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青少年黄色肉芽肿:研究单发、多发及皮肤外表现。

Juvenile xanthogranulomas: Examining single, multiple, and extracutaneous presentations.

作者信息

So Naomi, Liu Regina, Hogeling Marcia

机构信息

David Geffen School of Medicine at UCLA, Los Angeles, California.

Division of Dermatology, UCLA Department of Medicine, Los Angeles, California.

出版信息

Pediatr Dermatol. 2020 Jul;37(4):637-644. doi: 10.1111/pde.14174. Epub 2020 May 28.

DOI:10.1111/pde.14174
PMID:32468628
Abstract

BACKGROUND

Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis characterized by yellowish papules in the skin. JXGs most often occur in infancy or early childhood and are typically solitary and asymptomatic, often regressing after several years. While JXGs predominantly occur on the skin, extracutaneous JXGs also exist.

AIMS

In this paper, we review the literature on single, multiple, and visceral JXGs and provide recommendations on monitoring and work-up.

MATERIALS & METHODS: A literature review was conducted with the PubMed database using selective search terms for single, multiple, ocular, and visceral lesions as well as NF1/JMML.

RESULTS / DISCUSSION: JXG is typically a self-limited disorder if lesions are cutaneous and singular. While rare, JXGs may manifest as multiple and extracutaneous lesions. Further screening and referral to specialists may be warranted in these cases based on age and extent of involvement.

CONCLUSION

Our review demonstrates common presentations of single, multiple, and extracutaneous lesions in addition to those that occur with NF1 and JMML. We suggest patients be evaluated on a case-by-case basis by a dermatologist and referred to specialists as appropriate.

摘要

背景

幼年性黄色肉芽肿(JXG)是一种非朗格汉斯细胞组织细胞增生症,其特征为皮肤出现淡黄色丘疹。JXG最常发生于婴儿期或幼儿期,通常为单发且无症状,常在数年后自行消退。虽然JXG主要发生在皮肤,但也存在皮肤外的JXG。

目的

在本文中,我们回顾了关于单发、多发和内脏型JXG的文献,并提供了监测和检查的建议。

材料与方法

使用针对单发、多发、眼部和内脏病变以及NF1/JMML的选择性检索词,在PubMed数据库中进行文献综述。

结果/讨论:如果病变为皮肤单发,JXG通常是一种自限性疾病。虽然罕见,但JXG可能表现为多发和皮肤外病变。在这些情况下,根据年龄和受累程度,可能需要进一步筛查并转诊给专科医生。

结论

我们的综述展示了单发、多发和皮肤外病变以及与NF1和JMML相关病变的常见表现。我们建议由皮肤科医生根据具体情况对患者进行评估,并在适当时转诊给专科医生。

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