Bullous Congenital Ichthyosiform Erythroderma

—Electron Microscopic and Radioautographical Studies—

Abstract

A case of congenital ichthyosiform erythroderma was reported. The biopsied specimens were taken from the left chest and femoral skins and examined under electron microscopy, and their protein and DNA biosynthetic activities were also examined in vitro system. There were many vacuolated spinous cells in the epidermis and they were edematous in the cytoplasm in the electron micrograph in which no Golgi apparatus and mitochondria could be found. The nucleus, nuclear membrane and nucleole were preserved well. Desmosomes were also preserved in normal condition. No keratin pattern could be found in the stratum corneum which was consisted of two kind of cells. The one was filled up with keratin fibers and lined with thickened cellular membrane, and the other was cells in which keratin fibers were scanty and lack of thickened cellular membrane. The incorporation of ³H-glycine was observed remarkably in the basal cell layer and other upper two layers, However, only a few grains were found in the granular cell layers. No grain was observed in the vacuolated cells. The incorporation of ³H-thymidine was observed in the basal cell layers and other two upper cell layers.