A rare case of oral tumor presenting as first sign of immunoglobulin G4-related disease

Abstract

Immunoglobulin G4 (IgG4)-related disease is an idiopathic autoimmune disease characterized by elevated serum and tissue IgG4 levels, organ enlargement, and a clinical response to immunosuppressants. We present such a case in a 39-year-old female, whose lesion was located in the right buccal space involving the minor salivary gland. After the tumorlike growth was removed, diagnosis was confirmed with histopathologic slides showing lymphoid cell infiltration, dense fibrotic stroma, and IgG4-positive plasma cells. The patient underwent steroid therapy, and there has been no recurrence since. Rarely do we see IgG4-related sclerosing disease involve the buccal minor salivary gland in its early stages. Thus, it is important to include IgG4-related disease in the differential diagnosis when considering autoimmune diseases with oral manifestations.