Human ß-Defensin 3 and Its Mouse Ortholog Murine ß-Defensin 14 Activate Langerhans Cells and Exacerbate Psoriasis-Like Skin Inflammation in Mice.

Our data show that one-third of patients with PD have autoantibodies to Col XVII that also bind to THþ neurons. The lack of reactivity of PD autoantibodies with skin suggests the autoantibodies develop from neuronal Col XVII. The subset of patients with neurologic disease that develop BP likely result from epitope spreading to regions of Col XVII that are pathogenic in skin. A key question that remains is whether Col XVII autoantibodies have a detrimental effect on PD or are otherwise predictive of disease onset/outcomes. Finally, these data suggest loss of tolerance to neuronal Col XVII may contribute to the risk for pemphigoid.