Niemi K M, Virtanen I, Kanerva L, Muttilainen M
Department of Dermatology, Helsinki University Central Hospital, Finland.
Arch Dermatol Res. 1990;282(4):227-33. doi: 10.1007/BF00371641.
The pathogenesis of a rare form of the ichthyotic diseases, ichthyosis hystrix Curth-Macklin, was investigated by immunohistochemistry and electron microscopy. Monoclonal antibodies (Mabs) against keratins expressed in normal basal cells (PKK2 and KA1), Mabs against keratins only present in normal fetal skin (PKK1), and Mabs against keratins 1, 2, 10, and 11 (KA5 and K8.60) were used. The Mabs reacting with normal basel cells showed an increased reaction with many cell layers. The Mab PKK1 distinctly reacted with the basal cell layer, suggesting an expression of fetal keratins. Electron microscopic study of both normal-looking and involved skin revealed the keratinization disorder characterized by tonofilament shells, perinuclear vacuoles, and binuclear keratinocytes. The results suggest that there is no prematurity of keratinization, but rather a pathological expression of specific keratin genes leading to expression of fetal keratins in this form of ichthyosis hystrix.
通过免疫组织化学和电子显微镜研究了一种罕见的鱼鳞病——柯思-麦金豪猪状鱼鳞病的发病机制。使用了针对正常基底细胞中表达的角蛋白的单克隆抗体（Mabs）（PKK2和KA1）、仅存在于正常胎儿皮肤中的角蛋白的Mabs（PKK1）以及针对角蛋白1、2、10和11的Mabs（KA5和K8.60）。与正常基底细胞反应的Mabs与许多细胞层的反应增强。Mab PKK1与基底细胞层明显反应，提示胎儿角蛋白的表达。对外观正常和受累皮肤的电子显微镜研究揭示了以张力丝壳、核周空泡和双核角质形成细胞为特征的角化障碍。结果表明，这种豪猪状鱼鳞病不存在角化早熟，而是特定角蛋白基因的病理表达导致胎儿角蛋白的表达。
