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Diagnostic Challenges in a Case of IgG4-RD Affecting the Temporal Bone
Jackson Ross Vuncannon1, Nicholas John Panella2, Kelly R Magliocca3
11 Wake Forest School of Medicine, Bowman Gray Center for Medical Education, Winston Salem, North Carolina, USA.
The Annals of Otology, Rhinology, and Laryngology
|November 11, 2016
Summary
Immunoglobulin G4-related disease (IgG4-RD) is a rare condition affecting the temporal bone. Diagnosis can be challenging due to atypical histology, emphasizing the need for clinicopathologic correlation.
Area of Science:
- Pathology
- Otolaryngology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a distinct fibroinflammatory condition.
- While affecting various anatomical sites, IgG4-RD in the temporal bone is infrequently reported.
Observation:
- A case of IgG4-RD involving the temporal bone in a 35-year-old woman is presented.
- The histological findings were slightly atypical, complicating the diagnostic process.
Findings:
- The diagnosis of temporal bone IgG4-RD presented unique challenges.
- Histology fell outside the criteria typically considered highly suggestive of IgG4-RD.
Implications:
- IgG4-RD diagnosis remains challenging despite increased awareness.
- Clinicopathologic correlation is crucial for diagnosing IgG4-RD, as presentations may be broader than previously understood.

