Orthopaedic Surgery, University of Rochester
Understanding the role of the humoral response in pemphigus vulgaris
Pemphigus vulgaris (PV) is a potentially fatal, prototypical organ-specific autoimmune skin-blistering disease. Antibodies directed at desmosomal components, mainly Desmoglein 3 and 1, cause loss of cell adhesion resulting in the formation of flaccid blisters. Recently, it has become clear that patients produce antibodies directed at many other targets, but their role in disease is currently unknown. We hypothesize that the specificity of patients' autoantibody profiles underlie the disease heterogeneity seen in PV. Characterization of the population and cellular effects of PV associated autoantibodies will lead to an increased understanding of disease pathogenesis, resulting in improved prognostic capabilities and the identification of novel therapeutic targets