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


CONCLUSIONS
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.
1 Wake Forest School of Medicine, Bowman Gray Center for Medical Education, Winston Salem, North Carolina, USA.
Jackson Ross
JR
Vuncannon
2 Emory School of Medicine, Department of Otolaryngology-Head & Neck Surgery, Atlanta, Georgia, USA.
Nicholas John
NJ
Panella
3 Emory University School of Medicine, Department of Pathology and Laboratory Medicine, Atlanta, Georgia, USA.
Kelly R
KR
Magliocca
2 Emory School of Medicine, Department of Otolaryngology-Head & Neck Surgery, Atlanta, Georgia, USA.
Douglas E
DE
Mattox
D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D001847:Bone Diseases / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D034381:Hearing Loss / Q000209:etiology*; D006801:Humans; D007074:Immunoglobulin G; D013701:Temporal Bone
D007074:Immunoglobulin G
IgG4-related disease; mastoid; middle ear pathology; temporal bone; temporal bone tumor