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KMID : 0390220190300010116
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Journal of Clinical Otolaryngology, Head and Neck Surgery
2019 Volume.30 No. 1 p.116 ~ p.121
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A Case of IgG4 Related Disease in the Nasal Cavity and Paranasal Sinus : A Rare Cause of Nasal Bleeding
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Mun Sue-Jean
Shin Na-Ri
Koh Jung-Hee
Roh Hwan-Jung
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Abstract
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Immunoglobulin (Ig) G4-related disease is a systemic chronic inflammatory disease characterized by sclerosing lesions by IgG4 positive plasma cells and T lymphocytes. It is rarely described in the sinonasal cavity and the paranasal sinus. Here we report a case involving a 69-year-old woman whose chief complaint was recurrent nasal bleeding for 2 years. Her nasal cavity showed erosion of nasal septum as well as the synechia in between the nasal septum and the lateral nasal wall with severe edematous change in the left inferior turbinate. Imaging finding showed bilateral soft tissue density in the maxillary and ethmoid sinuses and mucosal swelling of left nasal cavity. Her IgG4 level was elevated and proteinase-3 Ab was positive. Histopathological examination from the left inferior turbinate showed dense lymphoplasma cell infiltration and storiform fibrosis. Immunostaining for IgG4 and IgG revealed numerous plasma cells positive for IgG4 (more than 90 cells per high power field) and IgG4/IgG ratio of 0.7. Otorhinolaryngologist should consider IgG4 related disease as one of the differential diagnosis in case of bloody rhinorrhea with mucosal erosion or synechia inside the nasal cavity.
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KEYWORD
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IgG4-related disease, Plasma cell, Nasal cavity, Paranasal sinus
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