A 44-week Monocentric Open Study Assessing the Efficacy and Safety of Deucravacitinib in Adults With Inflammatory Genodermatoses

Inclusion Criteria: * Subject has congenital skin fragility with skin and/or mucosal blisters, a clinical phenotype of EBS-sev and a laboratory confirmed diagnosis of EBS by KRT5 and/or 14 mutation (autosomal only) OR a clinical phenotype of inflammatory ichthyosis (IC) including keratinopathic ichthyosis due to KRT1/10 mutation, ECI with identified genetic mutations (TGM5, NIPAL4, ABCA12, etc…). * Subject has at least a mean daily number of 4 new blisters (EBS-sev) OR an ISS \> 50/128. * Subject agrees not to use any topical therapies other than the investigator approved. Exclusion Criteria: * Subjects with another form of ICI ie Netherton syndrome, Kid syndrome etc. * Infectious/Immune-related Exclusions * Medical History and Concurrent Diseases