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Diseases

Genetic and Rare Diseases Information Center (GARD)

Paget disease, extramammary


Other Names for this Disease
  • Extramammary Paget disease
  • EMPD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

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How might extramammary Paget disease be treated?

Extramammary Paget disease (EMPD) is usually treated by surgically removing the skin lesion. It is sometimes difficult to determine how much skin should be removed, particularly when lesions are spread throughout the anogenital region. Because recurrence is common, it is standard for patients to be re-examined every 3 months after surgery for the next 2 years. After 2 years, annual follow-ups are usually recommended.[1][2]

In cases where surgery is not a possibility due to recurrence or location; treatment options reported in the medical literature with varying levels of success include: chemotherapeutic agents such as 5-fluorouracilimiquimod, paclitaxel, trastuzumab, and docetaxel as well as aminolevulinic acid photodynamic therapy, laser ablation, and radiation therapy. Unfortunately, studies regarding the effectiveness of the above treatment options are limited to mainly case studies and small literature reviews with limited follow-up.[1][2][3][4]

Promising studies published recently include a study completed in Japan that reviewed the use of docetaxal as a first-line treatment in 13 individuals with EMPD with metastasis. 12 individuals were found to have a response to treatment with a disease control rate estimated to be 83%. Another promising study reviewed the use of imiquimod in 10 cases of EMPD of the vulva. Complete clinical remission was reported in 9 individuals. The remaining individual had partial remission.[3][4]

There is an ongoing research study focused on evaluating the effectiveness of imiquimod in the treatment of EMPD. To learn more about this trial, please click on the following link through ClinicalTrials.gov
https://clinicaltrials.gov/ct2/results?term=extramammary+paget%27s+disease&recr
Last updated: 3/8/2016

References
  1. Extramammary Paget Disease. New Zealand Dermatological Society. September 2015; http://dermnetnz.org/site-age-specific/extra-mammary-paget.html.
  2. Sandu N, Schwartz RA. Extramammary Paget disease. Medscape Reference. October 3, 2014; http://emedicine.medscape.com/article/1100397-overview.
  3. Lauro Lourival Lopes Filho, Lauro Rodolpho Soares Lopes, Alexandre Osores Michalany, Ione Maria Ribeiro, Milvia M.S.S. Enokihara, Nobuo Matsunaga. Mammary and extramammary Paget's disease. Anais Brasileiros Dermatologia. Mar-Apr 2015; 90(2):225-231. http://www-ncbi-nlm-nih-gov.ezproxy.nihlibrary.nih.gov/pmc/articles/PMC4371672/.
  4. Koji Yoshino, Yasuhiro Fujisawa, Yoshio Kiyohara, Takafumi Kadono, Yozo Murata, Hisashi Uhara, Naohito Hatta, Hiroshi Uchi, Shigeto Matsushita, Tatsuya Takenouchi, Toshihiko Hayashi, Kuniaki Ohara. Usefulness of docetaxel as first-line chemotherapy for metastatic extramammary Paget's disease. The Journal of Dermatology. November 25, 2015; 42:1-5. http://www.ncbi.nlm.nih.gov/pubmed/26603144.
  5. Claudia Marchitelli, Maria Sol Peremateu, Maria Celeste Sluga, Maria Teresa Berasategui, Delia Graciela Lopez, Alejandra Wernicke, Andrea Velazco, Sebastian Gogorza. Treatment of Primary Vulvar Paget Disease With 5% Imiquimod Cream. Journal of Lower Genital Tract Disease. October 2014; 18(4):347-350. http://www.ncbi.nlm.nih.gov/pubmed/25259595.


Clinical Trials & Research for this Disease

  • ClinicalTrials.gov lists trials that are studying or have studied Paget disease, extramammary. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
Other Names for this Disease
  • Extramammary Paget disease
  • EMPD
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.