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

To evaluate and further characterize the safety of EB-101 (LZRSECol7A1 Engineered Autologous Epidermal Sheets [LEAES]) for the treatment of RDEB wounds in new and previously EB-101 treated patients 6 years and older.

Inclusion Criteria:Clinical diagnosis of RDEBAge 6 years and older;Willing and able to give consent/assent; if under the age of 18 years, guardian(s) is/are willing and able to give consent;Positive expression of NC1+ amino-terminal fragment of C7 in the skin;Two confirmed RDEB C7 mutations with recessive inheritance patterns (or confirmation that parents don't have any evidence of dominant disease);Able to undergo adequate anesthesia during EB-101 treatment;All women of childbearing potential must have a negative urine pregnancy test and use a reliable birth control method throughout the duration of the study and for 24 Weeks post treatment;On stable pain medication regimen for at least 30 days prior to Screening;Must have at least one wound site that meets all of the following criteria:An area ≥20 cm2Present for ≥6 monthsStage 2 wound defined as an open skin wound with partial thickness loss of dermis that has not extended through the dermis into subcutaneous tissueExclusion Criteria:Medical instability limiting ability to travel to the study site or undergo EB-101 treatment;The presence of medical illness expected to complicate participation and/or compromise the safety of this technique, such as active infection with human immunodeficiency virus (HIV), hepatitis B, or hepatitis C;Evidence or history of immune response to C7 by indirect immunofluorescence (IIF) or enzyme-linked immunosorbent assay;Evidence of systemic infection;Current evidence or a history of squamous cell carcinoma (SCC) in the area that will undergo EB-101 application;Active drug or alcohol addiction;Hypersensitivity to vancomycin or amikacin;Receipt of chemical or biological investigational therapy for the specific treatment of RDEB in the 3 months prior to EB-101 application;Positive pregnancy test or breast-feeding;Inability to properly follow protocol assessments and protect keratinocyte sheet sites as determined by the Principal Investigator;Grade 3 clinical event or laboratory abnormality at Day 0. Abnormalities such as esophageal strictures, anemia, low albumin, and pain/itch are expected in RDEB patients, and these abnormalities will not exclude a patient;Unwillingness or inability to provide 4-biopsies or inability to manufacture patient's keratinocytes for use in EB-101 application.