Key Inclusion Criteria: 1. Subject, or legally authorized representative, must be willing and able to give informed consent/assent 2. Aged ≥6 months 3. A genetically confirmed diagnosis of TGM1-deficient ARCI 4. Clinical diagnosis of lamellar ichthyosis 5. Two target areas located on similar anatomical regions with the same scaling severity by IGA and scaling severity ≥3 6. Subjects taking systemic retinoids must be on a stable regimen for at least 4 weeks and agree to continue to take the medication consistently throughout the study. If the subject prefers to withhold systemic retinoids during the study, then a minimum 4-week washout period is required 7. Clinically stable and in good general health Key Exclusion Criteria: 1. Subject has a physical condition or other dermatological disorder (e.g., atopic, seborrheic, or contact dermatitis, psoriasis, tinea infections, etc.) which, in the Investigator's opinion, might impair evaluation of the target areas or which exposes the subject to unacceptable risk by study participation 2. Participation in another clinical study or treatment with an investigational agent within 30 days or 5 half-lives, whichever is longer 3. Any condition or illness (including a history or current evidence of substance abuse or dependence) that, in the opinion of the Investigator, would impact a subject's ability to complete all study-related procedures and/or poses an additional risk to the assessment of safety of KB105 4. Women who are pregnant or nursing 5. Subject who is unwilling to comply with contraception requirements per protocol 6. Subject is known to be noncompliant or is unlikely to comply with the requirements of the study protocol in the opinion of the Investigator