Targeting Collagen VII Antibodies With IV IgG in Dystrophic Epidermolysis Bullosa

Targeting Collagen VII Antibodies With IV IgG in Dystrophic Epidermolysis Bullosa

The study objective is to see if IV IgG treatment in Recessive Dystrophic Epidermolysis Bullosa (RDEB) skin in conjunction with VYJUVEK treatment improves wound healing and affects the levels of C7 and HSV-1 antibody levels in serum.

Fewer wounds, more rapidly healing wounds, and decreased C7 and HSV-1 antibodies could improve quality of life.

Inclusion Criteria:

1. Diagnosis of generalized Recessive dystrophic epidermolysis bullosa (RDEB) demonstrated by COL7A1 mutations.
2. Diagnosis of EBA demonstrated by the presence of levels of serum C7 antibodies above the normal ELISA range
3. Baseline skin blistering greater than 5% total body surface area
4. 1 wound at least 20 cm^2 able to be entirely treated with Vyjuvek weekly
5. 1 wound at least 20 cm^2 that has never been treated with Vyjuvek
6. Ongoing VYJUVEK treatment.

Exclusion Criteria:

1. History of thrombotic event(s)
2. History of cardiac failure
3. History of renal failure
4. IgA deficiency