Congenital Ichthyosis: A Practical Clinical Guide on Current Treatments and Future Perspectives
Evelyn Lilly, Christopher G. Bunick
Abstract
Abstract: Congenital ichthyoses are a group of hereditary disorders of keratinization that are challenging to treat. Affected individuals suffer not only from thickening of the skin but also associated complications such as growth restriction, hearing and eye complications, infections, and thermodysregulation. This clinical review provides a practical roadmap to the longitudinal care of patients with ichthyosis with both general and age- and disease-specific recommendations. The allure of pathogenesis-based and targeted treatments for these monogenetic severe but orphan conditions shines bright as dermatological therapies enter a new era. Keywords: cutaneous disease, genetic mutation, skin care, clinical management, topical and oral therapy, JAK and TYK2 inhibitors, retinoids