Abstract
Kopecki, Zlatko
PhD candidate, Discipline of Paediatrics, The University of Adelaide and Women's and Children's Health Research Institute
Regulating Flightless I protein in the skin may improve wound healing associated with genetic skin blistering diseases in children
Fragile skin disorders are characterized by recurrent skin blistering and often result in excessive scarring. Blisters and wounds form at the slightest touch. Wound healing in children suffering from blistering diseases posses a major challenge to their survival. The severity of the disease can range from mild blistering to large severe open wounds which require daily dressing changes. Continual skin blistering often results in fussed fingers or amputation of the limbs in children. Depending on extent of blistering majority of children do not survive to adulthood.
Children with fragile skin disorders often develop infections of large open wounds and aggressive skin cancers due to continual skin repair, resulting in high mortality rates. We have identified an important protein involved in wound healing. We have shown that decreasing this protein in skin results in improved wound healing. This harmful protein is naturally increased in skin of children with blistering diseases so reducing its levels could be expected to improve their healing.
Our studies have now shown that this protein is a key factor involved in blister formation and reducing its levels results in reduced blister formation. The present management of genetic blistering diseases is mainly supportive and no specific cure exists. Our studies could lead to the development of new therapies to reduce blister severity and enhance the quality and prolong the life of children suffering from blistering diseases.