Health Condition 1: Q803- Congenital bullous ichthyosiform erythroderma Health Condition 2: Q802- Lamellar ichthyosis Health Condition 3: Q808- Other congenital ichthyosis
Conditions
Interventions
Intervention1: injection secukinumab: Upon inclusion, the following drug protocol that will be used will be as follows:
ï?§ For patients weighing 50 kg- Injection secukinumab subcutaneously 150 mg on
Sponsors
Department of Health Research
Eligibility
Inclusion criteria
Inclusion criteria: 1.Congenital ichthyosis patients - patients with autosomal recessive congenital ichthyosis, Keratinopathic ichthyosis or Netherton syndrome- of age 6 years and older at the time of screening. 2.Confirmed diagnosis by next-generation sequencing 3.Women of child bearing potential with a negative urine pregnancy test and using adequate contraception. 4.Patients who can give valid consent. 5.Subjects must be clinically judged to be immunocompetent. 6.Subjects with no allergy to secukinumab or components of the product. 7.Subjects with normal baseline laboratory testing (CBC, HIV negative, hepatitis B, C negative, chest X ray)
Exclusion criteria
Exclusion criteria: 1.Subjects 2.Subjects who are unable to give informed consent or assent. 3.Subjects who have a known allergy to secukinumab. 4.Female subjects who are pregnant, considering becoming pregnant, or will breastfeed. 5.Subjects with a history of tuberculosis or immunosuppressed state 6.Subjects who have prior biologic use targeting IL-17A/IL-17 receptor A or IL-12/IL-23 or who have prior use of TNF-alpha blockers. 7.Subjects who have used a systemic retinoid within one month prior to initiation. 8.Subjects who have used topical retinoids or keratolytic within one week prior to initiation
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Reduction at week 24 in the IASI (Ichthyosis Area and Severity index)Timepoint: 1. Reduction at week 24 in the IASI (Ichthyosis Area and Severity index) | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Adverse events observed in patientsTimepoint: Anytime during study period;2. Change in the IASI-E (Ichthyosis Area and Severity index- erythema) and IASI-S (Ichthyosis Area and Severity index - Scaling) at 24 weeksTimepoint: 24 weeks;3. Reduction at week 24 in the VIIS (Visual Index for Ichthyosis severity)Timepoint: week 24;4. Reduction in the ichthyosis quality of life (IQoL-32) at week 24Timepoint: week 24;5. Proportion of patients achieving 50% reduction in IASI at week 24 (defined here as clinical improvement)Timepoint: week 24;6. Proportion of patients achieving 75% reduction in IASI at week 24 (defined here as clinical remission )Timepoint: week 24;7. Improvement in 5-D itch score at 24 weeksTimepoint: 24 weeks;8. Mean reduction at 24 weeks of mRNA levels of IL-17A and related genes in the peripheral blood mononuclear cells as measured by RT-PCRTimepoint: 24 weeks;9. Mean reduction at 24 weeks of serum levels of IL-17A and related genes as measured by ELISATimepoint: 24 weeks | — |
Countries
India
Contacts
Public ContactDr Rahul Mahajan
PGIMER Chandigarh
Outcome results
None listed