After ruling out systemic involvement, the patient was followed up for 2 years with no evidence of recurrence. We report this case to the best of our knowledge and discuss the literature about atypical clinical and histological presentations of MC.

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Sézary syndrome (SS) is a leukaemic variant of cutaneous T-cell lymphoma (CTCL). We report a patient with SS who developed granulomatous lesions. These lesions broke out during treatment with bexarotene when the disease had appeared to stabilize. After a partial clinical remission the disease showed rapid progression and finally led to the patient's death. This contradicts the initial assessment, which considered the granulomatous inflammation as a good prognostic factor in CTCL.

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