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A Painless Retroauricular Mass

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

A 31-year-old Hispanic woman had a 3-year history of a steadily growing, increasingly pruritic, painless mass behind the right ear. The patient denied bleeding from the mass, ear pain, trauma, fever, fatigue, night sweats, or recent illnesses. Her medical history included eczema treated with triamcinolone acetonide cream. Otherwise, the patient had no family history or exposure to tobacco or alcohol.

On physical examination, a 2-cm, nontender, mobile retroauricular mass with superficial excoriations was palpable over the right mastoid cortex. The patient had no hearing deficits and no fistula into the external auditory canal. There was no palpable head and neck lymphadenopathy. Serologic studies revealed an elevated absolute eosinophil count of 790/μL (reference range, <700/μL; to convert to ×109 per liter, multiply by 0.001) and an eosinophilia percentage of 9.7% (reference range, <6.0%). The T1-weighted postcontrast magnetic resonance imaging of the head and neck showed a heterogeneously enhancing mass with irregular borders in the right retroauricular space, with a tract coursing anteriorly toward the parotid gland (Figure, A).

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C. Kimura disease

Kimura disease (KD) is a chronic inflammatory disorder of the salivary glands or lymph nodes of the head and neck. Recognized in 1948 as a distinct clinicopathologic entity, much of our knowledge of this disease comes from a collection of sporadic case reports and small case series. It is characterized clinically by unilateral, painless cervical lymphadenopathy and deep subcutaneous masses of the head and neck, systemic eosinophilia, and elevated serum immunoglobulin E levels. This disease typically occurs in young Asian male patients in their second to fourth decades of life, although rare cases in other races and ethnicities have also been seen.1 We report a case of KD in an adult Hispanic woman in the United States.

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Article Information

Corresponding Author: Mark Swanson, MD, Caruso Department of Otolaryngology–Head and Neck Surgery, Keck School of Medicine, University of Southern California, 1540 Alcazar St, Ste 204M, Los Angeles, CA 90033 (mark.swanson@med.usc.edu).

Published Online: October 31, 2019. doi:10.1001/jamaoto.2019.3246

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Chen  H, Thompson  LDR, Aguilera  NSI, Abbondanzo  SL.  Kimura disease: a clinicopathologic study of 21 cases.  Am J Surg Pathol. 2004;28(4):505-513. doi:10.1097/00000478-200404000-00010PubMedGoogle ScholarCrossref
2.
Kapoor  NS, O’Neill  JP, Katabi  N, Wong  RJ, Shah  JP.  Kimura disease: diagnostic challenges and clinical management.  Am J Otolaryngol. 2012;33(2):259-262. doi:10.1016/j.amjoto.2011.05.005PubMedGoogle ScholarCrossref
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Ramchandani  PL, Sabesan  T, Hussein  K.  Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease.  Br J Oral Maxillofac Surg. 2005;43(3):249-252. doi:10.1016/j.bjoms.2004.11.023PubMedGoogle ScholarCrossref
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Tsukadaira  A, Kitano  K, Okubo  Y,  et al.  A case of pathophysiologic study in Kimura’s disease: measurement of cytokines and surface analysis of eosinophils.  Ann Allergy Asthma Immunol. 1998;81(5):423-427. doi:10.1016/S1081-1206(10)63139-0PubMedGoogle ScholarCrossref
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Sun  Q-F-F, Xu  DZ, Pan  SH,  et al.  Kimura disease: review of the literature.  Intern Med J. 2008;38(8):668-672. doi:10.1111/j.1445-5994.2008.01711.xPubMedGoogle ScholarCrossref
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Rajpoot  DK, Pahl  M, Clark  J.  Nephrotic syndrome associated with Kimura disease.  Pediatr Nephrol. 2000;14(6):486-488. doi:10.1007/s004670050799PubMedGoogle ScholarCrossref
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Kottler  D, Barète  S, Quéreux  G,  et al.  Retrospective multicentric study of 25 Kimura disease patients: emphasis on therapeutics and shared features with cutaneous IgG4-related disease.  Dermatology. 2015;231(4):367-377. doi:10.1159/000439346PubMedGoogle ScholarCrossref
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Yazici  D, Tuncer  U, Ergin  M.  Kimura disease of the parotid and retroauricular region: a case report.  Arch Otolaryngol Head Neck Surg. 2007;133(1):86-89. doi:10.1001/archotol.133.1.86PubMedGoogle ScholarCrossref
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