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线性硬斑病:成人和儿童硬斑病队列的临床特征、疾病进程和治疗。

Linear morphea: Clinical characteristics, disease course, and treatment of the Morphea in Adults and Children cohort.

机构信息

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas; Northeast Ohio Medical University, Rootstown, Ohio.

Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas.

出版信息

J Am Acad Dermatol. 2019 Jun;80(6):1664-1670.e1. doi: 10.1016/j.jaad.2019.01.050. Epub 2019 Apr 17.

DOI:10.1016/j.jaad.2019.01.050
PMID:31005342
Abstract

BACKGROUND

Prospective, longitudinal studies examining the features of linear morphea are limited.

OBJECTIVE

To utilize the Morphea in Adults and Children cohort to determine clinical characteristics, impact on life quality, and disease course of linear morphea in a prospective, longitudinal manner.

METHODS

Characteristics of linear morphea versus other subtypes were compared in a cross-sectional manner. Next, linear morphea participants were examined in depth over a 3-year period.

RESULTS

Linear morphea was the most common morphea subtype (50.1%, 291/581) in the cohort. Deep involvement was more common in linear (64.3%, 187/291) than other morphea subtypes. Linear morphea participants with deep involvement were more likely to have a limitation in range of motion (28.6%, 55/192) than those without (11.1%, 11/99, P < .001). Adult-onset disease occurred in 32.6% (95/291) of those with linear morphea. Frequency of deep involvement was similar between pediatric (66.8%, 131/196) and adult-onset linear morphea (58.9%, 56/95, P = .19). Quality of life and disease activity scores improved over time, while damage stabilized with treatment.

LIMITATIONS

Results of the study are associative, and the University of Texas Southwestern Medical Center is a tertiary referral center.

CONCLUSION

A substantial number of linear morphea patients have adult-onset disease. In all age groups, linear morphea with deep involvement was associated with functional limitations.

摘要

背景

前瞻性、纵向研究检查线状硬斑病的特征有限。

目的

利用成人和儿童硬斑病队列,以前瞻性、纵向的方式确定线状硬斑病的临床特征、对生活质量的影响和疾病过程。

方法

以横断面的方式比较线状硬斑病与其他亚型的特征。接下来,对线性硬斑病患者进行了为期 3 年的深入检查。

结果

线状硬斑病是队列中最常见的硬斑病亚型(50.1%,291/581)。深部受累在线状(64.3%,187/291)比其他硬斑病亚型更常见。深部受累的线状硬斑病患者比无深部受累的患者更有可能出现运动范围受限(28.6%,55/192)比那些没有深部受累的患者(11.1%,11/99,P<0.001)。线状硬斑病中有 32.6%(95/291)为成人发病。儿科(66.8%,131/196)和成人发病的线状硬斑病(58.9%,56/95,P=0.19)深部受累的频率相似。随着治疗的进行,生活质量和疾病活动评分有所改善,而损害则稳定下来。

局限性

研究结果是相关的,德克萨斯大学西南医学中心是一个三级转诊中心。

结论

相当数量的线状硬斑病患者有成人发病。在所有年龄组中,深部受累的线状硬斑病与功能受限有关。

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