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念珠状发:一项超微结构研究。

Monilethrix: an ultrastructural study.

作者信息

Ito M, Hashimoto K, Yorder F W

出版信息

J Cutan Pathol. 1984 Dec;11(6):513-21. doi: 10.1111/j.1600-0560.1984.tb00412.x.

DOI:10.1111/j.1600-0560.1984.tb00412.x
PMID:6520260
Abstract

Characteristic moniliform hairs of monilethrix were ultrastructurally examined. By scanning electron microscope, nodes and internodes were seen alternating on the affected hair; the nodes were normal in appearance and thickness, while the internodes were thin and showed ridges and flutes. By transmission electron microscope, the cross sections of the internodes revealed wrinkling of the hair cuticular cells and a reduced number of the cortical cells. Cross sections of the cortical cells per se showed a similar size and a normal keratin pattern in both nodes and internodes, compared with those of control hairs from normal individuals. From these findings, the internodes seemed to be the pathological portions of the moniliform hair, and such abnormal thinning of hair shaft might be caused by a periodical dysfunction of the hair matrix, especially in the hair cortex.

摘要

对毛发念珠状发特征性的串珠状毛发进行了超微结构检查。通过扫描电子显微镜观察,在患病毛发上可见结节和节间交替出现;结节外观和厚度正常,而节间较细,有嵴和沟。通过透射电子显微镜观察,节间的横截面显示毛表皮细胞起皱,皮质细胞数量减少。与正常个体的对照毛发相比,皮质细胞本身的横截面在结节和节间均显示出相似的大小和正常的角蛋白模式。从这些发现来看,节间似乎是串珠状毛发的病理部位,而这种毛干异常变细可能是由毛基质,尤其是毛皮质的周期性功能障碍引起的。

相似文献

1
Monilethrix: an ultrastructural study.念珠状发:一项超微结构研究。
J Cutan Pathol. 1984 Dec;11(6):513-21. doi: 10.1111/j.1600-0560.1984.tb00412.x.
2
Pathogenesis of monilethrix: computer stereography and electron microscopy.
J Invest Dermatol. 1990 Aug;95(2):186-94. doi: 10.1111/1523-1747.ep12477967.
3
Review and new case reports on scanning electron microscopy of pili annulati, Monilethrix and Trichothiodystrophy.毛发环状结构、念珠状发及毛发硫营养不良的扫描电子显微镜检查综述与新病例报告
Scanning Microsc. 1992 Jun;6(2):537-41.
4
Monilethrix: a clinicopathological illustration of a cortical defect.
Br J Dermatol. 1993 Mar;128(3):327-31. doi: 10.1111/j.1365-2133.1993.tb00179.x.
5
Monilethrix: an electron microscopic and electron histochemical study.念珠状发:一项电子显微镜及电子组织化学研究。
Br J Dermatol. 1981 Nov;105(5):529-41. doi: 10.1111/j.1365-2133.1981.tb00796.x.
6
Monilethrix. Comparative scanning electron microscopic study of the hair in one family.念珠状发。对一个家族毛发的比较扫描电子显微镜研究。
Dermatologica. 1982 Jan;164(1):1-9.
7
Pathogenesis of pili annulati.环状发的发病机制。
Arch Dermatol Res. 1988;280(5):308-18. doi: 10.1007/BF00440605.
8
Mutations in the hair cortex keratin hHb6 cause the inherited hair disease monilethrix.毛发皮质角蛋白hHb6中的突变会导致遗传性毛发疾病念珠状发。
Nat Genet. 1997 Aug;16(4):372-4. doi: 10.1038/ng0897-372.
9
Mutations in the desmoglein 4 gene are associated with monilethrix-like congenital hypotrichosis.桥粒芯糖蛋白4基因的突变与念珠状发样先天性少毛症相关。
J Invest Dermatol. 2006 Jun;126(6):1281-5. doi: 10.1038/sj.jid.5700113.
10
Hair shaft abnormalities: Part I.毛干异常:第一部分。
Australas J Dermatol. 1995 Nov;36(4):179-84; quiz 185-6. doi: 10.1111/j.1440-0960.1995.tb00969.x.

引用本文的文献

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Paediatric Hypotrichosis: A Clinical and Algorithmic Approach to Diagnosis.小儿毛发稀少症:诊断的临床及算法方法
Australas J Dermatol. 2025 May;66(3):e109-e119. doi: 10.1111/ajd.14429. Epub 2025 Feb 24.
2
An autosomal recessive mutation of DSG4 causes monilethrix through the ER stress response.DSG4的常染色体隐性突变通过内质网应激反应导致毛发干躁症。
J Invest Dermatol. 2015 May;135(5):1253-1260. doi: 10.1038/jid.2015.12. Epub 2015 Jan 23.
3
Pathogenesis of pili annulati.环状发的发病机制。
Arch Dermatol Res. 1988;280(5):308-18. doi: 10.1007/BF00440605.