Clinical Trial to Assess Safety and Efficacy of Autologous Cultured Epidermal Grafts Containing Epidermal Stem Cells Genetically Modified in Patients With JEB (HOLOGENE17)

Inclusion Criteria: 1. Signed and dated informed consent prior to any study-related procedures; 2. Male and female patients between 6 years old to 54 years old; 3. JEB molecular characterization by mutation analysis; 4. NC16A antibody immunofluorescence or positive staining in Western Blot analysis with polyclonal antibody produced against a synthetic peptide corresponding to amino acids 131-145 of human COL17A1; 5. Presence of chronic (persistent or recurrent for more than 3 months) large wounds (\>10 cm2) and/or persistent or recurrent erosions; 6. A cooperative attitude to follow up the study procedures (Caregivers in case of minors). Exclusion Criteria: 1. Known or suspected intolerances against anaesthesia; 2. Bad general condition (ECOG index \>1); 3. Unresectable metastasizing Squamous Cell Carcinomas (SCCs); 4. Antibodies to type XVII collagen associated antigens demonstrated on indirect immunofluorescence; 5. Clinical and/or laboratory signs of acute systemic infections at the time of screening. Patient can be re-screened after appropriate treatment; 6. Severe systemic diseases (i.e. uncompensated diabetes mellitus); 7. Female subjects: pregnant or lactating women and all women physiologically capable of becoming pregnant (i.e. women of childbearing potential) UNLESS they are willing to use one or more reliable methods of contraception with a Pearl index ≤1. Reliable contraception should be maintained throughout the study. 8. Allergy, sensitivity or intolerance to drugs, excipients or other material (as per Investigator's brochure): * Transport medium (Dulbecco's Modified Eagles Medium supplemented with L-glutamine) * Fibrin support * Povidone iodine 9. Contraindications to the local or systemic antibiotics and/ or corticosteroids foreseen by the protocol; 10. Contraindications to undergo extensive surgical procedures; 11. Clinically significant or unstable concurrent disease or other clinical contraindications to stem cell transplantation based upon investigator's judgment or other concomitant medical conditions affecting grafting procedure; 12. Patients (or parents in case of paediatric subject) unlikely to comply with the study protocol or unable to understand the nature and scope of the study or the possible benefits or unwanted effects of the study procedures and treatments; 13. Participation in another clinical trial where investigational drug was received less than 6 months prior to screening Visit.