The Efficacy and Safety of Secukinumab in Patients With Ichthyoses
Condition: Ichthyosis; Autosomal Recessive Congenital Ichthyosis; Lamellar Ichthyosis; Congenital Ichthyosiform Erythroderma; Epidermolytic Ichthyosis; Netherton Syndrome
MeSH terms: Ichthyosis; Ichthyosis, Lamellar; Netherton Syndrome; Ichthyosiform Erythroderma, Congenital; Hyperkeratosis, Epidermolytic; Dermatitis, Exfoliative
Intervention: Secukinumab; Placebo
Intervention description: Anti IL-17A antibody
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Allocation: Randomized
Intervention model: Crossover Assignment
Masking: Quadruple (Participant, Care Provider, Investigator, Outcomes Assessor)
Enrollment: 20.0
Sex: All
Minimum age: 18 Years
Healthy volunteers: No

Summary:

      The ichthyoses are a group of lifelong genetic disorders which share characteristics of
      generalized skin thickening, scaling and underlying cutaneous inflammation. There are no
      therapies based on growing understanding of what causes the disease. However, there have been
      recent discoveries of marked elevations in expression of interleukin-17A (IL-17A) and
      IL-17-related cytokines in the skin of individuals with ichthyosis, which may explain the
      inflammation. Investigators propose that IL-17-targeting therapeutics will safely suppress
      the inflammation and possibly the other features of ichthyosis, improving quality of life.
    

Eligibility criteria:

        Inclusion Criteria:

          -  Subject has provided informed consent

          -  Subjects are at least 18 years of age or older at the time of screening

          -  Female subjects must not be pregnant or breast-feeding

          -  Female subjects of child-bearing potential with a negative urine pregnancy test and
             using at least one form of contraception (abstinence allowed)

          -  Subjects must have a confirmed diagnosis of ARCI (divided phenotypically into ARCI-LI
             or ARCI-CIE), EI or NS (by genotype or willingness to be genotyped)

          -  Subjects must be clinically judged to be immunocompetent.

          -  Subjects will have no allergy to secukinumab or components of the product.

          -  Subjects will have normal baseline laboratory testing (CMP, CBC, HIV negative,
             hepatitis B, C negative, QuantiFERON®-TB gold negative)

          -  Subjects must have an erythema score of at least 18 on IASI and an IASI-E score of 12
             (at least moderate severity of erythema) at baseline

        Exclusion Criteria:

          -  Subjects who are unable to give informed consent or assent.

          -  Subjects without a confirmed diagnosis ARCI, EI, or NS.

          -  Subjects who have a known allergy to secukinumab.

          -  Female subjects who are pregnant, considering becoming pregnant, or will breastfeed.

          -  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.

          -  Subjects who have used a systemic retinoid within one month prior to initiation.

          -  Subjects who have used topical retinoids or keratolytics within one week prior to
             initiation.

          -  Subjects who have used emollient on the area to be biopsied in the previous 24 hours