Powell’s tissue engineering innovation targets rare skin disorder

Epidermolysis bullosa (EB) is a group of rare but devastating diseases that make the skin very fragile. Tears, sores, and blisters on the skin form when something rubs or bumps anywhere on the body, resulting in wounds that are difficult to heal. In some forms of EB, blisters may also develop inside the body, such as in the mouth, esophagus and major organs.

The symptoms of the disease usually begin at birth or during infancy, with the most severe cases causing lifelong disability that need ongoing medical care. There are no cures or FDA-approved treatments for the disease. But scientists like Ohio State University Materials Science and Engineering and Biomedical Engineering Professor Heather Powell are researching possible treatments and cures for EB.

Along with PI Dorothy Supp at the University of Cincinnati, Powell leads a team that earned a $2.5 million grant from the Department of Defense to advance an innovative skin tissue engineering technology that could improve the lives of those with recessive dystrophic epidermolysis bullosa, or RDEB. Their co-investigators include University of California Davis Professors Aijun Wang and Ping Zhou.

RDEB is caused by mutations in the collagen VII gene, COL7A1. People with RDEB suffer from painful wounds, disabling scarring, chronic morbidity, and shortened lifespan due to infections and aggressive squamous cell carcinomas.

Clinical trials by researchers elsewhere have shown that when sheets of COL7A1-modified autologous skin cells were grafted to wounds, improved healing was observed at six months, but the response declined over time. Powell and her colleagues believe they can improve the efficacy of this treatment.

“Our team’s strategy is to target near-term solutions by correcting the COL7A1 mutation and engineering patient-specific, full-thickness skin to replace the diseased tissue,” said Powell. “We will also develop technologies where a single or few gene corrected skin grafts could deliver collagen VII to the entire body, even difficult to graft locations like the esophagus.”

The team’s work will establish feasibility of this novel approach for local and systemic treatment of RDEB as well as other types of EB or for wound closure in patients with large skin injuries.