Poikilodermatous mycosis fungoides: a study of its clinicopathological, immunophenotypic, and prognostic features.

ABSTRACT

BACKGROUND: Poikilodermatous mycosis fungoides (MF) is a variant of MF, and its clinicopathological, immunophenotypic, molecular, and prognostic features have not previously been defined in the literature. OBJECTIVE: The purpose of this study was to improve the data available for this variant of MF thus enabling clinicians to apply the appropriate treatment and follow-up. METHODS: In a retrospective single center study we evaluated the clinical, histopathological, immunohistochemical, and molecular characteristics of patients with predominant (>50%) poikilodermatous lesions of MF. RESULTS: In all, 49 patients were identified. The median age at diagnosis was 44 years (15-81 years). Of 49 patients, 43 (88%) had early stage disease (≤IIA) at diagnosis. No patients had stage IV disease at presentation. A frequent association was coexistence of lymphomatoid papulosis (9/49; 18%). Histopathology review showed a high number of cases with CD8(+) CD4(-) atypical lymphocytes (38%). After diagnosis most patients were treated with expectant or skin-directed therapy. Psoralen plus ultraviolet A therapy was most frequently used and had high response rates (83%). Five (10%) of 49 received systemic therapy. The mean follow-up was 11 years, 10 months (1->40 years). In all, 47 (96%) of 49 patients had stable disease and two (4%) of 49 had progressive disease. No patients died during follow-up. LIMITATIONS: As a tertiary center our patient cohort may be expected to have more advanced and aggressive disease. CONCLUSION: Poikilodermatous MF represents a distinct clinicopathological entity from classic patch/plaque MF. It presents at a younger age and is more frequently associated with lymphomatoid papulosis. There is an increased number of cases with predominantly CD8(+) CD4(-) atypical lymphocytes. Overall there is a good response to phototherapy and the overall prognosis appears favorable.