The case of an extensive primary extramammary Paget's diseas
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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.