Understanding Immunity and Fibrosis in Recessive Dystrophic EB

This blog post was co-authored by Seamus Graham.

Dr Yanling Liao and her research team, at New York Medical College in the USA, are aiming to understand immunity and fibrosis better, specifically in Recessive Dystrophic EB (RDEB).

Fibrosis refers to the chronic scarring of the skin associated with RDEB. The team are hopeful that through gaining this understanding, better treatments can be developed to alleviate the symptoms of the condition.

Dr Liao and her Research Team – Front: Yanling liao, PhD; Mitchell S. Cairo, MD Back: Morgan Anderson-Crannage (PhD student), Edo Schaefer (Pediatric hematology, oncology & stem cell transplant fellow) and Jian Pan (research associate)

The research project places an emphasis on understanding immune cell response and signalling pathways that are preventing the body from resolving the inflammatory response. The inflammatory response is how the body prevents initial infection and creates new tissue at the site of a wound, which is highly important for the healing of the chronic wounds associated with EB. However, if the inflammatory response cannot resolve itself, this is where long-term scarring (fibrosis) can occur. Chronic inflammation is extremely painful for those who have EB and puts them at a higher risk of developing squamous cell carcinoma, an aggressive form of skin cancer.

If scientists had a better understanding of how the immune cells prevent this resolution, then they could develop treatments which could target the problematic cells to help the inflammatory response complete itself. Dr Liao’s research team are investigating the immune cells and their interaction with other cells in the skin of mice with RDEB in the laboratory to try and gain this understanding. This could mean that treatments could be developed to treat long-term inflammation and prevent the scarring that comes with it. It could also reduce the chances of developing squamous cell carcinoma, as both long-term inflammation and scarring are associated with higher incidence rates.

DEBRA Ireland is delighted to be co-funding this project for the next 4 years with our colleagues in DEBRA UK.

DEBRA Ireland would like to thank Seamus Graham for his fantastic contribution to this article. We really appreciate it.

We love to see those who have EB or their family members getting involved in research, as no one understands EB better than those who have experienced it.

If you would like to get involved in future blog posts or projects, please contact Sarah: sarah@debraireland.org

Please see a video below of Dr Liao explaining her research.