[Ichthyoses and related keratinization disorders. Management, clinical features and genetics].

This review concerns ichthyoses and related keratinization disorders. These are rare genetic skin diseases which are associated with marked scaling, often considerable cutaneous inflammation and a severe disease burden both physically as well as psychosomatically. The review briefly discusses how to establish a clinical diagnosis, how to provide genetic counseling for the patient and their family and how to best manage such diseases. Particular emphasis is given to the new possibilities in diagnosis and treatment opened by the recently established network for ichthyoses and related keratinization disorders (NIRK). This network is funded by the German Ministry for Education and Research and aims to integrate research efforts into disease mechanisms and to spread and transfer knowledge about these diseases to achieve better clinical care for the patients.