Pehlivan Ulutas Gizem, Atci Tugba, Baykal Can
Department of Dermatology and Venereology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
J Cutan Med Surg. 2025 May-Jun;29(3):243-249. doi: 10.1177/12034754241302822. Epub 2024 Dec 21.
Studies comparing the clinical and prognostic differences between pediatric- and adult-onset mycosis fungoides (MF) are limited.
To determine the impact of childhood-onset MF on clinical features and disease course in a large series.
Consecutive MF patients seen in a single centre between 2007 and 2021 were categorized into 3 groups: (i) MF patients diagnosed in the pediatric ages (≤18 years) (pediatric group), (ii) MF patients with disease onset in the pediatric period and diagnosis in adulthood (lately diagnosed pediatric-onset group), and (iii) MF patients with disease onset in the adulthood period (>18 years) (adult-onset group). Demographics, clinical features, stage at diagnosis, and progression to advanced stages during follow-up were recorded and compared.
Among 651 MF patients, 52 (8%) belonged to the pediatric group and 30 (4.6%) to the lately diagnosed pediatric-onset group. Pediatric MF presented with a combination of clinical variants in 48.1% of cases. While classical MF was the most common presentation in the 3 groups, hypopigmented (55.8%) and folliculotropic (17.3%) variants were more prevalent in the pediatric group compared to the others. Progression to the advanced stages was higher in the adult-onset MF group (12.1%) compared to the pediatric and lately diagnosed pediatric-onset MF groups (2.4%) ( .014).
This study highlights a relatively high prevalence of pediatric MF (8%) and lately diagnosed pediatric-onset MF (4.6%), often presenting with multiple and non-classical clinical variants. The higher rate of progression to advanced stages in the adult-onset group supports the relatively benign nature of MF in the pediatric-onset group.
比较儿童期和成人期蕈样肉芽肿(MF)临床及预后差异的研究有限。
在一个大型队列中确定儿童期起病的MF对临床特征和病程的影响。
将2007年至2021年在单一中心就诊的连续性MF患者分为3组:(i)在儿童期(≤18岁)确诊的MF患者(儿童组),(ii)在儿童期起病且成年期确诊的MF患者(迟发性儿童期起病组),以及(iii)在成年期(>18岁)起病的MF患者(成人期起病组)。记录并比较人口统计学、临床特征、诊断时的分期以及随访期间进展至晚期的情况。
在651例MF患者中,52例(8%)属于儿童组,30例(4.6%)属于迟发性儿童期起病组。48.1%的儿童期MF病例表现为多种临床亚型组合。虽然经典型MF是3组中最常见的表现形式,但与其他组相比,色素减退型(55.8%)和毛囊性(17.3%)亚型在儿童组中更为普遍。与儿童组和迟发性儿童期起病组(2.4%)相比,成人期起病组MF进展至晚期的比例更高(12.1%)(P<0.014)。
本研究强调儿童期MF(8%)和迟发性儿童期起病的MF(4.6%)的患病率相对较高,常表现为多种非经典临床亚型。成人期起病组进展至晚期的比例较高,支持儿童期起病组MF相对良性的性质。