Simoni S, Romano C, Bilenchi R, Alessandrini C, Fimiani M
G Ital Dermatol Venereol. 1989 Mar;124(3):97-102.
The occurrence of two cases of bullous congenital ichthyosiform erythroderma (BCIE) in a mother and son is reported. Clinical diagnosis was confirmed by histological and ultrastructural findings, which demonstrated marked changes in the cyto-skeleton of the keratinocytes of the Malpighian layer and areas of cytolysis and hypoplasia of the tonofilament-hemidesmosome complexes in the cells of the granulosa layer. These results and the possible aetiopathogenic mechanisms are discussed in the light of the most recent data in the literature. Treatment with oral etretinate proved to be helpful, but not long lasting.
本文报道了一对母子均患大疱性先天性鱼鳞病样红皮病（BCIE）的病例。临床诊断经组织学和超微结构检查得以证实，结果显示马尔皮基层角质形成细胞的细胞骨架有显著变化，颗粒层细胞中的张力丝-半桥粒复合体出现细胞溶解和发育不全区域。结合文献中的最新数据，对这些结果及可能的发病机制进行了讨论。口服依曲替酯治疗有效，但效果不持久。
