首页> 外文期刊>Journal of the European Academy of Dermatology and Venereology: JEADV >Childhood mycosis fungoides: A report of 20 cases from Turkey
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Childhood mycosis fungoides: A report of 20 cases from Turkey

机译:儿童真菌病真菌病:土耳其20例报告

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Background Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphomas in adults. In the recent past, several reports have focused on an increased prevalence of MF in children. Objective The present study was aimed to evaluate the clinical characteristics, treatment modalities and disease progression in childhood MF patients from Turkey. Methods In a retrospective analysis of 368 MF patients in a referral center at Istanbul, Turkey, 20 patients were diagnosed before the age of 18 years and were included in the study. Results Childhood cases constituted 5.4% of all MF patients. The age at the time of diagnosis ranged between 2-18 years with a mean of 9.20 ?? 4.52 and a median value of 9.50 years. The clinical presentation of MF consisted of solely patches in 60%, followed by plaques or plaques and patches in 40% of the patients. Hypopigmented lesions were seen in 45% and purpuric lesions in 30% of the patients. Four patients (20%) were diagnosed to have unilesional MF. The large majority of the patients (95%) had T1N0M 0 or T2N0M0 disease. Sixteen patients were followed between 1-13 years (median: 3.75 years). All the patients were treated with skin directed treatments including topical corticosteroids, topical bexarotene, topical carmustine, narrow band UVB and PUVA. None of the patients progressed to an advanced stage. After an initial clearance, 69.2% of the patients were observed to have recurrences. Conclusion In our experience, patches were more prevalent among childhood MF cases. Overrepresentation of hypopigmented and purpuric lesions was remarkable. Progression to an advanced stage was not seen. However, recurrences after discontinuation of therapy were common. ? 2011 European Academy of Dermatology and Venereology.
机译:背景蕈样真菌病(MF)是成人中最常见的皮肤T细胞淋巴瘤类型。在最近的过去,一些报道集中在儿童中MF的患病率增加。目的本研究旨在评估土耳其儿童MF患者的临床特征,治疗方式和疾病进展。方法对土耳其伊斯坦布尔转诊中心的368例MF患者进行回顾性分析,其中20例患者在18岁之前被诊断出并纳入研究。结果儿童病例占所有MF患者的5.4%。诊断时的年龄为2-18岁,平均为9.20 ??。 4.52,中值为9.50年。 MF的临床表现仅包括60%的斑块,然后是40%的患者的斑块或斑块和斑块。色素沉着病灶占45%,紫癜病灶占30%。四名患者(20%)被诊断患有单发性MF。绝大多数患者(95%)患有T1N0M 0或T2N0M0疾病。在1-13年之间(中位数:3.75年)对16例患者进行了随访。所有患者均接受皮肤定向治疗,包括局部糖皮质激素,局部贝沙罗汀,局部卡莫司汀,窄带UVB和PUVA。没有患者进展到晚期。初步清除后,观察到69.2%的患者复发。结论根据我们的经验,斑块在儿童MF病例中更为普遍。色素沉着不足和紫癜性病变的表现过多。未见进展到晚期。但是,停药后复发很常见。 ? 2011欧洲皮肤病与性病学会。

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