IV IgG for Dystrophic Epidermolysis Bullosa

(IV IgG Trial)

This trial tests a treatment called IV IgG (intravenous immunoglobulin) to determine if it aids in healing skin wounds in individuals with Recessive Dystrophic Epidermolysis Bullosa (RDEB) when combined with another treatment, VYJUVEK. The goal is to discover if this combination results in fewer and faster-healing wounds and to assess its impact on certain blood proteins. Potential candidates for this study include those with RDEB who are receiving ongoing VYJUVEK treatment and experience skin blistering over more than 5% of their body. As a Phase 1, Phase 2 trial, this research aims to understand how the treatment works and measure its effectiveness in an initial group of participants.

The trial protocol does not specify whether you need to stop taking your current medications. However, ongoing VYJUVEK treatment is required for participation.

Research has shown that IV IgG treatment holds promise for skin conditions similar to dystrophic epidermolysis bullosa (DEB). In past studies, IV IgG significantly helped, with 78.6% of patients experiencing disease remission in a similar condition called epidermolysis bullosa acquisita. This suggests that IV IgG might be well-tolerated.

Additionally, IV IgG is already used safely for other conditions. For instance, it serves as a safe and effective treatment for juvenile dermatomyositis, a muscle disease. Patients treated with IV IgG had good results and did not experience relapses over long periods.

Unlike the standard treatments for dystrophic epidermolysis bullosa, which often focus on topical applications and wound care, IV IgG offers a novel approach by utilizing immune globulin delivered intravenously. This treatment is unique because it potentially strengthens the body's immune response, helping to manage the condition from within, rather than just treating the symptoms externally. Researchers are excited because this could mean more comprehensive control of the disease and potentially fewer complications for patients.

Research has shown that IV IgG therapy might help treat conditions like dystrophic epidermolysis bullosa (EB). In some studies, IV IgG reduced inflammation, a major issue in EB. For people with EB, who often have non-healing wounds, IV IgG might help by adjusting the body's immune response. Although results have varied, some patients experienced wound healing improvements between 30% and 80%. In this trial, participants will continue their prescribed Vyjuvek application and receive IV IgG treatment. These findings suggest that IV IgG could be a useful addition to current treatments for improving wound healing.¹⁶⁷⁸⁹