Extramammary Paget’s disease of the groin treated with topical imiquimod 5% cream
Keywords:
Extramammary Paget's disease, groin, imiquimod.Abstract
Paget's disease is an intraepithelial adenocarcinoma, which appears on the nipple-areola complex or, in its extramammary form, on anatomical regions rich in apocrine glands. The exclusive location in the groin area, without compromising other sites, is rare and difficult to diagnose clinically. We present a case of a 72-year-old man with a localized lesion on his right groin of unknown evolution time, a history of Chagas’ disease, high blood pressure, two cerebrovascular accidents and anticoagulation with warfarin, with a confirmed diagnosis of extramammary Paget's disease. We ruled out the presence of extracutaneous neoplasm and he was treated with imiquimod 5% cream during 24 weeks, showing improvement and without recurrences of the lesion after a 2-year follow-up period. The isolated localization of Paget's disease in the groin is extremely rare, having clinical manifestations that can be easily confused with other dermatoses, which delays diagnosis and treatment. Topical imiquimod is a safe and effective alternative when surgery is contraindicated or refused by the patient.
Author Biographies
Camilo Andrés Morales
Médico dermatólogo, Oficina de Docencia e Investigación, Centro Dermatológico Federico Lleras Acosta, E.S.E., Bogotá, D.C., Colombia
Astrid Lorena Ruiz
Médica, residente de tercer año de Dermatología, Fundación Universitaria Sanitas, Bogotá, D.C., Colombia; Centro Dermatológico Federico Lleras Acosta, E.S.E., Bogotá, D.C., Colombia
References
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2. Chanda JJ. Extramammary Paget's disease: Prognosis and relationship to internal malignancy. J Am Acad Dermatol. 1985;13:1009-114.
3. Lloyd J, Flanagan A. Mammary and extramammary Paget's disease. J Clin Pathol. 2000;53:742-9.
4. Rao VR, Henry DH. Extramammary Paget's disease. Commun Oncol. 2004;1:109-15.
5. González BM, Bort GP. La enfermedad de Paget extramamaria. Piel. 2006;21:332-5.
6. Zampogna JC, Flowers FP, Roth WI, Hassenein AM. Treatment of primary limited cutaneous extramammary Paget's disease with topical imiquimod monotherapy: Two case reports. J Am Acad Dermatol. 2002;47:S229-35.
7. Yugueros P, Keeney GL, Bite U. Paget's disease of the groin: Report of seven cases. Plast Reconstr Surg. 1997;100:336-9.
8. Liegl B, Leibl S, Gogg-Kamerer M, Tessaro B, Horn L, Moinfar F. Mammary and extramammary Paget's disease: An immunohistochemical study of 83 cases. Histopathology. 2007;50:439-47.
9. Díaz de Leon ED, Carcangiu ML, Prieto VG, McCue PA, Burchette JL, To G, et al. Extramammary Paget disease is characterized by the consistent lack of estrogen and progesterone receptors but frequently expresses androgen receptor. Am J Clin Pathol. 2000;113:572-5.
10. Dionyssopoulos A, Mandekou-Lefaki I, Vakirlis E, Manoliadis I, Nikolis A, Dionyssopoulou E. Extramammary Paget’s disease: Isolated localization on the groin –wide local excision with immediate reconstruction. Ann Surg Oncol. 2006;13:1235-40.
11. Lee SJ, Choe YS, Jung HD, Ahn SK, Cha YC, Cho KH, et al. A multicenter study on extramammary Paget’s disease in Korea. Int J Dermatol. 2011;50:508-15.
12. Hamm H, Vroom TM, Czarnetzki BM. Extramammary Paget's cells: Further evidence of sweat gland derivation. J Am Acad Dermatol. 1986;15:1275-81.
13. Ariza SA, Rueda X, González S, Rolon M. Enfermedad de Paget extramamaria múltiple. Rev Asoc Col Dermatol. 2008;16:165-7.
14. Haberman H, Goodall J, Llewellyn M. Extramammary Paget's disease. CMA Journal. 1978;118:161-2.
15. Sawada Y, Bito T, Kabashima R, Yoshiki R, Hino R, Nakamura M, et al. Ectopic extramammary Paget's disease: Case report and literature review. Acta Derm Venereol. 2010;90:502-5.
16. Tanaka VDA, Sanches JA, Torezan L, Niwa AB, Festa Neto C. Mammary and extramammary Paget's disease: A study of 14 cases and the associated therapeutic difficulties. Clinics. 2009;64:599-606.
17. Ye J, Rhew DC, Yip F, Edelstein L. Extramammary Paget's disease resistant to surgery and imiquimod monotherapy but responsive to imiquimod combination topical chemotherapy with 5-fluorouracil and retinoic acid: A case report. Cutis. 2006;77:245-50.
18. Lam C, Funaro D. Extramammary Paget's disease: Summary of current knowledge. Dermatol Clin. 2010;28:807-26.
19. Shiomi T, Yoshida Y, Shomori K, Yamamoto O, Ito H. Extramammary Paget’s disease: Evaluation of the histopathological patterns of Paget cell proliferation in the epidermis. J Dermatol. 2011;38:1054-7.
20. Ohnishi T, Watanabe S. The use of cytokeratins 7 and 20 in he diagnosis of primary and secondary extramammary Paget’s disease. Br J Dermatol. 2001;142:243-7.
21. Perrotto J, Abbott JJ, Ceilley RI, Ahmed I. The role of immunohistochemistry in discriminating primary from secondary extramammary Paget disease. Am J Dermatopathol. 2010;32:137-43.
22. Kohler S, Smoller BR. Gross cystic disease fluid protein-15 reactivity in extramammary Paget's disease with and without associated internal malignancy. Am J Dermatopathol. 1996;18:118-23.
23. Kondo Y, Kashima K, Daa T, Fujiwara S, Nakayama I, Yokoyama S. The ectopic expression of gastric mucin in extramammary and mammary Paget's disease. A J Surg Pathol. 2002;26:617-23.
24. Helm KF, Goellner JR, Peters MS. Immunohistochemical stains in extramammary Paget's disease. Am J Dermatopathol. 1992;14:402-7.
25. Kuan S, Montag AG, Hart J, Krausz T, Recant W. Differential expression of mucin genes in mammary and extramammary Paget's disease. Am J Surg Pathol. 2001;25:1469-77.
26. Hendi A, Broadland DG, Zitelli JA. Extramammary Paget´s disease: Surgical treatment with Mohs micrographic surgery. J. Am Acad Dermatol. 2004;51:767-73.
27. Green JS, Burkemper NM, Fosko SW. Failure of extensive extramammary Paget disease of the inguinal area to clear with imiquimod cream, 5%: Possible progression to invasive disease during therapy. Arch Dermatol. 2011;147:704-8.
28. Luyten A, Sörgel P, Clad A, Gieseking F, Maass-Poppenhusen K, Lellé RJ, et al. Treatment of extramammary Paget disease of the vulva with imiquimod: A retrospective, multicenter study by the German Colposcopy Network. J Am Acad Dermatol. 2014;70:644-50.
2. Chanda JJ. Extramammary Paget's disease: Prognosis and relationship to internal malignancy. J Am Acad Dermatol. 1985;13:1009-114.
3. Lloyd J, Flanagan A. Mammary and extramammary Paget's disease. J Clin Pathol. 2000;53:742-9.
4. Rao VR, Henry DH. Extramammary Paget's disease. Commun Oncol. 2004;1:109-15.
5. González BM, Bort GP. La enfermedad de Paget extramamaria. Piel. 2006;21:332-5.
6. Zampogna JC, Flowers FP, Roth WI, Hassenein AM. Treatment of primary limited cutaneous extramammary Paget's disease with topical imiquimod monotherapy: Two case reports. J Am Acad Dermatol. 2002;47:S229-35.
7. Yugueros P, Keeney GL, Bite U. Paget's disease of the groin: Report of seven cases. Plast Reconstr Surg. 1997;100:336-9.
8. Liegl B, Leibl S, Gogg-Kamerer M, Tessaro B, Horn L, Moinfar F. Mammary and extramammary Paget's disease: An immunohistochemical study of 83 cases. Histopathology. 2007;50:439-47.
9. Díaz de Leon ED, Carcangiu ML, Prieto VG, McCue PA, Burchette JL, To G, et al. Extramammary Paget disease is characterized by the consistent lack of estrogen and progesterone receptors but frequently expresses androgen receptor. Am J Clin Pathol. 2000;113:572-5.
10. Dionyssopoulos A, Mandekou-Lefaki I, Vakirlis E, Manoliadis I, Nikolis A, Dionyssopoulou E. Extramammary Paget’s disease: Isolated localization on the groin –wide local excision with immediate reconstruction. Ann Surg Oncol. 2006;13:1235-40.
11. Lee SJ, Choe YS, Jung HD, Ahn SK, Cha YC, Cho KH, et al. A multicenter study on extramammary Paget’s disease in Korea. Int J Dermatol. 2011;50:508-15.
12. Hamm H, Vroom TM, Czarnetzki BM. Extramammary Paget's cells: Further evidence of sweat gland derivation. J Am Acad Dermatol. 1986;15:1275-81.
13. Ariza SA, Rueda X, González S, Rolon M. Enfermedad de Paget extramamaria múltiple. Rev Asoc Col Dermatol. 2008;16:165-7.
14. Haberman H, Goodall J, Llewellyn M. Extramammary Paget's disease. CMA Journal. 1978;118:161-2.
15. Sawada Y, Bito T, Kabashima R, Yoshiki R, Hino R, Nakamura M, et al. Ectopic extramammary Paget's disease: Case report and literature review. Acta Derm Venereol. 2010;90:502-5.
16. Tanaka VDA, Sanches JA, Torezan L, Niwa AB, Festa Neto C. Mammary and extramammary Paget's disease: A study of 14 cases and the associated therapeutic difficulties. Clinics. 2009;64:599-606.
17. Ye J, Rhew DC, Yip F, Edelstein L. Extramammary Paget's disease resistant to surgery and imiquimod monotherapy but responsive to imiquimod combination topical chemotherapy with 5-fluorouracil and retinoic acid: A case report. Cutis. 2006;77:245-50.
18. Lam C, Funaro D. Extramammary Paget's disease: Summary of current knowledge. Dermatol Clin. 2010;28:807-26.
19. Shiomi T, Yoshida Y, Shomori K, Yamamoto O, Ito H. Extramammary Paget’s disease: Evaluation of the histopathological patterns of Paget cell proliferation in the epidermis. J Dermatol. 2011;38:1054-7.
20. Ohnishi T, Watanabe S. The use of cytokeratins 7 and 20 in he diagnosis of primary and secondary extramammary Paget’s disease. Br J Dermatol. 2001;142:243-7.
21. Perrotto J, Abbott JJ, Ceilley RI, Ahmed I. The role of immunohistochemistry in discriminating primary from secondary extramammary Paget disease. Am J Dermatopathol. 2010;32:137-43.
22. Kohler S, Smoller BR. Gross cystic disease fluid protein-15 reactivity in extramammary Paget's disease with and without associated internal malignancy. Am J Dermatopathol. 1996;18:118-23.
23. Kondo Y, Kashima K, Daa T, Fujiwara S, Nakayama I, Yokoyama S. The ectopic expression of gastric mucin in extramammary and mammary Paget's disease. A J Surg Pathol. 2002;26:617-23.
24. Helm KF, Goellner JR, Peters MS. Immunohistochemical stains in extramammary Paget's disease. Am J Dermatopathol. 1992;14:402-7.
25. Kuan S, Montag AG, Hart J, Krausz T, Recant W. Differential expression of mucin genes in mammary and extramammary Paget's disease. Am J Surg Pathol. 2001;25:1469-77.
26. Hendi A, Broadland DG, Zitelli JA. Extramammary Paget´s disease: Surgical treatment with Mohs micrographic surgery. J. Am Acad Dermatol. 2004;51:767-73.
27. Green JS, Burkemper NM, Fosko SW. Failure of extensive extramammary Paget disease of the inguinal area to clear with imiquimod cream, 5%: Possible progression to invasive disease during therapy. Arch Dermatol. 2011;147:704-8.
28. Luyten A, Sörgel P, Clad A, Gieseking F, Maass-Poppenhusen K, Lellé RJ, et al. Treatment of extramammary Paget disease of the vulva with imiquimod: A retrospective, multicenter study by the German Colposcopy Network. J Am Acad Dermatol. 2014;70:644-50.
How to Cite
1.
Morales CA, Ruiz AL. Extramammary Paget’s disease of the groin treated with topical imiquimod 5% cream . rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2019 Aug. 9 [cited 2024 Jul. 3];22(3):222-8. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/1050
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Published
2019-08-09
How to Cite
1.
Morales CA, Ruiz AL. Extramammary Paget’s disease of the groin treated with topical imiquimod 5% cream . rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2019 Aug. 9 [cited 2024 Jul. 3];22(3):222-8. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/1050
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