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A 9-year-old, vaccinated boy with glucose-6-phosphate dehydrogenase deficiency and β-thalassemia trait presented with 1 month of progressive dyspnea, snoring, dysphagia, and weight loss. Both parents had a history of latent tuberculosis infection treated after immigrating to the US from Syria shortly after the patient’s birth. The patient’s pediatrician diagnosed him with reactive airway disease and adenotonsillar hypertrophy. He was referred to the otolaryngology clinic, where flexible laryngoscopy revealed diffuse upper airway lymphoid hyperplasia and supraglottic edema. He was sent to the emergency department, where he was stridulous and afebrile with a normal white blood cell count and an elevated erythrocyte sedimentation rate. A computed tomography scan of the neck revealed diffuse edema and contrast enhancement of the nasopharynx, oropharynx, hypopharynx, and supraglottis. A chest radiograph was normal.
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Sarcoidosis is a chronic granulomatous disease of unclear etiology. Its prevalence is about 10 to 35 cases per 100 000 people, with a significant African American and female predominance.1 The disease tends to affect those in the third or fourth decade of life, most often manifesting as cough or dyspnea.2 Sarcoidosis affects the head and neck region in 10% to 15% of patients and usually presents as cervical lymphadenopathy.3
The reported rate of laryngeal sarcoidosis ranges from 0.5% to 5% of all patients with sarcoidosis, and isolated laryngeal sarcoidosis is rarer still.2,4 Symptoms may include hoarseness, dyspnea, snoring, dysphagia, globus, and throat pain.2 Laryngoscopy shows pale edema of the epiglottis most commonly, followed by the arytenoids and aryepiglottic folds. The epiglottis may have a characteristic turbanlike appearance, as seen in this patient (Figure 1A).5 The glottis and subglottis are less frequently involved, likely owing to less lymphoid tissue in these areas.2,5 While sarcoidosis is a diagnosis of exclusion, serum ANA and ACE levels may be elevated, although the latter has low sensitivity (60%).2 Because ACE is primarily secreted by pulmonary endothelial cells and alveolar macrophages, levels may be normal in patients without pulmonary involvement.6 Histopathology will reveal noncaseating granulomas, as it did in this patient.
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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: Scott A. Hong, MD, Department of Otolaryngology–Head and Neck Surgery, St Louis University Hospital, 1008 S Spring Ave, 3rd Floor, St Louis, MO 63104 (firstname.lastname@example.org).
Published Online: September 3, 2020. doi:10.1001/jamaoto.2020.2577
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient’s parent for granting permission to publish this information.
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