A Case of Laryngeal IgG4-related Disease

IgG4-related disease is a lymphoproliferative disease of unknown etiology that is characterized by increased serum IgG4 concentrations, increased numbers of IgG4-positive plasma cells in the tissues, and enlarged, mass-like, and thickened lesions in various organs throughout the body. A 47-year-old female patient presented with pharyngeal pain and mucosal thickening of the larynx. She was referred to our hospital because of sore throat and mucosal thickening of the larynx. She was admitted to the hospital and started on antibiotic, intravenous steroid and antifungal therapy, and discharged from the hospital following symptomatic relief. Eight months later, she visited our hospital again with sore throat and mucosal thickening of the capsular region of the right tonsil. One and a half months after the second referral, white lesions and swelling of the right tonsil and soft palate were observed in addition to mucosal thickening of the capsule. A portion of the right tonsil was biopsied, including the white lesion, but histopathologic examination was negative for neoplastic lesions. The right tonsil was persistently swollen, and after obtaining consent from the patient, we performed bilateral tonsillectomy. Histopathological examination of the resected tonsils revealed IgG4-related disease. After resection of the palatine tonsils, the mucosal thickening of the larynx also improved. Thus, IgG4-related disease should be considered in patients presenting with recurrent laryngeal and tonsillar swelling of unknown cause.