EB-101 Treatment for New and Previously Treated Patients With Recessive Dystrophic Epidermolysis Bullosa (RDEB)

Inclusion Criteria:

1. Clinical diagnosis of RDEB.

2. Age 12 months and older.

3. Willing and able to give consent/assent; if under the age of 18 years, guardian(s)is/are willing and able to give consent.

4. (This inclusion criterion was deleted as of Amendment 1.)

5. Two confirmed RDEB C7 mutations with recessive inheritance patterns (or confirmationthat parents don't have any evidence of dominant disease).

6. Able to undergo adequate anesthesia during EB-101 treatment.

7. All women of childbearing potential must have a negative urine pregnancy test anduse a reliable birth control method throughout the duration of the study.

8. On stable pain medication regimen for at least 30 days prior to Screening (andthrough Baseline).

9. Must have at least one wound site that meets all of the following criteria:

10. An area ≥20 cm2,

11. Present for ≥6 months, and

12. Stage 2 wound defined as an open skin wound with partial thickness loss ofdermis that has not extended through the dermis into subcutaneous tissue.

Exclusion Criteria:

1. Medical instability limiting ability to travel to the study site or undergo EB 101treatment.

2. The presence of medical illness expected to complicate participation and/orcompromise the safety of this technique, such as active infection with humanimmunodeficiency virus (HIV), hepatitis B, or hepatitis C.

3. (This exclusion criterion was deleted as of Amendment 3.)

4. Evidence of systemic infection.

5. Current evidence or a history of SCC in the area that will undergo EB-101application.

6. Active drug or alcohol addiction.

7. Hypersensitivity to vancomycin or amikacin.

8. Receipt of chemical or biological investigational therapy for the specific treatmentof RDEB in the 3 months prior to EB-101 application.

9. Breast-feeding.

10. Inability to properly follow protocol assessments and protect keratinocyte sheetsites as determined by the PI.

11. Grade 3 clinical event or laboratory abnormality at Day 0. Abnormalities such asesophageal strictures, anemia, low albumin, and pain/itch are expected in RDEBpatients, and these abnormalities will not exclude a patient.

12. Unwillingness or inability to provide 4 skin biopsies, or patient's keratinocytescannot be manufactured for use in EB-101 application.

13. Any other circumstance where the PI believes that the patient may not be appropriatefor participation in the study.