中文摘要：

目的 探讨阿维A对大疱性鱼鳞病样红皮病组织病理和细胞超微结构的影响以及调控此类疾病趋向于正常角化过程的机制.方法 应用光镜及透射电镜观察4例大疱性鱼鳞病样红皮病患者阿维A治疗前后皮损的组织学及细胞超微结构改变.结果 治疗后4例患者临床皮损改善均超过75%,其中1例治疗后临床皮损改善达90%.4例患者组织学及超微结构均显示为角质层角化过度改善明显,角质化包膜的连续性有所改善,同时棘层及颗粒层角蛋白异常聚集的现象不同程度减轻,棘细胞增生与棘突松解现象则改善不明显.结论 大疱性鱼鳞病样红皮病患者经阿维A治疗后临床皮损改善明显,组织学及超微结构的改善主要在角质层,阿维A调节角质形成细胞的正常角化作用在角质层及颗粒层表现的更为明显.

英文摘要：

Objective To investigate the effect of acitretin on the histopathology and ultrastructure of lesions from patients with bullous ichthyosiform erythrodermia （BIE）, and to explore mechanisms underlying the modulation of keratinization process by acitretin. Methods Lesional tissue was obtained from the back of 4 patients with BIE before and after the treatment with acitretin. Light microscopy and transmission electron microscopy were performed to observe histopathological and ultrastructural changes in these lesions. Results After treatment, the improvement in clinical manifestations was more than 75% in all the 4 patients, and reached 90% in 1 of the 4 patients. As histopathology and ultrastructural study showed, there was an obvious improvement in hyperkeratosis and continuity of extra cellular lamellar membrane, and a decrease in keratin deposition in prickle and granular layer, but no remarkable changes were observed for the proliferation of prickle cells or acantholysis. Conclusions Acitretin shows a favorable efficacy in clinical treatment of BIE,with histopathological and ultrastructural improvement mainly located in the stratum corneum. The modulation of keratinization process in keratinocytes by acitretin appears more apparent in granular and corneum layers.