Department of dermatology, Rambam Health Care Campus, Haifa, Israel.
Acta Derm Venereol. 2023 Jul 14;103:adv6557. doi: 10.2340/actadv.v103.6557.
Mycosis fungoides is a rare cutaneous lymphoma in the paediatric population. The aim of this study was to examine the epidemiological, clinical, and histological characteristics, as well as the treatment modalities and response to therapy of paediatric patients with mycosis fungoides. This retrospective cohort study reviewed the records of 37 paediatric patients treated at Rambam Medical Center, Israel, between 2013 and 2021. Extracted data included epidemiology, clinical presentation, histological reports, infiltrate clonality status, treatment modalities and response to therapy. The mean follow-up period was 60 months. All patients were diagnosed with stage IA or IB disease. Folliculotropic mycosis fungoides was the most prevalent variant (49%). Most patients were treated with phototherapy (90%), with a response rate of 85%, and a complete response rate of 55% after the first course. There were no significant differences in response to phototherapy between the folliculotropic or other variants (p = 0.072). Similarly, delayed diagnosis, atopic diathesis, clonality, phototherapy type or number of treatments, were not associated with response to therapy, while protracted phototherapy was associated with prolonged remission. In conclusion, mycosis fungoides in the paediatric population is an indolent disease with a favourable prognosis and potentially prolonged response to phototherapy.
蕈样肉芽肿是儿童中罕见的皮肤淋巴瘤。本研究旨在研究蕈样肉芽肿患儿的流行病学、临床和组织学特征，以及治疗方式和治疗反应。本回顾性队列研究分析了 2013 年至 2021 年在以色列 Rambam 医疗中心治疗的 37 名儿科患者的记录。提取的数据包括流行病学、临床表现、组织学报告、浸润克隆性状态、治疗方式和治疗反应。平均随访时间为 60 个月。所有患者均诊断为 IA 期或 IB 期疾病。滤泡性蕈样肉芽肿是最常见的变异型（49%）。大多数患者接受光疗（90%），首次疗程后，缓解率为 85%，完全缓解率为 55%。滤泡性或其他变异型对光疗的反应无显著差异（p=0.072）。同样，延迟诊断、特应性素质、克隆性、光疗类型或治疗次数与治疗反应无关，而长时间光疗与缓解时间延长有关。总之，儿童蕈样肉芽肿是一种惰性疾病，预后良好，对光疗有潜在的长期反应。
