title: Distribution and immunophenotype of the inflammatory cell population in the benign lymphoepithelial lesion (Mikulicz's disease).
abstract: Benign lymphoepithelial lesion (BLL) is an autoimmune process characterized by swelling and diffuse inflammation of the major salivary glands. Autoantibodies have been isolated from lymphocyte cultures obtained from affected salivary glands, but the pathogenesis is still unknown. Previous studies have shown that the predominant population of inflammatory cells is represented by helper T cells, with only brief mention of the B cell population. Twenty-five surgical specimens from patients with BLL were studied immunohistochemically. Antisera used included monoclonal antibodies LN-1 and LN-2 for B cells, LN-3 for cells expressing human leukocyte antigen-DR (HLA-DR) antigens, UCHL-1 for T cells, Leu-7 for natural killer (NK) cells, and T suppressor lymphocytes and the polyclonal antibody to S100 protein for dendritic cells. A peculiar distribution of the inflammatory infiltrate was observed in all cases, characterized by the presence of very irregular "germinal centers" with pseudopod-like extensions surrounding epimyoepithelial islands. Lymphoid cells in this location were reactive with LN-1 and LN-2 antibodies. These structures were surrounded by a "mantle" of mixed small B and T lymphocytes. A well-defined "interfollicular" zone was composed of cells strongly reactive with UCHL-1 and LN-3 antibodies, indicating the presence and activation of T cells. Dendritic cells defined by S100 and LN-2 reactivity were intermixed with epimyoepithelial cells, and were identified in 18 cases. Epithelial expression of HLA-DR antigens was restricted to inflamed areas. In contrast to previous reports denying the presence of Leu-7-positive cells in these lesions, cells reactive for this antibody were identified in 13 of 20 cases, predominantly within germinal centers. The presence of dendritic cells, complex organization of the inflammatory infiltrate into well-defined B cell proliferation centers and activated interfollicular T areas, and the abnormal expression of HLA-DR antigens in epithelial cells support an antibody-mediated destruction of the epithelial cells in this disease.
authors.affiliation: Department of Laboratory Medicine and Pathology, University of Minnesota Hospital, Minneapolis 55455.
authors.forename: R E
authors.initials: RE
authors.lastname: Andrade
authors.forename: K A
authors.initials: KA
authors.lastname: Hagen
authors.forename: J C
authors.initials: JC
authors.lastname: Manivel
mesh_terms: D006684:HLA-DR Antigens / Q000276:immunology; D006801:Humans; D008214:Lymphocytes / Q000473:pathology*; D008882:Mikulicz' Disease / Q000276:immunology / Q000473:pathology*; D012469:Salivary Glands / Q000473:pathology
chemical_list: D006684:HLA-DR Antigens