Briggaman R A, Wheeler C E
J Invest Dermatol. 1976 Nov;67(5):567-76. doi: 10.1111/1523-1747.ep12541646.
Whole-skin grafts from a patient with lamellar ichthyosis were maintained on athymic nude mice for periods of 6 weeks to 4 months with excellent preservation of all gross and histologic features of the disease. In recombinant grafts composed of lamellar ichthyosis epidermis and normal dermis grown for periods up to 87 days on nude mice, the abnormal epidermis retained all the features of lamellar ichthyosis. Similar results were found in recombinants of lamellar ichthyosis epidermis and dermis. In recombinant grafts of normal epidermis and lamellar ichthyosis dermis, the epidermis remained normal and did not become ichthyotic. These observations support the hypothesis that the defective gene in lamellar ichthyosis acts directly on the epidermis as opposed to acting indirectly through a systemic abnormality or through an effect on neighboring dermis.
将来自板层状鱼鳞病患者的全层皮肤移植片在无胸腺裸鼠身上维持6周直至4个月，疾病的所有大体和组织学特征均得到了良好保存。在由板层状鱼鳞病表皮和正常真皮组成的重组移植物中，在裸鼠身上培养长达87天，异常表皮保留了板层状鱼鳞病的所有特征。在板层状鱼鳞病表皮与真皮的重组移植物中也发现了类似结果。在正常表皮与板层状鱼鳞病真皮的重组移植物中，表皮保持正常，未发生鱼鳞病样改变。这些观察结果支持了这样的假说，即板层状鱼鳞病中的缺陷基因直接作用于表皮，而不是通过全身异常或对邻近真皮的影响间接起作用。
