MT2015-20: Biochemical Correction of Severe EB by Allo HSCT and Serial Donor MSCs

Inclusion Criteria: * Diagnosis of severe form of EB characterized by collagen, laminin, integrin, keratin or plakin deficiency (by immunofluorescence staining with protein specific antibodies or Western blotting and by mutation analysis). * Adequate organ function within 4 weeks of study registration defined as: * Renal: glomerular filtration rate within normal range for age * Hepatic: Hepatic: bilirubin, AST/ALT, 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 EKG or approved by Cardiology for transplant * Sexually active participants must agree to use adequate birth control for the during the study period (from before the start of the preparative chemotherapy through 1 year post-transplant) * Available donor per section 5: targeted MFI \< 1,000 (MFI exceeding 1000 must be approved by the PI and treatment team.) * Voluntary written consent - adult or parent (with information sheet for minors, if applicable) prior to any research related procedures or treatment Exclusion Criteria: * beta 3 laminin JEB mutants * Active untreated systemic infection at time of transplantation (including active infection with Aspergillus or other mold within 30 days) * History of HIV infection * Evidence of squamous cell carcinoma * Pregnant or breast feeding. Females of child-bearing potential must have a negative pregnancy test prior to study registrati…