Kristy Lydia S. Bunagan, MD, DPDS, Janice Natasha C. Ng, MD, DPDS, Maricarr Pamela M. Lacuesta, MD, FPDS, and Mary Jo Kristine S. Bunagan, MD, FPDS Southern Philippines Medical Center, Davao City, Philippines Department of Dermatology
Corresponding Author: Kristy Lydia S. Bunagan
ABSTRACT Introduction: Extramammary Paget’s disease (EMPD) is a rare cutaneous slow growing tumor seen in areas rich in apocrine glands such as the anogenital region while ectopic EMPD is defined as EMPD arising on non-apocrine areas. The pigmented variant of EMPD is a very rare finding, with only a few reported cases, and can be misdiagnosed as melanoma.
Case report: We report a case of a 74-year-old woman who presented with a four-year history of pruritic, nonhealing erythematous plaques located on the right axilla and left lower abdomen. Histopathology revealed acanthotic epidermis with atypical keratinocytes that was negative for anti S-100 and Melan-A and was positive for carcinoembryonic antigen (CEA), cytokeratin (CK), CK 7 and epithelial membrane antigen (EMA). Patient was managed as pigmented and ectopic variant of extramammary Paget’s disease. Several tests and imaging were done to rule out malignancy. Wide excision with axillary node dissection, bilateral inguinal node dissection, frozen section biopsy and reconstruction using right pectoralis major musculocutaneous flap, split thickness skin graft with left inguinohypogastric drain were done by reconstructive surgery. Frozen section biopsy was negative for S-100, MELAN-A and HMB-45, ruling out malignant melanoma. Patient followed up every month for 6 months after the procedure with no recurrence and lymphadenopathy.
Conclusion: This case emphasizes that extramammary Paget’s disease is not a preventable disease and early diagnosis is the key to a favorable diagnosis. Any unilateral eczematous lesion that does not respond to an appropriate course of topical treatment warrants a skin biopsy.
extramammary Paget’s disease, melanoma, immunohistochemistry
1. Lloyd J, Flanagan AM. Mammary and extramammary Paget’s disease. J. Clin. Pathol. 2000; 53:742-9.
2. Sawada Y, Bito T, Kabashima R, et al. Ectopic extramammary Paget’s disease: case report and literature review. Acta Derm. Venereol. 2010; 90:502-5.
3. Hilliard NJ, Huang C, Andea A. Pigmented extramammary Paget’s disease of the axilla mimicking melanoma: case report and review of the literature. J. Cutan. Pathol. 2009; 36: 995-1000.
4. Onishi Y, Ohara K. Ectopic extramammary Paget’s disease affecting the upper abdomen. Br. J. Dermatol. 1996; 134:958-61.
5. Wagner G, Sachse MM. Extramammary Paget disease–clinical appearance, pathogenesis, management. J. Dtsch. Dermatol. Ges. 2011; 9:448-54.
6. Sardesai VR, Agarwal TD, Sakhalkar SP. Extra mammary Paget’s disease: a rare case report. Indian J. Dermatol. 2015; 60:105.
7. Ladak A, Bramley M, Titi S. Unilateral pigmented extramammary Paget’s disease of the axilla associated with a benign mole: a case study and a review of literature. Korean J. Pathol. 2014; 48:292-296.
8. Stefano C, Romano M, Carmine A. Primary bilateral extramammary Paget’s disease of the axillae: Another case of this strange disease. J. Cutan. Aesthet. Surg. 2014 ;7:131-134.
9. Vincent JG, Taube JM. Pigmented extramammary Paget disease of the abdomen: a potential mimicker of melanoma. Dermatol. Online J. 2011;17(8):13.
10. Kamer E, Peskersoy M, Unalp H, et al. Extramammary paget’s disease of the axilla. J. Breast Health. 2009; 5:47-49.
11. Wang EC, Kwah YC, Tan WP, et al. Extramammary Paget disease: immunohistochemistry is critical to distinguish potential mimickers. Dermatol. Online J. 2012;18(9):4.
12. Guerra R, Misra S. Management of extramammary Paget’s disease: a case report and review of the literature. Case Rep. Dermatol. Med. 2013; 2013:436390.
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