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

Skin blistering disorders result from damaged proteins involved in the dermal-epidermal adhesion. The damage is either caused by genetically induced defects or autoantibodies targeting those proteins. In hereditary epidermolysis bullosa (EB) skin pathogenetically linked to genetic deficiency of distinct epidermis junction, but circulating against these have also been identified EB patients. So far, pathogenetic role unclear, as they do not bind respective structures, shown negative indirect immunofluorescence (IIF). this project, we investigated sera 258 patients ELISA and found 22 % bullous pemphigoid antigen BP180. Among patients, six (2.33 %) with clinical features an autoimmune disorder (AIBD) positive IIF on human split skin. literature, four more cases developing disease-aggravating AIBD. Co-existence AIBD 2.33% our large cohort, compared prevalence 0.05% general population AIBD-onset at a very young age suggest that predisposition for development Our work highlights increased itch blister formation should be examined additional necessary confirm pathogenicity detected autoantibodies.