Vol 7, No 2 (2021)
Review paper
Published online: 2021-06-16
Extramammary Paget’s disease: the utility of photodynamic therapy
Forum Dermatologicum 2021;7(2):50-55.
Abstract
Extramammary Paget’s disease (EMPD) is a rare intraepithelial neoplasm which accounts for about 1% of malignant tumors in the anogenital region. It is often multifocal. Clinically it presents as a delineated erythematous plaque with typical white scales and erosions. Due to the non-specific clinical picture, the EMPD should always be considered as the differential diagnosis of pruritic lesions located in areas rich in apocrine glands and resistant to topical therapy. The golden standard in the EMPD treatment is the complete removal of the lesion with a safety margin. However, in patients with large tumors, when the surgical intervention would be mutilating for the patient, photodynamic therapy, especially in combination with imiquimod, is a good alternative and improves the patient’s quality of life.
Keywords: Paget’s diseaseadenocarcinomaphotodynamic therapyimiquimod
References
- Kacerovska D, Prieto VG, Singh R. Extramammary Paget’s disease. In: Elder DE. ed. WHO Classification of Skin Tumours. World Health Organization 2018: 217–218.
- St Claire K, Hoover A, Ashack K, et al. Extramammary Paget disease. Dermatol Online J. 2019; 25(4).
- Merritt BG, Degesys CA, Brodland DG. Extramammary Paget Disease. Dermatol Clin. 2019; 37(3): 261–267.
- Benito L, Blanco V, Fernandez R, et al. Sequential use of topical imiquimod and photodynamic therapy in Extrammamary Paget’s disease: a case report and review of the literature. .
- Diatta B. Etiologic profile of breast dermatoses in Dakar: A prospective study on 125 cas. Our Dermatology Online. 2019; 10(3): 244–250.
- Qian YT, Ma DL. Extramammary Paget disease of the glans penis. CMAJ. 2018; 190(38): E1142.
- Zhao Y, Gong X, Li N, et al. Primary extramammary Paget's disease: a clinicopathological study of 28 cases. Int J Clin Exp Pathol. 2019; 12(9): 3426–3432.
- Leong JY, Chung PH. A primer on extramammary Paget's disease for the urologist. Transl Androl Urol. 2020; 9(1): 93–105.
- Yamamoto T. Epidermotropic pagetoid spread and squamous cell carcinoma in situ in the overlying epidermis of Merkel cell carcinoma. Our Dermatology Online. 2014; 5(2): 210–211.
- Li L, Wang C, Wei M, et al. Mohs micrographic surgery combined local flaps in treatment of EMPD: A retrospective case. Dermatol Ther. 2018; 31(5): e12663.
- Stewart CL, Novoa RA, Seykora YT. Tumors of the epidermal appendages. In: Elder DE. ed. Lever’s histopathology of the skin. Lippincott Williams&Wilkins 2015: 2196–2199.
- Shi L, Liu P, Liu J, et al. Application of 5‐aminolevulinic acid‐photodynamic therapy in common skin diseases. Translational Biophotonics. 2020; 2(1-2).
- Eftimie LG, Costache DO, Uscatu CD, et al. Non-melanoma skin cancer (NMSC): Extramammary Paget ’s disease. Int J Qual Health Care. 2020; 3: 184–189.
- Choi J, Zemek A, Lee GK, et al. Perianal Extramammary Paget's Disease: More Than Meets the Eye. Dig Dis Sci. 2018; 63(11): 2853–2857.
- Fernandez-Flores A, Eraña I, Cuevas J. "Extramammary-Type" Paget Disease of the Breast. Am J Dermatopathol. 2018; 40(10): 711–720.
- Shepherd V, Davidson EJ, Davies-Humphreys J. Extramammary Paget's disease. BJOG. 2005; 112(3): 273–279.
- Weyandt G, Breitkopf C, Werner RN, et al. German S1 guidelines for the diagnosis and treatment of perianal dermatitis (anal eczema). J Dtsch Dermatol Ges. 2020; 18(6): 648–657.
- Calonje E. Extramammary Paget disease. In: Griffiths C. ed. Rook’s Textbook of Dermatology, 8th edition, 2010. John Wiley&Sons 2016: 3838–3839.
- Willman JH, Golitz LE, Fitzpatrick JE. Vulvar clear cells of Toker: precursors of extramammary Paget's disease. Am J Dermatopathol. 2005; 27(3): 185–188.
- Jones ISC, Crandon A, Sanday K. Paget's disease of the vulva: Diagnosis and follow-up key to management; a retrospective study of 50 cases from Queensland. Gynecol Oncol. 2011; 122(1): 42–44.
- Liao X, Liu X, Fan X, et al. Perianal Paget's disease: a clinicopathological and immunohistochemical study of 13 cases. Diagn Pathol. 2020; 15(1): 29.
- Zollo JD, Zeitouni NC. The Roswell Park Cancer Institute experience with extramammary Paget's disease. Br J Dermatol. 2000; 142(1): 59–65.
- Weng S, Zhu N, Li D, et al. Clinical Characteristics, Treatment, and Prognostic Factors of Patients With Primary Extramammary Paget's Disease (EMPD): A Retrospective Analysis of 44 Patients From a Single Center and an Analysis of Data From the Surveillance, Epidemiology, and End Results (SEER) Database. Front Oncol. 2020; 10: 1114.
- Lam C, Funaro D. Extramammary Paget's disease: Summary of current knowledge. Dermatol Clin. 2010; 28(4): 807–826.
- Morris CR, Hurst EA. Extramammary Paget Disease: A Review of the Literature-Part I: History, Epidemiology, Pathogenesis, Presentation, Histopathology, and Diagnostic Work-up. Dermatol Surg. 2020; 46(2): 151–158.
- Karam A, Dorigo O. Increased risk and pattern of secondary malignancies in patients with invasive extramammary Paget disease. Br J Dermatol. 2014; 170(3): 661–671.
- Karam A, Dorigo O. Treatment outcomes in a large cohort of patients with invasive Extramammary Paget's disease. Gynecol Oncol. 2012; 125(2): 346–351.
- Lee GC, Kunitake H, Stafford C, et al. High Risk of Proximal and Local Neoplasms in 2206 Patients With Anogenital Extramammary Paget's Disease. Dis Colon Rectum. 2019; 62(11): 1283–1293.
- Hutchings D, Windon A, Assarzadegan N, et al. Perianal Paget's disease as spread from non-invasive colorectal adenomas. Histopathology. 2021; 78(2): 276–280.
- Long B, Schmitt AR, Weaver AL, et al. A matter of margins: Surgical and pathologic risk factors for recurrence in extramammary Paget's disease. Gynecol Oncol. 2017; 147(2): 358–363.
- Asel M, LeBoeuf NR. Extramammary Paget's Disease. Hematol Oncol Clin North Am. 2019; 33(1): 73–85.
- Snast I, Sharon E, Kaftory R, et al. Nonsurgical Treatments for Extramammary Paget Disease: A Systematic Review and Meta-Analysis. Dermatology. 2020; 236(6): 493–499.
- Phyo AK, Mun KS, Kwan KC, et al. Genitourinary extramammary Paget's disease: review and outcome in a multidisciplinary setting. Int J Clin Exp Pathol. 2020; 13: 2369–2376.
- Yoshihara Y, Itoh M, Nobeyama Y, et al. A Case of Advanced Extramammary Paget's Disease Successfully Controlled by Monthly but Not Weekly Docetaxel Chemotherapy. Case Rep Oncol. 2016; 9(1): 6–10.
- Zhang X, Jin W, Zhu H, et al. Extramammary Paget's Disease in Two Brothers. Indian J Dermatol. 2015; 60(4): 423.
- Dogan A, Hilal Z, Krentel H, et al. Paget's Disease of the Vulva Treated with Imiquimod: Case Report and Systematic Review of the Literature. Gynecol Obstet Invest. 2017; 82(1): 1–7.
- Rioli DI, Samimi M, Beneton N, et al. Efficacy and tolerance of photodynamic therapy for vulvar Paget's disease: a multicentric retrospective study. Eur J Dermatol. 2018; 28(3): 351–355.
- Apalla Z, Lallas A, Tsorova A, et al. Complete response of extramammary Paget's disease with imiquimod and PDT: Report of two cases. Photodermatol Photoimmunol Photomed. 2018; 34(4): 273–275.
- Shim PJ, Zeitouni NC. Photodynamic therapy for extramammary Paget's disease: A systematic review of the literature. Photodiagnosis Photodyn Ther. 2020; 31: 101911.
