Background: Paget�s disease is a rare skin disorder occurring in the breast (mammary) or in the groin, genital, perianal\r\nand axillary regions (extra-mammary). Typical treatment involves surgical excision, which in the case of extramammary\r\nPaget�s disease, can lead to significant morbidity. Photodynamic therapy (PDT) which uses a topical or\r\nintravenous photosensitizing agent that is activated by a light source to ablate abnormal tissue, offers a minimally\r\ninvasive alternative. The purpose of this study was to assess the effectiveness of photodynamic therapy in the\r\ntreatment of Paget�s disease.\r\nMethods: Following Cochrane guidelines, a comprehensive systematic review of all clinical studies and reports\r\nexamining the use of PDT for mammary and extra-mammary Paget�s disease was conducted. Study quality was\r\nassessed using the Oxford Levels of Evidence Scale.\r\nResults: 21 retrospective and 2 prospective non-comparative studies were identified and included in the review: 9\r\ncase reports with 1-2 patients and 14 case series with 1-16 patients. These reports totalled 99 patients with 133\r\nextra-mammary Paget�s lesions and 3 patients (with 3 lesions) with mammary Paget�s disease. Follow-up periods\r\nwere typically one year or less, with 77/133 extra-mammary lesions exhibiting complete response to PDT. One\r\nrecurrent mammary skin lesion and two mammary lesions treated concomitantly with surgery also exhibited\r\ncomplete responses.\r\nConclusions: Evidence of the effectiveness of PDT for Paget�s disease is promising, but limited. This may, in part,\r\nbe explained by the rarity of the condition, making controlled comparative clinical trials challenging.
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