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局部使用雷帕霉素治疗表浅淋巴管畸形。

Use of topical rapamycin in the treatment of superficial lymphatic malformations.

机构信息

Dermatology Department, Hospital Costa del Sol, Marbella, Spain.

Dermatology Department, Hospital Costa del Sol, Marbella, Spain.

出版信息

J Am Acad Dermatol. 2019 Feb;80(2):508-515. doi: 10.1016/j.jaad.2018.09.050. Epub 2018 Oct 5.

Abstract

BACKGROUND

The superficial lymphatic component of vascular malformations poses a significant treatment challenge. It is responsible for the majority of symptoms presented, and to date, there is no consensus regarding treatment.

OBJECTIVE

To evaluate the effectiveness of topical rapamycin in treating superficial lymphatic malformations (LM).

METHODS

A case series study was performed of patients with superficial LM, treated with topical rapamycin. The clinical characteristics of patients and the concentration and application mode of the drug were recorded. The changes in the signs and symptoms observed and associated adverse effects were noted and analyzed.

RESULTS

The study population consisted of 11 patients of an average age of 10.5 years. All were treated with topical rapamycin: 6 patients with a 1% concentration, 1 with a 0.8% concentration, and 4 with a 0.4% concentration. Changes in the clinical appearance of the lesions were observed in all patients. The associated symptoms, present in 9 of 11 patients, improved in every case. The mean follow-up time was 16.1 months.

LIMITATIONS

This study is retrospective, with a small sample size and considerable heterogeneity of lesions and treatment approaches.

CONCLUSION

Treatment with topical rapamycin modifies the clinical appearance and alleviates symptoms of superficial LM.

摘要

背景

脉管畸形的浅表淋巴管成分是一个重大的治疗挑战。它是引起大多数症状的主要原因,目前对于治疗方法尚未达成共识。

目的

评估局部应用雷帕霉素治疗浅表淋巴管畸形(LM)的效果。

方法

对接受局部雷帕霉素治疗的浅表 LM 患者进行病例系列研究。记录患者的临床特征以及药物的浓度和应用方式。观察并分析症状和体征的变化以及相关不良反应。

结果

研究人群由 11 名平均年龄为 10.5 岁的患者组成。所有患者均接受局部雷帕霉素治疗:6 名患者使用 1%浓度,1 名患者使用 0.8%浓度,4 名患者使用 0.4%浓度。所有患者的病变临床外观均有变化。11 名患者中有 9 名存在相关症状,所有患者的症状均有改善。平均随访时间为 16.1 个月。

局限性

本研究为回顾性研究,样本量小,病变和治疗方法存在较大异质性。

结论

局部应用雷帕霉素可改善浅表 LM 的临床外观并缓解症状。

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