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Rare Presentation of Progressive Saddle Nose Deformity

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

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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Article Information

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 (lees6@upmc.edu).

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.

References
1.
Montone  KT .  Differential diagnosis of necrotizing sinonasal lesions.   Arch Pathol Lab Med. 2015;139(12):1508-1514. doi:10.5858/arpa.2015-0165-RAPubMedGoogle ScholarCrossref
2.
Song  BH , Baiyee  D , Liang  J .  A rare and emerging entity: sinonasal IgG4-related sclerosing disease.   Allergy Rhinol (Providence). 2015;6(3):151-157. doi:10.2500/ar.2015.6.0136PubMedGoogle ScholarCrossref
3.
Takano  K , Yamamoto  M , Takahashi  H , Himi  T .  Recent advances in knowledge regarding the head and neck manifestations of IgG4-related disease.   Auris Nasus Larynx. 2017;44(1):7-17. doi:10.1016/j.anl.2016.10.011PubMedGoogle ScholarCrossref
4.
Karim  AF , Verdijk  RM , Nagtegaal  AP ,  et al.  To distinguish IgG4-related disease from seronegative granulomatosis with polyangiitis.   Rheumatology (Oxford). 2017;56(12):2245-2247. doi:10.1093/rheumatology/kex335PubMedGoogle ScholarCrossref
5.
Moteki  H , Yasuo  M , Hamano  H , Uehara  T , Usami  S .  IgG4-related chronic rhinosinusitis: a new clinical entity of nasal disease.   Acta Otolaryngol. 2011;131(5):518-526. doi:10.3109/00016489.2010.533699PubMedGoogle ScholarCrossref
6.
Takano  K , Abe  A , Yajima  R ,  et al.  Clinical evaluation of sinonasal lesions in patients with immunoglobulin G4-related disease.   Ann Otol Rhinol Laryngol. 2015;124(12):965-971. doi:10.1177/0003489415593557PubMedGoogle ScholarCrossref
7.
Deshpande  V , Khosroshahi  A , Nielsen  GP , Hamilos  DL , Stone  JH .  Eosinophilic angiocentric fibrosis is a form of IgG4-related systemic disease.   Am J Surg Pathol. 2011;35(5):701-706. doi:10.1097/PAS.0b013e318213889ePubMedGoogle ScholarCrossref
8.
Carruthers  MN , Topazian  MD , Khosroshahi  A ,  et al.  Rituximab for IgG4-related disease: a prospective, open-label trial.   Ann Rheum Dis. 2015;74(6):1171-1177. doi:10.1136/annrheumdis-2014-206605PubMedGoogle ScholarCrossref
9.
Shimizu  Y , Yamamoto  M , Naishiro  Y ,  et al.  Necessity of early intervention for IgG4-related disease—delayed treatment induces fibrosis progression.   Rheumatology (Oxford). 2013;52(4):679-683. doi:10.1093/rheumatology/kes358PubMedGoogle ScholarCrossref
10.
Khosroshahi  A , Wallace  ZS , Crowe  JL ,  et al; Second International Symposium on IgG4-Related Disease.  International consensus guidance statement on the management and treatment of IgG4-related disease.   Arthritis Rheumatol. 2015;67(7):1688-1699. doi:10.1002/art.39132PubMedGoogle ScholarCrossref
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