HLA class II restriction of autoreactive T cell responses in pemphigus vulgaris: Review of the literature and potential applications for the development of a specific immunotherapy

Pemphigus vulgaris (PV) is a life-threatening autoimmune bullous disease of the skin and mucous membranes which requires immunosuppressive therapy, mo st commonly a combination of glucocorticoids and additional immunosuppressi ve agents. Since the side effects of long-term immunosuppressive therapy co ntribute to the poor prognosis of this disorder, there is considerable inte rest in a more specific treatment of this severe skin disease, PV may serve as a model disease for the development of a specific immunotherapy, becaus e its pathogenesis as well as involved immunogenetic factors are well-chara cterized. This review focuses on the characterization of autoreactive T cel l responses to desmoglein 3 (Dsg3), the autoantigen of PV, that presumably regulate the production of autoantibodies by providing help to the autoreac tive B cells. Current knowledge on T cell epitopes of Dsg3 and the HLA clas s II alleles that restrict Dsg3-specific autoreactive T cell responses, as well as potential applications for a specific immunotherapy of PV, are desc ribed.