Diagnostic Challenges in a Case of IgG4-RD Affecting the Temporal Bone

Abstract

Introduction: Immunoglobulin G4–related disease (IgG4-RD) is a recently described fibroinflammatory condition with a characteristic histology. While IgG4-RD can affect a great variety of anatomical sites, it has been seldom described in the temporal bone. Methods: Herein, a case IgG4-RD occurring in the temporal bone of a 35-year-old woman is reported. Discussion: This case of IgG4-RD of the temporal bone proved a uniquely challenging diagnosis due to slightly atypical histology falling outside of “highly suggestive” criteria. Conclusions: We suggest that IgG4-RD remains a challenging diagnosis to reach despite increased awareness of the condition. We further suggest that clinicopathologic correlation remain the cornerstone of diagnosis as the spectrum of presentations of this newly described disease may be wider than previously anticipated.