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遗传性大疱性表皮松解症中水疱形成的超微结构:V. 局限性单纯性大疱性表皮松解症(Weber-Cockayne型)

Ultrastructure of blister formation in epidermolysis bullosa hereditaria: V. Epidermolysis bullosa simplex localisata type Weber-Cockayne.

作者信息

Haneke E, Anton-Lamprecht I

出版信息

J Invest Dermatol. 1982 Mar;78(3):219-23. doi: 10.1111/1523-1747.ep12506502.

DOI:10.1111/1523-1747.ep12506502
PMID:7057054
Abstract

The ultrastructure of epidermolysis bullosa simplex type Weber-Cockayne has as yet not been studied systematically. Therefore biopsies of blisters freshly produced by friction after a hot bath were investigated by electron microscopy in 8 subjects of 6 families. Blister formation was found always to start in the basal layer of the epidermis. At first the cytoplasm with the cell organelles appears to become diluted, then holes develop in the cytoplasm which merge and finally form cytolytic blisters between the dermo-epidermal junction and the nucleus. The cell organelles remain remarkably intact. The cause of blister formation is still unknown, however, no structural abnormality was observed.

摘要

单纯性大疱性表皮松解症Weber-Cockayne型的超微结构尚未得到系统研究。因此,对6个家族的8名受试者进行了电子显微镜检查,研究了热水浴后因摩擦新产生的水疱活检标本。发现水疱形成总是始于表皮的基底层。起初,含有细胞器的细胞质似乎被稀释,然后细胞质中出现小孔,这些小孔合并,最终在真皮-表皮交界处和细胞核之间形成溶解性水疱。细胞器仍保持显著完整。水疱形成的原因尚不清楚,然而,未观察到结构异常。

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1
Ultrastructure of blister formation in epidermolysis bullosa hereditaria: V. Epidermolysis bullosa simplex localisata type Weber-Cockayne.遗传性大疱性表皮松解症中水疱形成的超微结构:V. 局限性单纯性大疱性表皮松解症(Weber-Cockayne型)
J Invest Dermatol. 1982 Mar;78(3):219-23. doi: 10.1111/1523-1747.ep12506502.
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[Ultrastructure of epidermolyses with junctional blister formation (author's transl)].
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