Mycosis fungoides: Review of epidemiological observations

Background: Mycosis fungoides (MF) is a chronic cutaneous T-cell lymphoma c haracterized by small cells with cerebriform nuclei that usually express a mature peripheral T-helper cell (CD4+) immunophenotype, Its evolution is ty pically quite slow, with years between the first manifestations and develop ment of advanced stages of disease. Objective: The purpose of the present p aper is to contribute to the material about MF already present in the liter ature, The review articles that have appeared to date fundamentally address the morphological characteristics, diagnostic criteria and treatment of th e disease; in contrast, the present study centers on the evolution of the i ncidence of MF and on the knowledge of the possible risk factors implicated in its development. Methods: Review of published papers about MF epidemiol ogy, Results: The evidence suggests that the incidence is increasing, but t his may be artifactual due to improved diagnostic techniques, The risk of M F is limited to gender and race, being higher in males and in blacks, Survi val is highly stage dependent, but 90% of patients survive 15 years with on ly 10% of cutaneous involvement, Few risk factors have been identified, but several studies have found an association with industrial exposure, partic ularly to oils. Conclusion: MF is a rare disease and its risk factors have not been studied in any great detail. A European case-control study in prog ress will substantially increase the evidence available and progress toward s identifying a prevention strategy, Copyright (C) 2000 S. Karger AG, Basel .