幼年黄色肉芽肿三例临床分析并文献复习

Juvenile xanthogranuloma： 3 cases report and literature review

目的探讨幼年黄色肉芽肿的临床特点和治疗方法。方法对3例幼年黄色肉芽肿患儿的临床特点和治疗效果进行总结分析。结果幼年黄色肉芽肿临床多以皮肤包块、眼睛受累为表现。也可累及其他脏器，如下丘脑。诊断需和朗格汉斯细胞组织细胞增生症（LCH）相鉴别。单一皮肤受累可外科切除或观察，多系统受累可应用LCH治疗方案。结论幼年黄色肉芽肿为临床少见病，属于组织细胞疾病的一种。临床表现多样，对于多系统受累病例应用LCH方案有效。

Objective To report the clinical characteristics and treatment of 3 patients with juvenile xanthograuuloma（JXG）. Methods A retrospective review of the medical records of 3 patients with JXG. Results JXG was characterized by solitary or multiple yellowish cutaneous nodules, or eye involvement . It could also affect pituitary. JXG was easily misdiagnosed as Langerhans cell histiocytosis （LCH）. Treatment for JXG was surgical excision of a solitary skin lesion and some cases might be, spontaneous regression. In cases with multisystem involvement, chemotherapy regimens used to treat LCH may be effective. Conclusions JXG is one of the more common non-Langerhans histiocytic proliferations and is frequently seen in infants and children. LCH-like chemotherapy is effective for patients with symptomatic muhisystem JXG.