Introduction
Ichthyoses, Erythrokeratodermas, and Related Disorders 57
Gabriele Richard
Key features
Ichthyoses and erythrokeratodermas are heterogeneous groups of disorders of cornification
Ichthyoses are characterized by scaling of the skin in a widespread distribution
Erythrokeratodermas feature discrete areas of erythema and hyper- keratosis, usually without substantial scale
Most inherited ichthyoses and erythrokeratodermas are evident at birth or manifest in infancy
Clinical features and pattern of inheritance as well as the underlying structural, biochemical, and molecular abnormalities help to differentiate these disorders
Therapy is symptomatic and primarily aimed at reducing hyperkera- tosis. Topical management consists of emollients, keratolytics, and retinoids
Many, but not all, disorders respond well to oral retinoids.
Treatment is initiated at low doses and titrated to response. Benefits and side effects must be carefully considered because of the chronic nature of these conditions