Hashimoto K, Mizuguchi R, Tanaka K, Dorman M
Department of Dermatology and Syphilology, Wayne State University School of Medicine, Detroit, MI 48201, USA.
J Dermatol. 2000 Jan;27(1):1-9. doi: 10.1111/j.1346-8138.2000.tb02108.x.
A case of the Voerner type palmoplantar keratoderma was studied for abnormalities of keratinization parameters. An enzyme and materials used to build the marginal band or cellular envelope of the cornified cell were all abnormally expressed; i.e. transglutaminase I (TGK), loricrin, and involucrin were abnormally immunostained. In the normal controls, their expression was limited to the upper epidermis, mainly in the granular layer. In the lesional skin, they were detected from the suprabasal layer to the lower horny layer. Filaggrin, the protein of the keratohyalin granule, was also expressed more widely than in controls. Ultrastructural abnormalities included a significantly higher frequency of composite keratohyalin granules than controls, early formation of a marginal band in the midepidermis, and, most remarkably, the clumping of tonofilaments causing vacuolization of the cytoplasm of affected keratinocytes.
对一例Voerner型掌跖角化病患者的角质化参数异常情况进行了研究。用于构建角质形成细胞边缘带或细胞包膜的一种酶及相关物质均表达异常；即转谷氨酰胺酶I（TGK）、兜甲蛋白和内披蛋白免疫染色异常。在正常对照中，它们的表达局限于表皮上层，主要在颗粒层。在病变皮肤中，从基底层上方至角质层下层均能检测到它们。角蛋白透明颗粒的蛋白质丝聚蛋白的表达也比对照更为广泛。超微结构异常包括复合角蛋白透明颗粒的出现频率显著高于对照、表皮中层边缘带的早期形成，最显著的是张力丝聚集导致受累角质形成细胞的细胞质空泡化。
