Targeting Collagen VII Antibodies with IV IgG in Dystrophic Epidermolysis Bullosa

M. Peter Marinkovich

Status and phase: Not yet enrolling; Phase 2; Phase 1

Conditions: Epidermolysis Bullosa Acquisita; Recessive Dystrophic Epidermolysis Bullosa; Dystrophic Epidermolysis Bullosa; Epidermolysis Bullosa

Treatments: Biological: Immunoglobulin G

Study type: Interventional

Funder types: Other

Identifiers

NCT06834035

73958

Details and patient eligibility

About

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.

Enrollment: 8 estimated patients

Sex: All

Ages: 6+ years old

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Treatment

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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8 participants in 1 patient group

Intra-Personal Control

Experimental group

Description:

There is one arm of the study. First, each participant undergoes a 3-month observational period, during which they continue application of their prescribed Vyjuvek. After 3 months, they enter the treatment period, during which they continue their Vyjuvek application and, in addition, receive the IV IgG treatment.

Treatment:

Biological: Immunoglobulin G