title: [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.
mesh_terms: D000328:Adult; D002648:Child; D003937:Diagnosis, Differential; D004476:Ectodermal Dysplasia / Q000175:diagnosis; D005260:Female; D006197:Hair; D006801:Humans; D007057:Ichthyosis / Q000175:diagnosis*; D007223:Infant; D008297:Male; D012536:Scalp Dermatoses / Q000175:diagnosis; D012867:Skin / Q000473:pathology*; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis; D013577:Syndrome
vernacular_title: Ichtyose linéaire circonflexe de Comel et syndrome de Netherton. Conception générale. A propos de 4 observations.