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We present two cases of MF with clinical urticarial lesions and a striking blood involvement that responded to mogamulizumab treatment. Histopathologically, both cases had classic MF features and shared a peculiar immunophenotype, with positivity for CD25 and FOXP3. Differential diagnoses included urticarial lymphomatoid drug reactions and other lymphomas, like T\u2010cell prolymphocytic leukemia, atypical S\u00e9zary syndrome, or adult T\u2010cell lymphocytic leukemia. A low suspicion threshold is necessary for the diagnosis of atypical presentations of MF.<\/jats:p>","DOI":"10.1111\/cup.14642","type":"journal-article","created":{"date-parts":[[2024,5,21]],"date-time":"2024-05-21T05:20:16Z","timestamp":1716268816000},"page":"649-653","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Urticarial mycosis fungoides: A distinctive presentation with blood involvement and a peculiar immunophenotype"],"prefix":"10.1111","volume":"51","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0444-7419","authenticated-orcid":false,"given":"Juan","family":"Torre\u2010Castro","sequence":"first","affiliation":[{"name":"Dermatology Department Hospital Universitario Fundaci\u00f3n Jim\u00e9nez D\u00edaz, Universidad Aut\u00f3noma  Madrid 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