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获得性皮肤松弛症(全身性弹性组织离解):光镜及电镜研究

Acquired cutis laxa (generalized elastolysis): light and electron microscopic studies.

作者信息

Nanko H, Jepsen L V, Zachariae H, Søgaard H

出版信息

Acta Derm Venereol. 1979;59(4):315-24.

PMID:92145
Abstract

The case is described of a 44-year-old man with acquired cutis laxa. The primary clinical manifestation was an erythema of the chest. Gradually there developed persistent circumscribed lesions in other areas of the skin, spreading into large erythematous plaques with loose skin. Slight restrictive ventilatory insufficiency, elongation of the vocal cord and diverticulosis of the colon were also found, indicating a chronic and widespread disorder. Skin biopsies showed diminution and degeneration of the elastic fibres. The light-microscopic features were subdivided in relation to the various skin manifestations. Ultrastructure of the skin showed various amounts of electron-dense, amorphous material and loosely bound microfibrils in and around elastic and collagen fibres as well as some microfibrils with knobs in the interfibrouer, were found normal. It is suggested that the first changes in the elastic fibres induced an inflammatory response and mild mucinous changes of the collagen fibres. Furthermore elastic fibres seemed to be replaced by newly formed collagen.

摘要

本文描述了一名44岁患有获得性皮肤松弛症的男性病例。主要临床表现为胸部红斑。逐渐地,皮肤其他部位出现了持续性局限性病变,发展为大片伴有皮肤松弛的红斑性斑块。还发现有轻度限制性通气功能不全、声带延长和结肠憩室病,提示存在一种慢性广泛性疾病。皮肤活检显示弹性纤维减少和变性。根据各种皮肤表现对光镜特征进行了细分。皮肤超微结构显示,在弹性纤维和胶原纤维内及周围有不同数量的电子致密无定形物质和松散结合的微原纤维,以及纤维间一些带有小结的微原纤维,而其他结构正常。提示弹性纤维的最初变化引发了炎症反应和胶原纤维的轻度黏液样改变。此外,弹性纤维似乎被新形成的胶原所替代。

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Acquired cutis laxa (generalized elastolysis): light and electron microscopic studies.获得性皮肤松弛症(全身性弹性组织离解):光镜及电镜研究
Acta Derm Venereol. 1979;59(4):315-24.
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[Late-onset cutis laxa (generalized elastolysis)].迟发性皮肤松弛症(全身性弹性组织离解)
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Ultrastructural demonstration of a relationship between acquired cutis laxa and monoclonal gammopathy.获得性皮肤松弛症与单克隆丙种球蛋白血症之间关系的超微结构显示。
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引用本文的文献

1
Cutis laxa: a report of two interesting cases.皮肤松弛症:两例有趣病例报告。
Indian J Dermatol. 2013 Jul;58(4):328. doi: 10.4103/0019-5154.113986.
2
Marshall's syndrome.马歇尔综合征。
An Bras Dermatol. 2013 Mar-Apr;88(2):279-82. doi: 10.1590/S0365-05962013000200020.
3
Cutaneous manifestations in celiac disease.乳糜泻的皮肤表现
World J Gastroenterol. 2006 Feb 14;12(6):843-52. doi: 10.3748/wjg.v12.i6.843.
4
Congenital cutis laxa with a dominant inheritance and early onset emphysema.具有显性遗传和早发性肺气肿的先天性皮肤松弛症。
Thorax. 1994 Aug;49(8):836-7. doi: 10.1136/thx.49.8.836.