[Comel's linear circumflex ichthyosis and Netherton's syndrome. General conceptions based on study of 4 cases (author's transl)]

Abstract

We have recently observed 4 new particularly demonstrative cases and we think it is adequate to give a general conception in order to bring together in one and a single syndrom the ichthyosis linearis circumflexa Comel and the Netherton's syndrom. We also emphasize some features:--the possible association of ichthyosis linearis circumflexa with family epileptogenous encephalopathy;--a particular type of trichorrhexis which we named "frayed pili torti";--the usual lethality in males, with possible serious viral and pyococcic superinfection;--the characteristics of ichtyosiform erythroderma in the Netherton's syndrom. At the end of this study we confirm that a unicist theory which would consider Netherton's syndrom and ichthyosis linearis circumflexa as one and a single disease should be adopted.