382 Searching for targets to combat fibrosis in RDEB patients

Recessive Dystrophic Epidermolysis Bullosa (RDEB) is a devastating skin blistering disease for which there is still no available cure. Although RDEB is characterized by blisters caused by minimal trauma, non-healing wounds, recurrent infections, pseudosyndactyly, squamous cell carcinoma and others, one of the most challenging symptoms to treat in these patients is fibrosis. Healing takes place with an obvious scarring phenotype and RDEB can be viewed as a fibrotic disorder characterized by excessive ECM deposition, accumulation of collagen fibers, increase tissue stiffness and TGF β signaling.
Source: Journal of Investigative Dermatology - Category: Dermatology Authors: Tags: Genetic Disease, Gene Regulation, and Gene Therapy Source Type: research