Case Report

Treatment of Extramammary Paget Disease with Topical Imiquimod Cream: Case Report and Literature Review

Authors: Philip R. Cohen, MD, Keith E. Schulze, MD, Jaime A. Tschen, MD, George W. Hetherington, MD, Bruce R. Nelson, MD

Abstract

Abstract:Extramammary Paget disease is an uncommon cutaneous neoplasm that presents as erythematous plaques most frequently located in the anogenital region. Management of patients with extramammary Paget disease involves evaluation of the individual for: (1) a disease-associated, unsuspected, visceral malignancy and (2) secondary adenocarcinoma in the underlying dermis or regional lymph nodes. Several modalities, each with variable effectiveness, are available to treat the cutaneous component of the disease: electrodesiccation and curettage, laser surgery, aminolevulinic acid photodynamic therapy, radiotherapy, topical chemotherapy, and wide surgical excision. However, surgical excision using the Mohs micrographic technique is currently the modality of choice for treating the cutaneous lesions of extramammary Paget disease. Recently, a topical imidazoquinoline immunomodulator that induces cytokine production and stimulates the innate and cellular immune responses-imiquimod cream-has been used for the management of primary or relapsing extramammary Paget disease. Complete healing, without recurrence, of extramammary Paget disease in patients whose cutaneous lesions were treated topically with imiquimod 5% cream was observed. We describe a man with suprapubic extramammary Paget disease whose condition was primary and limited to his skin. Biopsy-confirmed complete resolution of his disease was observed after the topical application of imiquimod 5% cream 3 times per week (on alternate days) for 16 weeks. After reviewing the published reports of other patients with extramammary Paget disease whose disease was successfully treated with imiquimod cream, we suggest that topical imiquimod 5% cream–at least 3 times per week (with 1–2 d of nontreatment in between) for a minimum of 8 to 16 weeks-be considered as an initial treatment for primary cutaneous extramammary Paget disease. Surgical excision or an alternative therapeutic modality is recommended for patients whose extramammary Paget disease persists or recurs after treatment with topical imiquimod.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Chaudhuri SP, Smoller BR. Extramammary Paget's disease: diagnosis and disease pattern. Cutis1992;50:195–196.
 
2. Heymann WR. Extramammary Paget's disease. Clin Dermatol 1993;11:83–87.
 
3. Sauder DN, Mofid MZ. Topical immunotherapy: what's new. Dermatol Clin 2005;23:245–258.
 
4. Hengge UR, Ruzicka T. Topical immunomodulation in dermatology: potential of toll-like receptor agonists. Dermatol Surg 2004;30:1101–1112.
 
5. Dahl MV. Imiquimod: a cytokine inducer. J Am Acad Dermatol 2002;47:S205–S208.
 
6. Miller R. Imiquimod stimulates innate and cell mediated immunity which controls virus infections and tumors. Int J Dermatol 2002;41(Suppl 1):3–6.
 
7. Navi D, Huntley A. Imiquimod 5 percent cream and the treatment of cutaneous malignancy. Dermatol Online J 2004;10(1):4.
 
8. 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(Suppl 4):S229–S235.
 
9. Berman B, Spencer J, Villa A, et al. Successful treatment of extramammary Paget's disease of the scrotum with imiquimod 5% cream. Clin Exp Dermatol 2003;28(Suppl 1):36–38.
 
10. Berman B, Poochareon VN, Villa AM. Novel dermatologic uses of the immune response modifier imiquimod 5% cream. Skin Therapy Lett 2002;7(9):1–6.
 
11. Berman B, Spencer J, Villa A, Poochareon V, Elgart G. Successful Treatment of Extramammary Paget's Disease of the Scrotum With Imiquimod 5% Cream. European Academy of Dermatology Meeting, October 2002, Prague, Czech Republic.
 
12. Spencer J, Berman B. Successful Treatment of Recurrent Extramammary Paget's Disease. Abstracts for the 3rd Combined Annual Meeting of the American Society of Dermatologic Surgery and American College of Mohs Micrographic Surgery and Cutaneous Oncology, October 30, 2002, p 190, Chicago, IL.
 
13. Hieken TJ, Lasser A. Successful nonoperative treatment of extensive extramammary Paget's disease: 2005 ASCO Annual Meeting Proceedings. J Clin Oncol 2005;23(16S):2584.
 
14. Qian Z, Zeitoun NC, Shieh S, et al. Successful treatment of extramammary Paget's disease with imiquimod. J Drugs Dermatol 2003;2:73–76.
 
15. Wasserman J. Presentation at the Annual Meeting of the Florida Society of Dermatologic Surgeons, 2004, Florida. Cited in McNamara D: Case of the month. Skin & Allergy News 2005;36(1):1,51.
 
16. Bamford J, Seidelmann S. Clinical and immunologic response of extramammary Paget's disease to imiquimod. [Abstract 888] J Invest Dermatol 2001;117:537–.
 
17. Wang LC, Blanchard A, Judge DE, et al. Successful treatment of recurrent extramammary Paget's disease of the vulva with topical imiquimod 5% cream. J Am Acad Dermatol 2003;49:769–772.
 
18. Guarner J, Cohen C, De Rose PB.. Histogenesis of extramammary and mammary Paget cells: an immunohistochemical study. Am J Dermatopathol 1989;11:313–318.
 
19. Smith AA, Silver TM. Zirconyl hematoxylin vs alcian blue staining in a case of extramammary Paget's disease. J Cutan Pathol 2003;30:401–404.
 
20. Hendi A, Brodland DG, Zitelli JA. Extramammary Paget's disease: surgical treatment with Mohs micrographic surgery. J Am Acad Dermatol 2004;51:767–773.
 
21. O'Connor, WJ, Lim KK, Zalla MJ, et al. Comparison of Mohs micrographic surgery and wide excision for extramammary Paget's disease. Dermatol Surg 2003;29:723–727.
 
22. Eliezri YD, Silvers DN, Horan DB. Role of preoperative topical 5-fluorouracil in preparation for Mohs micrographic surgery of extramammary Paget's disease. J Am Acad Dermatol 1987;17:497–505.
 
23. Chang YC, Madkan V, Cook-Norris R, et al. Current and potential uses of imiquimod. South Med J2005;98:914–920.
 
24. Wooten JM. Imiquimod [Editorial]. South Med J 2005;98:852–.