Haftek M, Thivolet J, Thomas L, Joubaud A, Faure M
CNRS UA 601/INSERM U. 209, Dermatology Clinic, Edouard Herriot Hospital, Lyon, France.
J Cutan Pathol. 1989 Feb;16(1):1-6. doi: 10.1111/j.1600-0560.1989.tb00001.x.
Epidermal keratinocytes separated from skin lesions of non-bullous congenital ichthyosiform erythroderma were investigated in an attempt at experimental reproduction of this keratinization disorder. In vitro studies on growth and differentiation of pathological keratinocytes isolated from the influence of the host's dermal and humoral components were performed using the immersed epidermal cell culture technique. Ten to 25-day-old confluent and stratified cultures were examined by means of photonic and electron microscopy, and stained with various differentiation markers for indirect immunofluorescence studies. The cultured epidermis showed low-grade differentiation and no clear-cut evenly distributed signs of the original disease. Grafting on congenitally athymic nude mice allowed further differentiation of the epidermal sheets and re-expression of the histologic and ultrastructural features of non-bullous congenital ichthyosiform erythroderma. Thus, the purely epidermal origin of this particular form of autosomal recessive ichthyosis could be confirmed. Large amounts of pathological keratinocytes generated from small skin biopsies may be used for experimental purposes after grafting on several athymic animals.
对从非大疱性先天性鱼鳞病样红皮病皮肤病变中分离出的表皮角质形成细胞进行了研究，试图对这种角化障碍进行实验性再现。使用浸没式表皮细胞培养技术，对从宿主真皮和体液成分影响中分离出的病理性角质形成细胞的生长和分化进行了体外研究。通过光子显微镜和电子显微镜检查10至25天龄的汇合和分层培养物，并用各种分化标志物染色以进行间接免疫荧光研究。培养的表皮显示出低级别分化，没有原始疾病清晰均匀分布的迹象。移植到先天性无胸腺裸鼠身上可使表皮片进一步分化，并重新表达非大疱性先天性鱼鳞病样红皮病的组织学和超微结构特征。因此，可以证实这种特殊形式的常染色体隐性鱼鳞病的纯粹表皮起源。从小皮肤活检中产生的大量病理性角质形成细胞在移植到几只无胸腺动物后可用于实验目的。
