title: Immunoglobulin G4-related disease in the skull base mimicking nasopharyngeal carcinoma.
abstract: Immunoglobulin G4-related disease (IgG4-RD) is a rare clinical condition. Although it has been described in the head and neck region, skull base involvement is exceedingly uncommon. We present a clinical report of a patient with IgG4-RD of the skull base that mimicked nasopharyngeal cancer. This case highlights challenges in the clinical diagnosis of this rare condition. The diagnosis could be made only on histopathologic evaluation, which showed dense fibrosis with lymphoplasmacytic infiltration by IgG4-positive plasma cells. Serum IgG4 level was also elevated. Once IgG4-RD was confirmed, treatment with glucocorticoid yielded a good clinical outcome.
authors.affiliation: From the Departments of *Otolaryngology, †Pathology, and ‡Radiology, China-Japan Friendship Hospital, Beijing, China.
authors.forename: Jianfeng
authors.initials: J
authors.lastname: Liu
authors.forename: Binglin
authors.initials: B
authors.lastname: Zhang
authors.forename: Hongliang
authors.initials: H
authors.lastname: Sun
authors.forename: Jun
authors.initials: J
authors.lastname: Han
authors.forename: Dazhang
authors.initials: D
authors.lastname: Yang
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005355:Fibrosis; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis* / Q000097:blood; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009303:Nasopharyngeal Neoplasms / Q000175:diagnosis*; D010950:Plasma Cells / Q000473:pathology; D019291:Skull Base / Q000473:pathology
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D008775:Methylprednisolone