Abstract
IgG4-related sclerosing disease is a well-recognized systemic chronic inflammatory disease histologically characterized by infiltration of IgG4-positive plasma cells and extreme fibrosis. It involves multiple organs and is assotiated with many chronic inflammatory diseases, such as autoimmune pancreatitis, retroperitoneal fibrosis, Mikulicz’s disease, and Küttner’s tumor. Recently, many cases of IgG4-related sclerosing disease with rhino-sinus involvements have been reported. Here, we report two cases of IgG4-related sclerosing disease with nasal involvement. Case 1, a 56-year-old male of Mikulicz’s disese complained of nasal obstruction and nasal bleeding. The nasal mucosa was covered with crust and susceptible to bleeding. Pathologic findings showed infiltration of IgG4-positive plasma cells in the nasal mucosa. Serum IgG4 level was 2,500mg/dl (maximum). Case 2, 76 year-old-male with Küttner’s tumor complained of nasal obstruction and nasal bleeding. The nasal mucosa was covered with thick crust and susceptible to bleeding. There was also a small polyp in the nasal cavity. Pathologic findings showed infiltration of IgG4-positive plasma cells in nasal mucosa. Serum IgG4 level was 1,260mg/dl (maximum). In both cases, nasal steroids were not effective. Oral steroids were effective for sialadenitis, dacryadenitis, dry mouth, and nasal symptoms. IgG4-related sclerosing disease should be included in the differential diagnosis of abnormal rhinitis, when the nasal mucosa is covered with crust and susceptible to bleeding.
