Topical KB105 Gene Therapy for the Treatment of TGM1-deficient Autosomal Recessive Congenital Ichthyosis (ARCI)

Inclusion Criteria:

   - Genetic diagnosis of TGM1-deficient ARCI with a null TGM-1 mutation;

   - Clinical diagnosis of lamellar ichthyosis;

   - Age: 18 years old or older;

   - Individual site IGA score of 3 to 4 at the target areas

   - Subject is, in the opinion of the Investigator, able to understand thestudy, cooperate
   with the study procedures, and is willing to return to the clinic for all required
   follow-up visits;

   - Except for their moderate-to-severe ARCI, subject is in good general health; and

   - Willing and able to give consent/assent.

Exclusion Criteria:

   - Medical instability limiting ability to travel to the investigative center;

   - Medical illness expected to complicate participation, such as an active infection
   with: HIV, hepatitis B (as determined by hepatitis B surface antigen screening),
   hepatitis C (as determined by detection of hepatitis C antibodies or a positive result
   of hepatitis C);

   - Patient 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 patient to unacceptable risk by study participation;

   - Pregnant or breast-feeding women, or women planning to become pregnant or to
   breast-feed. Women of childbearing potential must have a negative urine pregnancy test
   at the Screening visit and Day 1 visit and must commit to using an acceptable form of
   contraception during the entire study period, up to three months after last KB105
   administration. Women using oral contraception must also have done so for 3 months
   prior to Baseline or will be willing to use a combination of barrier methods. To be
   considered not of childbearing potential, women must be post-menopausal for at least 1
   year or surgically sterile

   - Known allergy to any of the constituents of the product

   - Hypersensitivity to local anesthesia (e.g., lidocaine/prilocainecream)

   - Current enrollment in a clinical trial

   - Treatment with an investigational drug or investigational device within 30 days prior
   to Day 1

   - Male who is not surgically sterile nor willing to use effective forms of contraception
   from Day 1 until 3 months following the last dose of study drug.

   - Subject is known to be noncompliant or is unlikely to comply with the requirements of
   the study protocol in the opinion of the Investigator