SUMMARY OF RESEARCH BEING UNDERTAKEN

Epidermolysis Bullosa is a set of genetically inherited conditions affecting at least 1:17,000 of the population. A fault in the gene causes the skin to be extremely fragile. The layers of the skin do not adhere properly and painful, widespread blisters occur very easily. Currently, there is no effective treatment. Wound healing is a very important area of DebRA’s research interests, alongside gene therapy and other radical treatments.

The development of physically and psychologically disabling scarring is a common feature of dystrophic epidermolysis bullosa. The purpose of this study is to develop improved means of reducing scarring in these individuals. The rationale underpinning the study is based on the following observations: (a) wound healing in the fetus is accompanied by considerably less scar formation than in the adult, (b) certain soluble molecules preferentially produced by fetal cells have been implicated in mediating this desirable situation and (c) the precise effects of these soluble molecules on cell behaviour is determined by the nature of the molecular "scaffolding" to which cells are attached. The immediate goal is to test the efficacy of several bioactive fetal-associated molecules, both alone and in combination, on the behaviour of adult skin cells growing on several distinct wound dressings which have been developed to simulate the structure of human skin. It is hoped that the information generated in this study will generate a productive collaboration between basic researchers and the wound dressing industry which will facilitate the translation of emerging biological insight regarding the control of wound healing into the improved clinical care of dystrophic epidermolysis bullosa patients.