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CC BY 4.0 · Journal of Coloproctology 2025; 45(04): s00451813735
DOI: 10.1055/s-0045-1813735
Case Report

Beyond the Ordinary: A Surgical Approach to Perianal Paget Disease

Autor*innen

  • Rita Ribeiro Dias 

    1   Department of General Surgery, São João University Medical Center, Porto, Portugal

  • André Gonçalves

    1   Department of General Surgery, São João University Medical Center, Porto, Portugal

  • Joana Costa 

    2   Department of Plastic Surgery, São João University Medical Center, Porto, Portugal

  • Pedro Organista Machado 

    2   Department of Plastic Surgery, São João University Medical Center, Porto, Portugal

  • Eliane Jaconiano 

    2   Department of Plastic Surgery, São João University Medical Center, Porto, Portugal

  • Daniela Marinheiro Silva 

    2   Department of Plastic Surgery, São João University Medical Center, Porto, Portugal

  • Miguel Machado 

    1   Department of General Surgery, São João University Medical Center, Porto, Portugal

  • Carolina Leal 

    3   Department of Pathology, São João University Medical Center, Porto, Portugal

  • Silvestre Carneiro 

    1   Department of General Surgery, São João University Medical Center, Porto, Portugal

Funding The authors declare that they did not received funding from agencies in the public, private, or nonprofit sectors to conduct the present study.
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Abstract

Perianal Paget Disease (PPD) is a rare form of extramammary Paget disease characterized by erythematous or eczematous plaques in the perianal region. Its rarity and resemblance to benign skin conditions often result in delayed diagnosis.

We present the case of a 56-year-old woman with a 4-year history of a refractory perianal lesion. A sharply demarcated erythematous plaque was identified, and biopsy confirmed PPD. Further workup excluded any underlying malignancy. Wide local excision with a 10-mm margin was performed, sparing the anal sphincters, followed by immediate reconstruction using a right gluteal fasciocutaneous V-Y advancement flap. The postoperative period was uneventful, and pathology confirmed negative margins.

Perianal Paget Disease requires differentiation from benign skin conditions and thorough evaluation for underlying malignancies. Many treatment options are available. However, surgery—more specifically wide local excision—remains the standard treatment. Despite the lack of standardized guidelines, long-term follow-up is essential due to the risk of recurrence and malignancy.

The present report highlights the importance of considering PPD in cases of chronic refractory perianal lesions and the need for developing guidelines for managing patients with PPD.

Keywords

perianal Paget disease - extramammary Paget disease - perianal neoplasms

Authors' Contributions

Rita Ribeiro Dias: conceptualization, data curation, formal analysis, investigation, methodology, project administration, visualization, writing– original draft André Gonçalves: conceptualization, data curation, formal analysis, investigation, methodology, project administration, visualization, writing– review & editing Joana Costa, Pedro Organista Machado, Eliane Jaconiano, Daniela Marinheiro Silva, Miguel Machado, Carolina Leal:Investigation, visualization Silvestre Carneiro: ressources, supervision.




Publikationsverlauf

Eingereicht: 05. Februar 2025

Angenommen: 11. August 2025

Artikel online veröffentlicht:
29. Dezember 2025

© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)

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Bibliographical Record
Rita Ribeiro Dias, André Gonçalves, Joana Costa, Pedro Organista Machado, Eliane Jaconiano, Daniela Marinheiro Silva, Miguel Machado, Carolina Leal, Silvestre Carneiro. Beyond the Ordinary: A Surgical Approach to Perianal Paget Disease. Journal of Coloproctology 2025; 45: s00451813735.
DOI: 10.1055/s-0045-1813735