The different faces of mycosis fungoides: results of a single-center study.

Int J Dermatol

1st Department of Dermatology-Venereology, Faculty of Medicine, National and Kapodistrian University of Athens, Cutaneous Lymphoma Clinic, "A. Sygros" Hospital for Skin & Venereal Diseases, Athens, Greece.

Published: March 2020

Background: Mycosis fungoides (MF) accounts for the majority of cutaneous lymphomas. Apart from the predominant Alibert-Bazin type, several clinicopathological variants of diverse prevalence and biological behavior have been described. Data on clinical and epidemiological aspects of MF clinical subtypes are still weak.

Aim: To outline the clinical and epidemiological profile of the different MF types in a large volume of Greek patients.

Methods: Retrospective analysis of 688 MF cases treated in our lymphoma clinic. Epidemiological, clinical, pathological, and immunohistochemical data were retrieved.

Results: Six-hundred and thirty-six patients (416 males, 220 females) were included. The mean age at diagnosis was 60.2 years; the mean duration of disease prior to diagnosis was 63.2 months. Early-stage MF (I-IIA) involved 475 cases (74.7%). The prevalent type was classical MF (68.5%), followed by folliculotropic (17%), poikilodermic (5.5%), and psoriasiform (4.7%) MF. Atypical MF lesions as the sole manifestation of folliculotropic mycosis fungoides (FMF) - alopecia areata-like lesions (n = 10), keratosis pilaris-like lesions (n = 9) or acneiform rash (n = 4) - were also observed. Both poikilodermic and folliculotropic subtypes mainly involved younger patients. A significant diagnostic latency concerning poikilodermic and psoriasiform MF cases was recorded. Only 23 (3.3%) cases were of juvenile onset, with classical and poikilodermic MF equally affecting this age group, closely followed by FMF.

Conclusions: Our study presents the whole clinical-epidemiological spectrum of MF in a large Greek cohort. The high prevalence of atypical MF manifestations characterized by early onset and indolent clinical course stood out among our FMF sample.

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Source
http://dx.doi.org/10.1111/ijd.14735DOI Listing

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