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.
Diagnosis of generalized Recessive dystrophic epidermolysis bullosa (RDEB) demonstrated by COL7A1 mutations.
Diagnosis of EBA demonstrated by the presence of levels of serum C7 antibodies above the normal ELISA range
Baseline skin blistering greater than 5% total body surface area
1 wound at least 20 cm² able to be entirely treated with Vyjuvek weekly
History of thrombotic event(s)
History of cardiac failure
History of renal failure
IgA deficiency
M. Peter Marinkovich
Lead sponsor
Stanford University
Sponsor institution
Epidermolysis Bullosa Research Partnership
Collaborator