Dystrophic Epidermolysis Bullosa, Epidermolysis Bullosa, Epidermolysis Bullosa Acquisita, Recessive Dystrophic Epidermolysis Bullosa
Conditions
Keywords
Epidermolysis Bullosa, Epidermolysis Bullosa Acquisita, Dystrophic Epidermolysis Bullosa, Recessive Dystrophic Epidermolysis Bullosa, Collagen 7, C7, Col7, EB, EBA, DEB, RDEB, Antibodies, Collagen VII, IV IgG, IgG, IVIgG, C7 Antibodies, Collagen 7 Antibodies, Collagen VII Antibodies, Vyjuvek, B-VEC
Brief summary
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.
Interventions
BIOLOGICALImmunoglobulin G
Purified IgG from human serum, delivered via IV
Sponsors
M. Peter Marinkovich
Epidermolysis Bullosa Research Partnership
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
6 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Adverse Events and Effects | 9 Months | Occurrence of adverse events and effects |
| Percent Change in Wound Area | 9 Months | The wound area at the end of the treatment phase compared to the wound area at the beginning of the treatment phase. Wound healing (% and cm\^2) over the course of the treatment phase compared to the wound healing (% and cm\^2), over the observation phase. |
Countries
United States
Contacts
CONTACTKunju Clinical Research Coordinator, PhD
PRINCIPAL_INVESTIGATORMatt P Marinkovich, MD
Associate Professor of Dermatology
Outcome results
None listed