Jong Hoon Kim
Pemphigus is a chronic autoimmune bullous diseases characterized by the production of autoantibodies against desmogleins (desmoglein 1 and 3). Antibody-producing B cells are usually developed and activated in germinal centers of secondary lymphoid organs such as lymph nodes and spleen. Ectopic lymphoid structures (ELSs) resembling the germinal centers have been recognized at inflamed tissues of various infectious or autoimmune diseases; however, the ELSs remain undetermined in the skin lesions of autoimmune bullous diseases including pemphigus. Here, we found tight clusters of B cells and CD4+ T cells in the dermis of chronic skin lesions from 6 patients with pemphigus vulgaris, 3 patients with pemphigus foliaceus, and 1 patient with paraneoplastic pemphigus. The clusters in the dermis contain peripheral node addressin+ venules which are only observed in secondary lymphoid organs. Furthermore, lymphotoxin β, podoplanin+ venules, follicular dendritic cells, and CD11c+ dendritic cells were also detected in the ELSs. Desmoglein-specific B cells and plasma cells are present inner and peripheral areas of ELSs, whereas CD4+ T cells do not show the features of T follicular helper cells (e.g. PD-1, bcl-6) in CXCL13-enriched ELSs. In summary, we identified desmoglein-specific B cells in the dermal ELSs of chronic skin lesions from the patients with pemphigus.
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