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A 19-year-old White woman who was previously in good health, with no reported history of drug abuse, presented with progressive bilateral nasal airway obstruction, crusting, and saddle nose deformity over a 6-month period. The patient denied systemic symptoms, including oral ulcers, sicca, photosensitivity, dermatitis, or arthralgias. Her medical history had negative results for allergic rhinitis, asthma, chronic rhinosinusitis, epistaxis, nasal trauma, or surgery. Her family history was noncontributory. An in-office nasal endoscopy demonstrated synechiae bridging from the septum to the lateral nasal walls, polypoid changes, and yellow crusting bilaterally.
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B. IgG4-related disease
Midline destructive disease presents challenges for diagnosis even in the setting of extensive clinicopathologic workup. Sinonasal lesions may be severely disfiguring, with decreased quality of life and psychosocial sequelae. The differential diagnosis of destructive lesions of the sinonasal tract may be categorized as autoimmune disease and vasculitis (eosinophilic granulomatosis with polyangiitis [GPA] or Churg-Strauss syndrome), neoplastic disease (extranodal natural killer or T-cell lymphoma or angiocentric T-cell lymphoma), infectious disease (bacterial, fungal, and parasitic agents), iatrogenic conditions (outcomes of cocaine abuse or acetaminophen use), rare inflammatory conditions of unknown causative mechanism (including IgG4-related disease [IgG4-RD]), eosinophilic angiocentric fibrosis, and the more recently described lymphoplasmacytic chronic rhinosinusitis.1
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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Stella Lee, MD, Department of Otolaryngology–Head and Neck Surgery, University of Pittsburgh Medical Center, 1400 Locust St, Bldg D, Ste 2100, Pittsburgh, PA 15219 (email@example.com).
Published Online: August 6, 2020. doi:10.1001/jamaoto.2020.1756
Conflict of Interest Disclosures: Dr Lee reported grants from Sanofi Regeneron Genzyme, GlaxoSmithKline, and AstraZeneca outside the submitted work. No other disclosures were reported.
Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Raja Seethala, MD, for providing the pathology photographs and contributing to the analysis of the slides; no compensation was received for these contributions.
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