Inclusion Criteria:

- Diagnosis of severe form of epidermolysis bullosa (EB) characterized by collagen,
laminin, integrin, keratin or plakin deficiency. Assessment criteria for severe EB:

- Severe blistering disease requiring dressings on >50% of the body surface area

- Documented collagen, laminin, integrin, keratin or plakin deficiency (by
immunofluorescence staining with protein specific antibodies or Western blotting
and by mutation analysis)

- Poor performance status as a result of EB (Lansky or Karnofsky <50%)

- Adequate Organ Function Criteria

- Renal: glomerular filtration rate > 60ml/min/1.73m2 patients aged ≤ 10 years

- Hepatic: bilirubin, aspartate aminotransferase/alanine aminotransferase
(AST/ALT), Alkaline phosphatase (ALP) < 5 x upper limit of normal

- Pulmonary: oxygen saturation >92%

- Cardiac: left ventricular ejection fraction ≥ 45%, normal electrocardiogram
(EKG) or approved by Cardiology for transplant.

- Performance status: ≥ 50% Lansky; ≥ 50% Karnofsky

- 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

Exclusion Criteria:

- Active systemic infection at time of transplantation (including active infection with
Aspergillus or other mold within 30 days).

- History of human immunodeficiency virus (HIV) infection

- Prior transplantation with donor skin

- Evidence of squamous cell carcinoma

- Donor has EB

- Pregnancy or breast feeding. Females of child-bearing age must have a documented
negative pregnancy test and agree to use contraception as a condition for enrollment.