Clinical Features of Autosomal Dominant and Sex-linked Ichthyosis in an English Population

Abstract

Three hundred and eighty-nine persons with ichthyosis ascertained in a population survey were examined. Through classifying patients by mode of inheritance the clinical and histological characteristics of common varieties of ichthyosis due to autosomal dominant or sex-linked genes were defined. Comparison of characteristics showed that separation of the two groups was possible. Apart from differences in distribution of ichthyosis on the body surfaces, males with sex-linked ichthyosis were more extensively affected and scaling did not improve with advancing years. By contrast, autosomal dominant ichthyosis was a milder condition with less prominent scales, and was frequently associated with manifestations of atopy, keratosis pilaris, and chapping. The appearance of the latter variety corresponds to the previous descriptive terms ichthyosis nacree or nitida and of the former variety to ichthyosis saurodermia or serpentina. The population frequency of ichthyosis was discussed and an unexpectedly high prevalence of sex-linked ichthyosis noted in Berkshire. © 1966, British Medical Journal Publishing Group. All rights reserved.