A 12-Week Multicentre, Randomized, Blinded, Parallel-Group Study Comparing the Efficacy and Safety of Ixekizumab to Placebo in Patients > 6 years of age with EB simplex generalized severe

INTERVENTION: Trade Name: Ixekizumab Product Name: Taltz 80mg Product Code: 3400930060797 Pharmaceutical Form: Injection Pharmaceutical form of the placebo: Injection Route of administration of the placebo: Subcutaneous use CONDITION: Epidermolysis bullosa simplex ; MedDRA version: 20.0 Level: PT Classification code 10014989 Term: Epidermolysis bullosa System Organ Class: 10010331 ‐ Congenital, familial and genetic disorders Therapeutic area: Diseases [C] ‐ Skin and Connective Tissue Diseases [C17] PRIMARY OUTCOME: Main Objective: to assess whether ixekizumab every 2 weeks (Q2W) is superior to placebo at Week 12 in the treatment of patients > 6 years of age with EBS‐gen sev in improving their QoL Primary end point(s): proportion of patients achieving = 75% of diminution of the dermatologic QoL (DLQI and cDLQI) at week 12 compared to baseline Secondary Objective: ‐To assess the safety of ixekizumab treatment in EBS‐gen sev treated patients; ‐To compare the efficacy and health outcomes measures evolution between ixekizumab Q2W and placebo at Week 12 in term of severity, itch, pain and duration of dressing; ‐To compare the efficacy and health outcomes measures evolution between ixekizumab Q2W and placebo between S12 and S20; Timepoint(s) of evaluation of this end point: AT 12 WEEKS SECONDARY OUTCOME: Secondary end point(s): ‐ Safety and tolerability will be assessed through the description of specific events occurring during the study ; ‐ Secondary efficacy and health outcomes measures ; ‐ Severity of EB will be assessed using the validated score iscorEB, the static ‐ Physician Global Assessment (sPGA) and the patient global assessment (PaGA) ; ‐ Itch will be assessed using a visual analogic scale (VAS). ; ‐ Pain will be assessed using a VAS ; ‐ The duration of dressing will be evaluated by patients/parents on the week before visit ; ‐ The QoL and the efficacy and health outcomes measures during the follow‐up post‐treatment period (S12‐S20) will be defined as for the treatment period ; Timepoint(s) of evaluation of this end point: AT 12 WEEKS INCLUSION CRITERIA: ‐ Male or female patients 6 years or older ‐ Laboratory confirmed diagnosis of EBS‐gen sev due to KRT5 or 14 mutation ‐ DLQI or cDLQI = 10 ‐ Subject/caregiver agrees not to use any topical therapies other than the investigator approved Are the trial subjects under 18? yes Number of subjects for this age range: 25 F.1.2 Adults (18‐64 years) yes F.1.2.1 Number of subjects for this age range 20 F.1.3 Elderly (>=65 years) yes F.1.3.1 Number of subjects for this age range 5