Wevers A, Kuhn A, Mahrle G
Department of Dermatology, University of Köln, FRG.
J Am Acad Dermatol. 1991 Apr;24(4):638-42. doi: 10.1016/0190-9622(91)70099-n.
A 61-year-old man with palmoplantar keratoderma with an unusual tonotubular keratin is reported. The histologic findings, genetic transmission, and clinical course were similar to epidermolytic palmoplantar keratoderma (Voerner type), but keratinocytes ultrastructurally displayed a tonotubular cytoskeleton, which has not been previously described, instead of a tonofilamentous one. Electrophoretically, we found no difference in the keratin pattern of normal plantar skin, skin in palmoplantar keratoderma of Voerner, and that of our patient's skin. Therefore the tubular keratin most likely formed as a result of a posttranslational change of keratin polymerization.
报告了一名61岁患有掌跖角化病的男性,其具有一种不寻常的张力微管角蛋白。组织学发现、遗传传递和临床过程与表皮松解性掌跖角化病(沃纳型)相似,但角质形成细胞在超微结构上显示出一种此前未被描述过的张力微管细胞骨架,而非张力丝细胞骨架。在电泳方面,我们发现正常足底皮肤、沃纳型掌跖角化病皮肤以及该患者皮肤的角蛋白模式没有差异。因此,这种管状角蛋白很可能是角蛋白聚合翻译后变化的结果。