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Treatment-refractory extramammary Paget disease responsive to radiotherapy

Published:November 10, 2021DOI:https://doi.org/10.1016/j.ajog.2021.11.028
      A 66-year-old female with extramammary Paget disease of the mons, perineum, vulva, vagina, and lower abdomen, status postradical vulvectomy, multiple excisional procedures, laser treatments, and radical cystectomy for bladder carcinoma in situ, presented to the dermatology clinic. Examination revealed diffuse red erosions of the vulva extending into the introitus with overlying maceration and scattered pink plaques with overlying crust and scale of the mons pubis and the abdomen (Figure, A). Treatment with a mixture of 1:1 fluorouracil 5% cream with calcipotriene 0.005% cream to the right mons and the lower abdomen and imiquimod 5% cream to the left mons and the lower abdomen was begun but was discontinued secondary to irritation. A palliative course of radiation therapy (30Gy in 10 fx) followed by imiquimod resulted in skin and quality-of-life improvement. Radiation therapy should be considered in extramammary Paget disease when surgery is not possible. Examination 10 weeks after radiation therapy revealed significant erosion resolution (Figure, B).
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      FigureClinical images
      A, Before radiotherapy, examination revealed diffuse red erosions of the vulva extending into the introitus with overlying maceration and scattered pink plaques with overlying crust and scale of the mons pubis and abdomen. B, After radiotherapy, examination revealed significant resolution of erosions.
      Pyle. Extramammary Paget disease responsive to radiotherapy. Am J Obstet Gynecol 2021.
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