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.
Hematopathology Department, "Evangelismos" General Hospital, Athens, Greece.
Int J Dermatol. 2020 Mar;59(3):314-320. doi: 10.1111/ijd.14735. Epub 2019 Nov 29.
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.
To outline the clinical and epidemiological profile of the different MF types in a large volume of Greek patients.
Retrospective analysis of 688 MF cases treated in our lymphoma clinic. Epidemiological, clinical, pathological, and immunohistochemical data were retrieved.
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.
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.
蕈样肉芽肿（MF）占皮肤淋巴瘤的大多数。除了主要的 Alibert-Bazin 型外，还描述了几种具有不同流行率和生物学行为的临床病理变异型。关于 MF 临床亚型的临床和流行病学方面的数据仍然薄弱。
在大量希腊患者中概述不同 MF 类型的临床和流行病学特征。
回顾性分析在我们淋巴瘤诊所治疗的 688 例 MF 病例。检索了流行病学、临床、病理和免疫组织化学数据。
纳入 636 例患者（416 名男性，220 名女性）。诊断时的平均年龄为 60.2 岁；诊断前疾病的平均持续时间为 63.2 个月。早期 MF（I-IIA）累及 475 例（74.7%）。常见类型为经典 MF（68.5%），其次是滤泡性（17%）、斑驳性（5.5%）和银屑病样 MF（4.7%）。蕈样肉芽肿的滤泡性 MF 唯一表现也观察到了非典型 MF 病变，如斑秃样病变（n=10）、毳毛角化病样病变（n=9）或痤疮样皮疹（n=4）。斑驳性和滤泡性亚型主要涉及年轻患者。记录到斑驳性和银屑病样 MF 病例的明显诊断潜伏期。只有 23 例（3.3%）为青少年发病，经典和斑驳性 MF 同样影响这一年龄组，紧随其后的是 FMF。
我们的研究在一个大型希腊队列中呈现了 MF 的整个临床-流行病学谱。在我们的 FMF 样本中，以早期发病和惰性临床过程为特征的非典型 MF 表现的高患病率引人注目。
