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生长期松动毛发作为儿童遗传性脱发的一个原因。

Loose anagen hair as a cause of hereditary hair loss in children.

作者信息

Baden H P, Kvedar J C, Magro C M

机构信息

Department of Dermatology, Harvard Medical School, Massachusetts General Hospital, Boston.

出版信息

Arch Dermatol. 1992 Oct;128(10):1349-53.

PMID:1417022
Abstract

BACKGROUND AND DESIGN

The loose anagen hair syndrome is a recently described disorder with enhanced hair loss in which anagen hairs can be painlessly pulled from the scalp.

RESULTS

In three families, we have observed an autosomal dominant form of inheritance. We have confirmed that the extracted bulbs consist only of cortical cells and that the hair shafts have an abnormal shape. Using routine light and electron microscopy, we observed disadhesion between all layers of the follicle and within some layers and premature keratinization of the inner root sheath of the follicle. In addition, abnormal maturation of the various layers was observed at the electron microscopic level, including regions of the cortex that contained decreased numbers of filaments. The disorder appears to be cyclic, since relatively normal and markedly affected regions of the shaft can be observed within a single follicle.

CONCLUSIONS

Possible mechanisms responsible for the disease include abnormal intercellular signaling and disturbances of desmosomal components and/or cadherins.

摘要

背景与设计

松动生长期毛发综合征是一种最近描述的脱发增强的疾病,其中生长期毛发可从头皮无痛拔出。

结果

在三个家族中,我们观察到常染色体显性遗传形式。我们已证实拔出的毛球仅由皮质细胞组成,且毛干形状异常。使用常规光学显微镜和电子显微镜,我们观察到毛囊各层之间以及某些层内的分离,以及毛囊内根鞘的过早角化。此外,在电子显微镜水平观察到各层的异常成熟,包括皮质中细丝数量减少的区域。该疾病似乎是周期性的,因为在单个毛囊内可以观察到相对正常和明显受影响的毛干区域。

结论

该疾病可能的发病机制包括细胞间信号异常以及桥粒成分和/或钙黏蛋白的紊乱。

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