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线状硬皮病沿布氏线分布。

Linear morphoea follows Blaschko's lines.

作者信息

Weibel L, Harper J I

机构信息

Department of Paediatric Dermatology, Great Ormond Street Hospital for Children, London WC1N 3JH, UK.

出版信息

Br J Dermatol. 2008 Jul;159(1):175-81. doi: 10.1111/j.1365-2133.2008.08647.x. Epub 2008 Jul 1.

Abstract

BACKGROUND

The aetiology of morphoea (or localized scleroderma) remains unknown. It has previously been suggested that lesions of linear morphoea may follow Blaschko's lines and thus reflect an embryological development. However, the distribution of linear morphoea has never been accurately evaluated.

OBJECTIVES

We aimed to identify common patterns of clinical presentation in children with linear morphoea and to establish whether linear morphoea follows the lines of Blaschko.

METHODS

A retrospective chart review of 65 children with linear morphoea was performed. According to clinical photographs the skin lesions of these patients were plotted on to standardized head and body charts. With the aid of Adobe Illustrator a final figure was produced including an overlay of all individual lesions which was used for comparison with the published lines of Blaschko.

RESULTS

Thirty-four (53%) patients had the en coup de sabre subtype, 27 (41%) presented with linear morphoea on the trunk and/or limbs and four (6%) children had a combination of the two. In 55 (85%) children the skin lesions were confined to one side of the body, showing no preference for either left or right side. On comparing the overlays of all body and head lesions with the original lines of Blaschko there was an excellent correlation.

CONCLUSIONS

Our data indicate that linear morphoea follows the lines of Blaschko. We hypothesize that in patients with linear morphoea susceptible cells are present in a mosaic state and that exposure to some trigger factor may result in the development of this condition.

摘要

背景

硬斑病(或局限性硬皮病)的病因尚不清楚。此前有研究表明,线状硬斑病的皮损可能沿布氏线分布,从而反映胚胎发育过程。然而,线状硬斑病的分布情况从未得到准确评估。

目的

我们旨在确定线状硬斑病患儿的常见临床表现模式,并确定线状硬斑病是否沿布氏线分布。

方法

对65例线状硬斑病患儿进行回顾性病历审查。根据临床照片,将这些患者的皮肤损害绘制在标准化的头部和身体图表上。借助Adobe Illustrator软件生成最终图形,其中包括所有个体损害的叠加图,用于与已发表的布氏线进行比较。

结果

34例(53%)患者为剑伤型亚型,27例(41%)表现为躯干和/或四肢线状硬斑病,4例(6%)患儿两种情况均有。55例(85%)患儿的皮肤损害局限于身体一侧,左右侧无明显偏好。将所有身体和头部损害的叠加图与原始布氏线进行比较,发现相关性极佳。

结论

我们的数据表明,线状硬斑病沿布氏线分布。我们推测,在线状硬斑病患者中,易感细胞呈镶嵌状态,接触某些触发因素可能导致该病的发生。

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