The goal of this clinical trial is to learn about deucravacitinib treatment in adults with inflammatory epidermal genodermatoses . The main question it aims to answer are: describe efficacity and safety of this treatment.
Participants will take treatments and have to use bullets during the study period.
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.
Subjects with another form of ICI ie Netherton syndrome, Kid syndrome etc.
Infectious/Immune-related Exclusions
Medical History and Concurrent Diseases
Centre Hospitalier Universitaire de Nice
Lead sponsor