Clinicopathologic similarities between Mikulicz disease and Küttner tumor

Abstract 

Purpose

Recent studies have revealed that Mikulicz disease (MD) differs from Sjögren syndrome and is an immunoglobulin G₄ (IgG₄)–related systemic disease. Küttner tumor (KT) is also reported to be an IgG₄-related disease. In this study, we examined the clinicopathologic and serologic findings in MD (39 patients) and KT (6 patients) and attempted to discern the similarities between MD and KT.

Materials and methods

We diagnosed 39 patients with MD and 6 patients with KT. We analyzed the clinicopathologic and serologic findings (IgG subclasses) in 39 patients with MD and 6 patients with KT. Submandibular and labial salivary gland specimens obtained from patients with MD and KT were stained with anti-IgG₄ antibodies.

Results

The mean IgG₄ concentration (±SD) was 931.1 ± 796.2 mg/dL in patients with MD and 756.2 ± 449.2 mg/dL in patients with KT. Abundant infiltration of IgG₄-positive plasmacytes into the salivary glands was observed in both patients with MD and patients with KT.

Conclusion

The serologic and histopathologic findings in MD and KT are very similar, and these 2 conditions may be IgG₄-related systemic diseases.