Extrammary Paget’s Disease of The Vulva and Pubis: A Rare Case Report

Authors

  • Naila Fauziah Al Muhtady Universitas Airlangga, Surabaya, Indonesia
  • Roy Novri Ramadhan Universitas Muhammadiyah Yogyakarta, Indonesia

Abstract

Background: Extramammary Paget’s disease is a rare neoplasm developing from the apocrine glands. Between the ages of 60 and 80, women had the highest incidence. The erythematous, well-defined lesions typically involve skin that bears apocrine glands and manifest as plaques.

Purpose: The purpose of this case report is to highlight a case of extramammary paget’s disease is still rare and with initial symptoms like herpes simplex.

Case: A 48-year-old woman came to the hospital with complaints of redness in her vagina spreads gradually and feel painful since 3 years ago. The patient also complained of a small lump in the vagina since 1 year ago that was getting bigger and felt painful. On genital area present erythematous macules and plaques, well demarcated, verrucous nodules measuring 2x1 cm, hard consistency and tenderness. Punch biopsy procedure was performed for histopatology and immunohistochemistry examination. The result were consistent with extramammary Paget’s disease. The patient was referred to surgical oncology.

Discussion: Extramammary Paget’s disease is a rare neoplasm, andnecessary to anamnese and examination to get appropriate therapy.

Conclusion: EMPD is confirmed by histology examination and immunohistochemistry. The immunohistochemistry used to differentiate primary and secondary EMPD is CK7 and CK20. A sensitive marker for primary EMPD is GATA-binding protein 3 (GATA3).

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Published

2026-05-19

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