家族性持续性皮肤脱屑

Familial continual skin peeling.

作者信息

Abdel-Hafez K, Safer A M, Selim M M, Rehak A

出版信息

Dermatologica. 1983;166(1):23-31. doi: 10.1159/000249827.

Abstract

A clinical picture of familial continual peeling of the skin is described and discussed together with light and ultramicroscopic manifestations in one of the probands. The pattern of splitting distinguishes this condition from keratolysis exfoliativa. The ultramicroscopy exhibited mainly predominance of low-density keratocytes in the corneal layer, abnormalities in distribution of lamellar granules and disrupted coating of the cells in the intercellular spaces of the corneal layer.

摘要

本文描述并讨论了一例先证者的家族性皮肤持续剥脱的临床症状以及光镜和超微结构表现。其分裂模式将这种疾病与剥脱性角质松解症区分开来。超微结构显示，角膜层主要以低密度角质形成细胞为主，板层颗粒分布异常，角膜层细胞间隙中的细胞包膜破坏。