Ichthyosis Scoring System—A Powerful Tool in the Era of Immune Pathway–Targeted Therapies for Ichthyosis

Inherited ichthyoses are a group of rare genetic keratinization disorders characterized by generalized hyperkeratosis, scaling, and frequently erythema.¹ These ichthyoses consist of nonsyndromic ichthyoses and ichthyosis syndromes, and more than 50 types of inherited ichthyoses are known.² Usually, the skin symptoms of inherited ichthyoses have their onset at birth or in early infancy, and they typically persist for life. Although congenital ichthyoses are a group of rare diseases, most congenital ichthyoses involve severe symptoms in the skin and in various other organs that substantially affect patient quality of life.^1,3