topical on me and it seemed like I was getting worse. A scrape biopsy at U of Chgo was 1st done by a dermatologist and empd was dx. I was then sent to another doctor, gyne/oncologist that did 2 more punch biopsies with the same empd dx. The skin being primary.
I have been reading numerous articles about vulvar empd. There is Mohs, modified Mohs procedures that saves as much good tissue as possible. Then there is WLE which stands for Wide Local Excision. After all my reads this last one has a high recurrence. I am on the midst of seeing if U of C does Mohs as I don't want to have this keep coming back.
I have also read up on Imiquimod and am scared to use this. How bad 8s the burning/oozing/
Stabbing and having to use for months. When you're reoccurs is the area reddened, itching, burning? What do you use for barrier cream that works?
I pray they find a better resolution for all of us and I also pray for peace of mind. I'm sorry you and everyone else 😔 is on this journey.

Good morning, Naomi: By surveillance I meant: once a year I go to my OB/GYN for her to look at the vulva area; I would go twice a year if I could, but I think that is what I am allowed on my Medicare plan. She has spotted manifestations of EMPD every time. This is what I meant by visuals. Of course she uses a very strong light to observe. Then either she does the biopsies or has my oncologist perform them. Every time I have a biopsy, it is positive. Then, I start Imiquimod like same day or day after for about 8 weeks. After the treatment I go see my oncologist. Imiquimod treatment was suggested by another gynecologist skin specialist who knows both my Ob/GYN and my oncologist. I am being told EMPD is slow at developing but then I read a comment on this site from a lady who said, her EMPD was very aggressive and now the cancer has spread. I was told Imiquimod was originally used for other skin related treatments. I would not have known I had EMPD of the vulva if I did not go to my last Pap Smear at 65. Both my OB/GYN and my oncologist encourage me to tell all my friends and others to keep going once a year for the visual. My OB/GYN thinks that Papilloma virus might be causing EMPD but there is no supporting evidence yet. However, my oncologist says that she does not think so. In any event: I had Papilloma virus about 20 or 22 years ago and went through burning of the tissues affected around the cervix. My OB/GYN is guessing that then the doctor may have not gotten every cell affected by Papilloma Virus. Now children (boys and girls) are getting the vaccination.

I am so sorry you have gone through so much. I have had Lichen Sclerosis for years and recently had a biopsy and was diagnosed with non-mammary paget disease of the vulva. Seeing a gynocological oncologist. He said he does not want to remove as it just comes back. I get that but frustrated with credible information on best treatment options such as the Immiquimod cream. After performing an assessment the dr. found another area that he "didn't like at all ". So now I have surgery scheduled to remove that area that was not biopsied. Very frustrated 🙁
Did you have any testing for underlying cancer?

Feb 7 2025
I haven’t checked in for awhile. I thought I had offered everything I could. My update: I had my 7th, yes seventh, Paget’s surgery since 2013 in Dec. 2024. Each time I say “never again” & each time I say, “one more time”, thinking maybe this will be the last one. Never had totally clean margins, but also no invasive cancer, so I guess I feel I’m ahead. I just keep wanting to get rid of as many Paget’s cells as possible.
I unsuccessfully tried Immiquimod two separate times between surgeries. Had burning, itching, & even swelling in my leg.
Each surgery takes a few months to fully recover, so back to skirts, carrying pillows, & depression. Yet I do not regret my decisions to have the surgeries. Anything I can do to fight this wretched disease is worth it. However I will be 80 in 2025, so my recent surgery may be my last.
At least I have educated literally 100s of individuals, including medical personnel, about Paget’s. I talk about it to anyone who will listen. Hopefully, with increased awareness, there will be increased research & help for future sufferers.

I hear you, and wish there was a painless medication-free treatment, but we don’t seem to have one yet. Imiquimod (Aldara) works by irritating the tissue, thereby stimulating the body’s immune system to attack and kill the cancer cells. Think of the old exercise adage, “No pain, no gain.” My first time using imiquimod was tough—I only got through 6 of the prescribed 12-week treatment because the rawness became unbearable. Still, the lesions did shrink. The second time I used it, I was able to tolerate it much better. I applied the cream with a Q-Tip to keep the treated area as small as possible. Also, applying lidocaine cream 30 minutes prior to the imiquimod eased the discomfort enough to allow me to go to sleep. Some women say they take a week or two off to let the skin heal a bit, then resume treatment. I know how frustrated you are to be facing this, and I hope it is a tolerable option for you.

Feb 7 2025
I haven’t checked in for awhile. I thought I had offered everything I could. My update: I had my 7th, yes seventh, Paget’s surgery since 2013 in Dec. 2024. Each time I say “never again” & each time I say, “one more time”, thinking maybe this will be the last one. Never had totally clean margins, but also no invasive cancer, so I guess I feel I’m ahead. I just keep wanting to get rid of as many Paget’s cells as possible.
I unsuccessfully tried Immiquimod two separate times between surgeries. Had burning, itching, & even swelling in my leg.
Each surgery takes a few months to fully recover, so back to skirts, carrying pillows, & depression. Yet I do not regret my decisions to have the surgeries. Anything I can do to fight this wretched disease is worth it. However I will be 80 in 2025, so my recent surgery may be my last.
At least I have educated literally 100s of individuals, including medical personnel, about Paget’s. I talk about it to anyone who will listen. Hopefully, with increased awareness, there will be increased research & help for future sufferers.

Feb 7 2025
I haven’t checked in for awhile. I thought I had offered everything I could. My update: I had my 7th, yes seventh, Paget’s surgery since 2013 in Dec. 2024. Each time I say “never again” & each time I say, “one more time”, thinking maybe this will be the last one. Never had totally clean margins, but also no invasive cancer, so I guess I feel I’m ahead. I just keep wanting to get rid of as many Paget’s cells as possible.
I unsuccessfully tried Immiquimod two separate times between surgeries. Had burning, itching, & even swelling in my leg.
Each surgery takes a few months to fully recover, so back to skirts, carrying pillows, & depression. Yet I do not regret my decisions to have the surgeries. Anything I can do to fight this wretched disease is worth it. However I will be 80 in 2025, so my recent surgery may be my last.
At least I have educated literally 100s of individuals, including medical personnel, about Paget’s. I talk about it to anyone who will listen. Hopefully, with increased awareness, there will be increased research & help for future sufferers.