Genodermatoses with itch as a prominent feature.

A number of inherited conditions cause chronic itch as a part of the recognized phenotype. Advances in the understanding of the genetic factors that cause these diseases elucidate the molecular underpinning of itch as a symptom. Our knowledge of the causes of chronic itch has also advanced, providing an opportunity to integrate the genetic pathophysiology with the molecular landscape of chronic itch mediators. This article reviews select genodermatoses that have itch as a predominant feature with a focus on the pathophysiology of the disease, how it may lead to itch and potential therapeutic targets.