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Perianal Paget’s disease: one century later and still a challenge
  1. Gonçalo João Guidi1,
  2. João Pinto de Sousa2,
  3. Rita Marques2 and
  4. Cátia Ferreira2
  1. 1General Surgery, Centro Hospitalar de Trás-os-Montes e Alto Douro EPE, Vila Real, Portugal
  2. 2Surgery, Tras-os-montes and Alto Douro Hospital Centre, Vila Real, Portugal
  1. Correspondence to Dr Gonçalo João Guidi; gjlima{at}


Perianal Paget disease (PPD) is a rare neoplastic condition defined by the presence of atypical Paget cells in the perianal skin, the aetiology of which remains largely unknown. It can be divided in primary forms, arising as an intraepithelial disease or manifestation of an underlying skin adenocarcinoma or secondary forms resulting from epidermotropic spread or metastasis of a concealed carcinoma. Indeed, because of its rarity, clear options regarding the treatment of these patients are yet to be clarified. A high level of suspicion is needed whenever dealing with any unhealed perianal skin lesions and, therefore, the need for close long-term follow-up must be highlighted. Herein, two cases of PPD, one primary and another secondary, treated at the same institution, are presented in an attempt to document the involved complexity and to bring further insight into the understanding of this entity.

  • surgical oncology
  • general surgery
  • gastrointestinal surgery

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  • Contributors GJG was mainly responsible for writing the article, was one of the surgeons involved in the surgery of case 1 and was responsible for the patient of case 1 during hospital stay. JPdS contributed greatly to the development of the article and was responsible for revising the manuscript critically for important intellectual content. CF was the main surgeon of case 1, helped in writing the article and was responsible for contributing with clinical data of the patient and images of the surgery. RM is the responsible surgeon of the patient in case 2, provided all the clinical data of the patient, helped greatly in writing the article and is responsible for the follow-up of the patient.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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