065 Evaluating pathogenicity of skin autoantibodies in hereditary epidermolysis bullosa hints towards predisposition of autoimmunity

Abstract

Skin blistering disorders result from damaged proteins involved in the dermal-epidermal adhesion. The damage is either caused by genetically induced defects or by autoantibodies targeting those proteins. In hereditary epidermolysis bullosa (EB) skin blistering is pathogenetically linked to genetic deficiency of distinct proteins of the epidermis or the dermal-epidermal junction, but circulating autoantibodies against these proteins have also been identified in EB patients. So far, the pathogenetic role of these autoantibodies is unclear, as they do not bind the respective skin structures, as shown by negative indirect immunofluorescence (IIF).