J Rhinol.  2017 May;24(1):60-64. 10.18787/jr.2017.24.1.60.

A Case of Immunoglobulin G4-Related Sclerosing Disease of the Paranasal Sinus Mimicking Nasal Malignancy

• Chung J
• Lee JH

• KMID: 2379793
• DOI: http://doi.org/10.18787/jr.2017.24.1.60

Abstract

Immunoglobulin G4 (IgG4)-related sclerosing disease (RSD) is a chronic inflammatory disorder characterized by elevated serum IgG4 levels as well as an abundant infiltration of IgG4-positive plasmacytes. Involvement of the paranasal sinus is very rare, so it is difficult to make an accurate diagnosis. Hence, various differential diagnoses, which typically include elevated serum IgG4 levels and the infiltration of IgG4-postive cells into tissues, need to be excluded, especially for malignant diseases and mimicking disorders. Systemic corticosteroids are generally effective at inducing IgG4-RSD remission. Recently, a 43-year-old woman presented with a mass originating from the ethmoid sinus, suggesting malignancy. The patient underwent biopsy of the lesion. The pathologic specimen revealed the magnitude of IgG4-positive plasma cell infiltration, which was consistent with IgG4-RSD. In addition, serum IgG4 levels were markedly increased. After glucocorticoid treatment, the serum IgG4 levels decreased and the symptoms improved. We present a rare case of IgG4-RSD with isolated involvement of the paranasal sinuses.

Keyword

Immunoglobulin G4; Sclerosing disease; Paranasal sinus

MeSH Terms

Adrenal Cortex Hormones
Adult
Biopsy
Diagnosis
Diagnosis, Differential
Ethmoid Sinus
Female
Humans
Immunoglobulin G
Immunoglobulins*
Paranasal Sinuses
Plasma Cells
Adrenal Cortex Hormones
Immunoglobulin G
Immunoglobulins

Figure

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  Fig. 1 Endoscopic examination shows a polypoid mass that involve the left ethmoid sinus.

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  Fig. 2 Paranasal CT scans shows an expansive mass of the left ethmoid sinus with erosion of the lamina papyracea.

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  Fig. 3 Paranasal MRI scans shows mass-like lesion of the left ethmoid sinus with isosignal intensity on T1-weighted (A) and T2-weighted MR image (B).

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  Fig. 4 A: Hematoxylin-eosin staining revealed diffuse fibrosis with infiltration of lymphocytes and plasma cells (HE stain, ×400). B: Immunostain for immunoglobulin G4 (IgG4) shows many plasma cells that contain the IgG4 subclass, measuring up to 60/HPF (anti-IgG4 immunostain, original magnification ×400).

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