Unmasking Extramammary Paget Disease: A Case Report on the Importance of Biopsy in Persistent Dermatitis

Authors

  • Cintya Dipta Riswanto
  • Yuri Widia
  • Bagus Haryo Kusumaputra
  • Maylita Sari
  • Irmadita Citrashanty
  • M. Yulianto Listiawan

Keywords:

Extramammary Paget’s disease

Abstract

Introduction: Extramammary Paget’s disease (EMPD) is a very rare cutaneous neoplasm that occurs predominantly in locations with apocrine glands. In Asia, the incidence of EMPD is extremely low, ranging from 0.04 to 0.12 cases per 100,000 population. The clinical features of EMPD resemble chronic inflammation, making it difficult to distinguish from other skin disorders. Here, we present a rare case of EMPD in Indonesia.

Case report: A 67-year-old woman presented with a 5-year history of erythematous plaque over her vulva and pubic area. The cutaneous lesions were painful and progressed slowly. Lesions manifest as well-defined erythematous plaques with irregular margins, an overlying scale or crust, and hypopigmentation or hyperpigmentation. The patient had received topical treatment for one year, but the condition remained unchanged. Histopathologic findings showed infiltration of anaplastic cells with round, pleomorphic, and hyperchromatic nuclei. Immunohistochemical staining showed CK7+, CK20-, p63+, and GATA3+. The growth confines itself to the epidermis, without penetrating the basal membrane. Discussion: A comprehensive examination, including lymph node palpation, can diagnose EMPD, and a skin biopsy and histopathology confirm the diagnosis. Immunohistochemical staining is essential for ruling out other possible diagnoses. Surgery is the primary treatment for EMPD. Given the risk of recurrence and additional malignancies, a close follow-up is recommended for the first 5 years.

Conclusion: The diagnosis of EMPD is a challenge due to the nonspecific clinical appearance, leading to delayed therapy. A skin biopsy is recommended for chronic anogenital skin lesions that don’t respond to topical therapy.

 

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References

Kang S, Amagai M, Bruckner AL, Enk AH, Margolis DJ, McMichael AJ, et al. Fitzpatrick’s Dermatology. 9th ed. 2019. 1934–1941 p.

Ghazawi FM, Le M, Alakel A, Rahme E, Sasseville D, Litvinov IV. 125 The Epidemiology and Clinical Characteristics of Extramammary Paget Disease Patients in Canada and Assessing the Risk of Second Malignancies. J Invest Dermatol. 2019 Sep;139(9):S235.

Herrel LA, Weiss AD, Goodman M, Johnson T V., Osunkoya AO, Delman KA, et al. Extramammary Paget’s Disease in Males: Survival Outcomes in 495 Patients. Ann Surg Oncol. 2015 May 11;22(5):1625–30.

Yin S, Xu L, Wang S, Feng J, Liu L, Liu G, et al. Prevalence of extramammary Paget’s disease in urban China: a population-based study. Orphanet J Rare Dis. 2021 Dec 17;16(1):134.

Cheng PS, Lu CL, Cheng CL, Lai FJ. Significant male predisposition in extramammary Paget disease: a nationwide population-based study in Taiwan. Br J Dermatol. 2014 Jul;171(1):191–3.

Asel M, LeBoeuf NR. Extramammary Paget’s Disease. Hematol Oncol Clin North Am. 2019 Feb;33(1):73–85.

St. Claire K, Hoover A, Ashack K, Khachemoune A. Extramammary Paget disease. Dermatol Online J. 2019;25(4).

Stasenko M, Jayakumaran G, Cowan R, Broach V, Chi DS, Rossi A, et al. Genomic Alterations as Potential Therapeutic Targets in Extramammary Paget’s Disease of the Vulva. JCO Precis Oncol. 2020 Nov;(4):1054–60.

Ghazawi FM, Iga N, Tanaka R, Fujisawa Y, Yoshino K, Yamashita C, et al. Demographic and clinical characteristics of extramammary Paget’s disease patients in Japan from 2000 to 2019. J Eur Acad Dermatology Venereol. 2021 Feb 10;35(2).

Zhao M, Zhou L, Sun L, Song Y, Guo Y, Zhang X, et al. GATA3 is a sensitive marker for primary genital extramammary paget disease: an immunohistochemical study of 72 cases with comparison to gross cystic disease fluid protein 15. Diagn Pathol. 2017 Dec 10;12(1):51.

Pappova T, Pec J, Kozarova A, Adamicova K. Extramammary Paget’s Disease Versus Lichen Sclerosus. Acta Medica Martiniana. 2016 Dec 1;16(3):43–6.

Lee J, Kim M, Moon J, Yoon H, Cho S, Park H. Pagetoid Bowen Disease Initially Misdiagnosed as Ectopic Extramammary Paget’s Disease. Ann Dermatol. 2018;30(2):218.

Ishizuki S, Nakamura Y. Extramammary Paget’s Disease: Diagnosis, Pathogenesis, and Treatment with Focus on Recent Developments. Curr Oncol. 2021 Aug 5;28(4):2969–86.

Kibbi N, Owen JL, Worley B, Wang JX, Harikumar V, Downing MB, et al. Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease. JAMA Oncol. 2022 Apr 1;8(4):618.

van der Linden M, Oonk MHM, van Doorn HC, Bulten J, van Dorst EBL, Fons G, et al. Vulvar Paget disease: A national retrospective cohort study. J Am Acad Dermatol. 2019 Oct;81(4):956–62.

Ito Y, Igawa S, Ohishi Y, Uehara J, Yamamoto AI, Iizuka H. Prognostic Indicators in 35 Patients with Extramammary Paget’s Disease. Dermatologic Surg. 2012 Dec;38(12):1938–44.

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Published

2025-04-23

How to Cite

1.
Riswanto CD, Widia Y, Kusumaputra BH, Sari M, Citrashanty I, Listiawan MY. Unmasking Extramammary Paget Disease: A Case Report on the Importance of Biopsy in Persistent Dermatitis. J Neonatal Surg [Internet]. 2025Apr.23 [cited 2025Sep.13];14(15S):2071-4. Available from: https://www.jneonatalsurg.com/index.php/jns/article/view/4428

Issue

Vol. 14 No. 15S (2025): Journal of Neonatal Surgery

Section

Original Article

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