VULVAR EKSTRAMAMMARİAN PAGET HASTALIĞI

Year 2014, Volume: 17 Issue: 1, 40 - 44, 01.01.2014

Mehmet Salih Gürel Esma İnan Yüksel Remzi Abalı Aslı Vefa Erdemir Erol Bozkurt

Abstract

Ekstramammarian Paget hastalığı (EMPH) başlıca anogenital bölge olmak üzere apokrin bezlerin yoğun olduğu bölgelerde ortaya
çıkan, nadir görülen bir intraepitelyal karsinomdur. Primer veya altta yatan malignitelere sekonder olarak ortaya çıkabilir. Genellikle
keskin sınırlı ekzematize veya erode plak şeklinde ortaya çıkar ve birçok farklı dermatozu taklit edebilir. Bu nedenle EMPH
kronik vulvar dermatozların ayırıcı tanıda daima hatırlanmalıdır. Bu yayında internal malignitenin eşlik etmediği, ilki hipopigmente
plak, diğeri vejetatif kitle şeklinde, iki EMPD olgusu sunulmuştur.

Keywords

Ekstramammarian Paget hastalığı, vulva

EXTRAMAMMARY PAGET’S DİSEASE OF THE VULVA

Abstract

Extramammary Paget’s disease (EMPD) is a rare intraepithelial carcinoma, that presents on apocrine-bearing skin areas, mainly on
anogenital region. EMPD may occur primary or secondary to an underlying malignancy. It usually manifests as well-demarcated,
eczematous or eroded plaques and can mimic many different dermatosis. Therefore, EMPD always be considered in the differantial
diagnosis of cronic vulvar dermatoses. In this report, two EMPD cases without association with internal malignancy are reported
;one with a hypopigmentated plaque and the other with vegetative appearence.

Keywords

Extramammary Paget’s disease, vulva

References

• 1. Chanda, J.J., Extramammary Paget’s disease: prognosis and relationship to internal malignancy. J Am Acad Dermatol, 1985. 13(6): p. 1009-14.
• 2. Lai, Y.L., et al., Penoscrotal extramammary Paget’s disease: a review of 33 cases in a 20-year experience. Plast Reconstr Surg, 2003. 112(4): p. 1017-23.
• 3. Lloyd, J. and A.M. Flanagan, Mammary and extramammary Paget’s disease. J Clin Pathol, 2000. 53(10): p. 742-9.
• 4. Teixeira, M.R., et al., Karyotypic fi ndings in tumors of the vulva and vagina. Cancer Genet Cytogenet, 1999. 111(1): p. 87-91.
• 5. Willman, J.H., L.E. Golitz, and J.E. Fitzpatrick, Vulvar clear cells of Toker: precursors of extramammary Paget’s disease. Am J Dermatopathol, 2005. 27(3): p. 185-8.
• 6. Belousova, I.E., et al., Vulvar toker cells: the long-awaited missing link: a proposal for an ori gin-based histogenetic classifi cation of extramammary paget disease. Am J Dermatopathol, 2006. 28(1): p. 84-6.
• 7. Sawada, Y., et al., Ectopic extramammary Pa get’s disease: case report and literature review. Acta Derm Venereol, 2010. 90(5): p. 502-5.
• 8. Shepherd, V., E.J . Davidson, and J. Davies-Humphreys, Extramammary Paget’s disease. BJOG, 2005. 112(3): p. 273-9.
• 9. Chiba, H., et al., Two cases o f vulval pigmented extramammary Paget’s disease: histochemical and immunohistochemical studies. Br J Dermatol, 2000. 142(6): p. 1190- 4.
• 10. Kakinuma, H., et al., A case of extramammary Paget’s disease with depigmented macules as the sole manifestation. Br J Dermatol, 1994. 130(1): p. 102-5.
• 11. Sawamura, D., et al., Depigmented macula as an initial manifestation of extramammary Paget’s disease. J Dermatol, 1996. 23(6): p. 429-30.
• 12. Chen, Y.H., T.W. Wong, and J.Y. Lee, Depigmented genital extramammary Paget’s dis ease: a possible histogenetic link to Toker’s clear cells and clear cell papulosis. J Cutan Pathol, 2001. 28(2): p. 1 05-8.
• 13. Van Hamme, C., et al., [Paget’s extramammary disease of the axillae and perineum]. Ann Dermatol Venereol, 2002. 129(5 Pt 1): p. 717-9.
• 14. Yang, C.C., J.Y. Lee , and T.W. Wong, Depigmented extramammary Paget’s disease. Br J Dermatol, 2004. 151(5): p. 1049-53.
• 15. Brainard, J.A. and W.R. Hart, Proliferative epiderma l lesions associated with anogenital Paget’s disease. Am J Surg Pathol, 2000. 24(4): p. 543-52.
• 16. Honda, Y. and K. Egawa, Extramammary Paget ’s disease not only mimicking but also accompanying condyloma acuminatum. A case report. Dermatology, 2005. 210(4): p. 315-8.
• 17. Fanning, J., et al., Paget’s disease of the vulva: prevalence of associated vu lvar adenocarcinoma, invasive Paget’s disease, and recurrence after surgical excision. Am J Obstet Gynecol, 1999. 180(1 Pt 1): p. 24-7.
• 18.Zollo, J.D. and N.C. Zeitouni, The Roswell Park Cancer Institute experience with extramammary Paget’s disease. Br J Dermato l, 2000. 142(1): p. 59-65.
• 19. O’Connor, W.J., et al., Comparison of mohs micrographic surgery and wide excision for extramammary Paget’s disease. Derma tol Surg, 2003. 29(7): p. 723-7.
• 20. Besa, P., et al., Extramammary Paget’s disease of the perineal skin: role of radiotherapy. Int J Radiat Oncol Biol Phys, 1992. 24(1): p . 73-8.
• 21. Brierley, J.D. and A.D. Stockdale, Radiotherapy: an effective treatment for extramammary Paget’s disease. Clin Oncol (R Coll Radiol), 1991. 3(1): p. 3-5.
• 22. Pang, J., et al., Extramammary Paget disease: re view of patients seen in a non-melanoma skin cancer clinic. Curr Oncol, 2010. 17(5): p. 43-5.
• 23. Green, J.S., N.M. Burkemper, and S.W. Fosko, Failure of extensive extramammary Paget disease of the inguinal area to clear with imiquimod cream, 5%: possible progression to invasive disease during therapy. Arch Der matol, 2011. 147(6): p. 704-8.
• 24. Karam, A., et al., HER-2/neu targeting for recurrent vulvar Paget’s disease A case report and literature review. Gynecol Oncol, 2008. 111(3): p. 568-71.
• 25. Delport, E.S., Extramammary Paget’s disease of the vulva: An annotated review of the current literature. Australa s J Dermatol, 2012.
• 26. Siesling, S., et al., Epidemiology and treatment of extramammary Paget disease in the Netherlands. Eur J Surg Oncol, 2007. 33(8): p. 951-5.
• 27. Shaco-Levy, R., et al., Paget disease of the vulva: a study of 56 cases. Eur J Obstet Gynecol Reprod Biol, 2010. 149(1): p. 86-91.