Laryngeal Coccidioidomycosis: A Delayed Diagnosis With Consequences

To the Editors: The incidence of coccidioidomycosis continues to rise in the United States, especially in the southwest.1 Coccidioidomycosis most commonly presents as a nonspecific flu-like illness, leading to frequent misdiagnosis despite it being the cause of up to 29% of community-acquired pneumonias in endemic areas.2,3 Less than 1% develop extrapulmonary or disseminated disease, including infection of the bones, joints, nervous system, cervical lymph nodes, skin or larynx.4 Laryngeal disease—the presenting feature in our patient—is uncommon in all age groups and particularly so in children.5 We describe a previously healthy 13-year-old boy with 11 months of hoarseness and a cough. He was initially diagnosed with “puberty” by his primary care physician and then with asthma by an otolaryngologist in Tijuana, Mexico. His exposure history was unremarkable except for multiple visits to Tijuana within the year prior to the onset of symptoms. On physical examination, he was an obese and well-appearing boy with a very soft, hoarse voice. Decreased breath sounds were noted in the left upper lung lobe, and the oropharynx was clear. A complete blood count and comprehensive metabolic panel were within normal limits. A chest radiogram showed a small amount of scarring in the left upper lobe, and a lateral neck radiogram revealed increased prevertebral thickness of the soft tissue at the lower cervical spine levels. Computed tomography with contrast of the neck revealed circumferential thickening of the walls of the larynx and partially visualized a thick-walled cavitary lesion in the left lung apex. Multiple sputum samples were negative by polymerase chain reaction for Mycobacterium tuberculosis complex, and multiple sputum and urine smears were also negative for acid-fast bacilli. The human immunodeficiency virus antibody screen was negative. Laryngoscopy revealed diffuse and non-obstructive papillomatous lesions involving the entire supraglottis and glottis extending inferiorly along the posterior tracheal wall until approximately 2 cm above the level of the carina. Spherules were seen on biopsied laryngeal samples, and Coccidioides immitis was isolated (Fig. 1). Complement fixation titer for coccidioidomycosis was elevated to 1:256. We started the patient on 800 mg of oral fluconazole per day.Figure 1.: A: Direct laryngoscopy revealing diffuse papillomatous lesions involving the entire supraglottic, glottic and subglottic regions. B. Granuloma containing spherules filled with endospores, surrounded by mixed inflammatory infiltrate, hematoxylin and eosin (200×). The spherule measures up to 100 μm in diameter, with an eosinophilic wall, containing multiple basophilic endospores (5 μm).Complement fixation titer one year after presentation improved to 1:32. However, due to insurance coverage limitations, sporadic outpatient follow-up and inconsistent medication adherence, the patient developed recurrent Coccidioides meningitis, thus requiring lifelong treatment with antifungal therapy. As of four years after the initial diagnosis, he continued to experience severe disabilities in his speech and ultimately was referred to mental health services for depressive symptoms and social anxiety. Diagnosis and proper treatment of coccidioidomycosis are commonly delayed, which can lead to poor outcomes as experienced by our case patient. Even in endemic regions, coccidioidomycosis is often not included as part of the initial workup of pneumonia, particularly for less common presentations, as our patient.2,4 The continuing rise in the incidence of coccidioidomycosis and wide variations in its presentation urge consideration of coccidioidomycosis among patients with relevant exposure history to avoid delay in definitive diagnostic testing and therapy.