Biochemical Correction of Severe EB by Allo HSCT and "Off-the-shelf" MSCs

Inclusion Criteria: * Diagnosis of severe form of epidermolysis bullosa (EB) characterized by collagen, laminin, integrin, keratin or plakin deficiency. Assessment criteria for severe EB: * Documented collagen, laminin, integrin, keratin or plakin deficiency (by immunofluorescence staining with protein specific antibodies or Western blotting and by mutation analysis) * Adequate Organ Function Criteria * Renal: glomerular filtration rate within normal range for age * Hepatic: bilirubin, aspartate aminotransferase/alanine aminotransferase (AST/ALT), Alkaline phosphatase (ALP) \< 5 x upper limit of normal * Pulmonary: adequate pulmonary function in the opinion of the enrolling investigator * Cardiac: left ventricular ejection fraction ≥ 45%, normal electrocardiogram (EKG) or approved by Cardiology for transplant. * Available Healthy HSC Donor (order of preference) * Related Donor (marrow or UCB) * HLA-A, B, C, DRB1 genotypic identical (sibling) donor * HLA-A, B, C, DRB1 phenotypic identical donor * 7/8 HLA matched donor at HLA-A, B, C, DRB1 * Unrelated Donor * Marrow * HLA-A, B, C, DRB1 phenotypic identical donor * 7/8 HLA matched donor at HLA-A, B, C, DRB1 * UCB * HLA-A, B (antigen level) and DRB1 (allele level) matched donor * 5/6 HLA matched donor at HLA-A, B, DRB1 * 4/6 HLA matched donor at HLA-A, B, DRB1 * Voluntary written consent Absence of Exclusion Criteria: * Active systemic infection at time of …