Scientific Substantiation and Assessment of the Effectiveness of Pathogenetic Methods of Therapy for Congenital Ichthyosis in Children
Congenital Ichthyosis
About this trial
This is an interventional treatment trial for Congenital Ichthyosis focused on measuring Congenital ichthyosis, Netherton syndrome, Autosomal recessive congenital ichthyosis, Biologic therapy
Eligibility Criteria
Inclusion Criteria:
The subject has signed an informed consent; parental or legal representative consent for patients under 18 years of age, as well as additional consent for patients aged ≥ 15 and <18.
- At the time of participation in the study, the age of the subject is not less than 6 months and not more than 18 years.
- At the time of participation in the study, a clinical diagnosis was established: Congenital ichthyosis with various clinical forms, with the exception of vulgar and X - linked congenital ichthyosis (genetic research is not a prerequisite for participation in the study).
- Subjects should have at least moderate IASI erythema associated with his / her ichthyosis, and a decrease in the quality of life according to CDLQI ≥ 10
- Absence of signs of severe infectious diseases (pneumonia, tuberculosis, etc.)
- No previous history of the use of the following genetically engineered biological drugs: ustekinumab, secukinumab, dupilumab
Exclusion Criteria:
- Subjects who have an allergic reaction to ustekinumab, secukinumab or other components of the drugs.
- Subjects who have bacterial and/or fungal diseases.
- Subjects who have problems in dynamic observation.
- Subjects who will have a worsening of clinical symptoms
Sites / Locations
- National Medical Research Center for Children's HealthRecruiting
Arms of the Study
Arm 1
Arm 2
Arm 3
Arm 4
Experimental
Experimental
Experimental
Active Comparator
Experimental group №1 (Secukinumab )
Experimental group №2 (Ustekinumab)
Experimental group №3 (Dupilumab)
Control group (Symptomatic therapy)
Secukinumab - subcutaneous injections into the shoulder according to the schedule of 0,1,2,3 weeks, then injections 1 time in 3 months up to 52 weeks of therapy.
Ustekinumab - subcutaneous injections in the shoulder on schedule 0; 1 month, then every 2 months up to 52 weeks of therapy.
Dupilumab - subcutaneous injections in the shoulder: for patients weighing from 15 to <30 kg: initial dose - 600 mg (2 injections of 300 mg), then 300 mg every 4 weeks; for patients weighing from 30 to <60 kg: initial dose - 400 mg (2 injections of 200 mg), then 200 mg every 2 weeks; for patients weighing 60 kg or more: the initial dose is 600 mg (2 injections of 300 mg), then 300 mg every 2 weeks.
symptomatic therapy with emollients + systemic retinoids