Diagnostic Challenges in a Case of IgG4-RD Affecting the Temporal Bone.
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.
['Vuncannon|Jackson Ross|JR|', 'Panella|Nicholas John|NJ|', 'Magliocca|Kelly R|KR|', 'Mattox|Douglas E|DE|']
D000328:Adult D001327:Autoimmune Diseases D001847:Bone Diseases D005260:Female D034381:Hearing Loss D006801:Humans D007074:Immunoglobulin G D013701:Temporal Bone
temporal bone IgG4-related disease mastoid middle ear pathology temporal bone tumor