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通过抽吸水疱证明隐性营养不良性大疱性表皮松解症中透明层存在功能缺陷的证据。

Evidence for a functional defect of the lamina lucida in recessive dystrophic epidermolysis bullosa demonstrated by suction blisters.

作者信息

Tidman M J, Eady R A

出版信息

Br J Dermatol. 1984 Oct;111(4):379-87. doi: 10.1111/j.1365-2133.1984.tb06599.x.

Abstract

Suction blisters were raised in non-lesional skin of sixteen patients with various types of epidermolysis bullosa (EB). The ultrastructural level of separation was found in each type to be within the lamina lucida of the epidermal basement membrane zone. Suction blister times were normal in EB simplex, dominant dystrophic and localized recessive dystrophic EB, but were markedly reduced in both junctional EB and severe generalized recessive dystrophic EB. These data indicate an unpredicted abnormality of adhesion within the lamina lucida in severe recessive dystrophic EB, in addition to the well-recognized defect at a deeper level, beneath the lamina densa, in this condition. The use of suction blister times may prove valuable in the diagnosis of EB.

摘要

在16例患有各种类型大疱性表皮松解症(EB)的患者的非病变皮肤处产生了抽吸性水疱。发现每种类型在超微结构水平上的分离都位于表皮基底膜带的透明层内。单纯性EB、显性营养不良性EB和局限性隐性营养不良性EB的抽吸水疱形成时间正常,但交界性EB和严重全身性隐性营养不良性EB的抽吸水疱形成时间均显著缩短。这些数据表明,除了在这种情况下公认的致密层下方更深层次的缺陷外,严重隐性营养不良性EB的透明层内存在意想不到的黏附异常。抽吸水疱形成时间的测定可能对EB的诊断有价值。

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