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
Single group
deucravacitinib
Drugtreatment by deucravacitinib in two phases of challenge
efficacy of deucravacitinib
number of new blister conting every day
Safety of deucravacitinib treatment
record of adverse events
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