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A phase I/II, multicenter, randomized, double-blind, placebo within-patient controlled study to evaluate the safety and efficacy SXR1096 skin cream in patients with Netherton syndrome (NS)

Phase 1
Conditions
etherton syndrome
MedDRA version: 20.0Level: PTClassification code 10062909Term: Netherton's syndromeSystem Organ Class: 10010331 - Congenital, familial and genetic disorders
Therapeutic area: Diseases [C] - Skin and Connective Tissue Diseases [C17]
Registration Number
EUCTR2021-003210-39-AT
Lead Sponsor
Sixera Pharma
Brief Summary

Not available

Detailed Description

Not available

Recruitment & Eligibility

Status
ot Recruiting
Sex
All
Target Recruitment
20
Inclusion Criteria

1.Male or female patients aged 18 to 65 years at the screening visit and also adolescents (12-17 years) only after initial cohort of 5 adult patients have been treated for at least 7 days.
2.Patients/legal guardian endpoint must be willing to provide written informed consent. (Adolescents - as applicable in Part B - are eligible if all inclusion/exclusion criteria are met, benefits outweigh risks, are irreplaceable as a participant, and a legal guardian has conveyed and clarified the Informed Consent information to ascertain the subject's understanding of it as adequate for the decision).
3.Clinical diagnosis of NS including at least 3 out of the 4 following clinical criteria;
a.Neonatal erythroderma
b.Bamboo hair and/or alopecia
c.Chronic atopy specified as food allergy, asthma, rhino conjunctivitis and/or eczema for at least 2 years
d.Ichthyosis linearis circumflexa
4.Patients must be willing and able to understand and can comply with study requirements, apply the medication as instructed and be able to complete the study.
5.Absent LEKTI on immunohistochemistry of skin biopsy and/or confirmed mutation in SPINK5 gene
6.NS involvement = 20% of Body Surface Area (BSA) required at both the screening and baseline visits.
7.Investigator Global Assessment (IGA) of two areas to be treated, score =3, i.e. moderate or severe for each area required. Each target area approx. 9% of BSA. i.e. equal to one arm.
8.Female of childbearing potential must either commit true abstinence when this is in line with the preferred and usual lifestyle of the subject or use an adequate highly effective method of contraception throughout the study and for 4 weeks after the last study drug application. This criterion also applies to a prepubertal female subject who begins menses during the study.
Are the trial subjects under 18? yes
Number of subjects for this age range: 5
F.1.2 Adults (18-64 years) yes
F.1.2.1 Number of subjects for this age range 15
F.1.3 Elderly (>=65 years) no
F.1.3.1 Number of subjects for this age range 0

Exclusion Criteria

1.Female patient who is pregnant, nursing an infant or planning a pregnancy throughout the course of the study or is unwilling or unable to adhere to the contraception
2.Patient with any uncontrolled systemic disease. A potential patient in whom therapy for a systemic disease is not yet stabile for at least 3 months will not be considered for entry into the study.
3.Patient with positive serology tests like HIV, HCV & HBsAg.
4.Patient with presence of any skin disease that might interfere with the diagnosis or evaluation of the test medications. Cutaneous infection within 1 week before the baseline visit or, any infection requiring treatment with oral or parenteral antibiotics, antivirals, antiparasitics, or antifungals within 2 weeks before the baseline visit.
5.Patient that has a condition or is in a situation, which in the investigator's opinion may put the patient at significant risk, may confound the study results, or may interfere significantly with the patient's participation in the study.
6.Use of topical drugs that might alter the course of NS (e.g., topical corticosteroids and topical calcineurin inhibitors) within two weeks before baseline visit.
7.Patient with a known sensitivity to any of the study treatments and/or their components.
8. Patients with contact dermatitis-like reactions to the vehicle cream (placebo) at an evaluation conducted at Screening
9. Patient who anticipates a need to use other topical or systemic therapy that might alter the course of NS. Emollients/creams can be used on remaining skin area but not the test areas. Use of topical prescription treatment within 2 weeks prior to initial dosing of study drug. Recent systemic treatment for NS (e.g. systemic corticosteroids, antibiotics, immunosuppressant, biologic and biosimilars treatments). A washout period of 4 weeks will be required for such patients to be eligible to participate in the trial.
10.Patient who anticipates the need for surgery or hospitalization during the study.
11.Concurrent involvement in any other clinical study/expanded access program with an investigational drug or device, or participation in a clinical study within 30 days prior to entering the study.
12.Suspected or confirmed COVID-19 infection within 4 weeks before the screening or baseline visit. Unresolved COVID-19 infection. Planned vaccination for COVID-19 during screening, treatment period or before the follow-up visit.

Study & Design

Study Type
Interventional clinical trial of medicinal product
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Main Objective: The primary objective is to evaluate clinical safety and efficacy of the treatment with the IMP, compared to placebo, in patients with NS.;Secondary Objective: Secondary objective:<br>Part A) To assess the safety of SXR1096 multiple doses (the initial 7 days of treatment) in adults.<br>Part B) To assess the safety and efficacy of 4 weeks treatment with SXR1096 in adults and adolescents (12-17 years).;Primary end point(s): Primary endpoints:<br>•Safety assessment includes adverse events (nature and incidence), physical examination, vital signs, laboratory safety, and local tolerability.<br>•Primary efficacy endpoint:<br>o The change in Investigator Global Assessment (IGA) score at EOT compared to baseline.<br>;Timepoint(s) of evaluation of this end point: Part A) To assess the safety of SXR1096 multiple doses (7 days of treatment) in adults<br>Part B) To assess the safety and efficacy of 4 weeks treatment with SXR1096 in adults and adolescents (12-17 years)<br><br>
Secondary Outcome Measures
NameTimeMethod
Secondary end point(s): Secondary efficacy endpoints:<br>•Skin itching will be assessed by VAS and a multidimensional 5-D pruritus scale (Phan et al., 2012).<br>•Skin surface pH and transepidermal water loss (TEWL) will be measured on treated areas.;Timepoint(s) of evaluation of this end point: Part A) To assess the safety of SXR1096 multiple doses (7 days of treatment) in adults<br>Part B) To assess the safety and efficacy of 4 weeks treatment with SXR1096 in adults and adolescents (12-17 years)
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