Case Report
 
The case of an extensive primary extramammary Paget's disease diagnosis and treatment
Apostolos Sarivalasis1, Cécile Triboulet2, Sandro Anchisi3
1Consultant, Department of medical oncology, Centre Hospitalier du Valais Romand (CHVR), Sion, Switzerland.
2Dermato-Pathologist. Department Dermatology and dermatopathology, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.
3Head Oncologist, Director. Department of medical oncology, Centre Hospitalier du Valais Romand (CHVR), Sion, Switzerland.

Article ID: Z01201701CR10744AS
doi:10.5348/ijcri-201705-CR-10744

Address correspondence to:
Dr. Apostolos Marinos Sarivalasis
MD, Centre Hospitalier du Valais Romand (CHVR)
Av. du Grand Champsec 80, 1951 Sion, Valais (VS)
Switzerland

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How to cite this article
Sarivalasis A, Triboulet C, Anchisi S. The case of an extensive primary extramammary Paget's disease diagnosis and treatment. Int J Case Rep Images 2017;8(1):46–50.


Abstract
Introduction: Extramammary Paget's disease (EMPD) is a rare neoplasm commonly related with underlying malignancy. It mainly affects intimate body areas and presents with eczema like lesions. Due to the rarity, the indolent natural history and the anatomical distribution of this disease, the diagnosis is difficult and often delayed. Treatment can be challenging especially since the majority of patient are elderly and frail.
Case Report: A 91-year-old male suffering from coronary disease and myelodysplastic syndrome presented with a slowly evolving scrotal eruption. A differential diagnosis was established and a punch biopsy was performed. An EMPD was diagnosed. The known relation of EMPDs with underlying malignancies mandated further complementary examinations. An underlying malignancy was excluded and a personalized treatment was undertaken.
Conclusion: Extramammary Paget's disease diagnosis and treatment are challenging. A comprehensive differential diagnosis is essential for integrating this rarity in the diagnostic assessment of persistent eczematous eruptions. A skin biopsy is paramount for diagnosis. A guided workout with multimodal examinations to rule out underlying malignancies is strongly recommended. The gold standard of EMPD treatment is surgery. Nevertheless, alternative less invasive treatments should be considered, depending on patient's comorbidities.

Keywords: Elderly, Extramammary Paget's disease, Skin rash


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Author Contributions
Apostolos Sarivalasis – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Cécile Triboulet – Substantial contributions to conception and design, Acquisition of data, Analysis and interpretation of data, Final approval of the version to be published
Sandro Anchisi – Substantial contributions to conception and design, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of submission
The corresponding author is the guarantor of submission.
Source of support
None
Conflict of interest
Authors declare no conflict of interest.
Copyright
© 2017 Apostolos Sarivalasis et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.