Journal of Immunology and Infectious Diseases

ISSN: 2394-6512

Open Access
Case Report
Max Screen >>

Isolated Laryngeal Coccidioidomycosis - A Case Report and Review of the Literature

Received Date: November 04, 2014 Accepted Date: January 02, 2015 Published Date: January 08, 2015

Copyright: © 2015 Antony N. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Related article at Pubmed, Google Scholar


Coccidioidomycosis involving the larynx is a rare occurrence which seems to present with non-specific upper airway symptoms. The diagnosis is made by tissue biopsy and the treatment is usually fluconazole for 6-12 months depending on the clinical presentation. In unusual presentations of laryngeal problems clinicians should consider Coccidioidomycosis, especially in endemic regions.

Case Presentation

We present a 59-year-old Hispanic female resident of West Texas who presented with symptoms of hoarseness, sore throat, and low-grade fevers for 2-3 months. She had been evaluated by her primary care physician who had treated her with several courses of antibiotics including quinolones, azithromycin, and cephalosporins. Because of the persistence of her symptoms she was sent to an otolaryngologist. On examination her temperature was 98.4 F, blood pressure was 124/78 mm Hg, and pulse 78 bpm. Laryngoscope revealed a soft tissue mass along the left side of the airway just above the vocal cords. No cervical lymphadenopathy was noted. The rest of the examination was unremarkable.

A biopsy of the lesion revealed spherules with endospores and accompanying granulomas; fungal cultures were consistent with Coccidiodes immitis. Chest x-ray was unremarkable. Computed tomography (CT) scan of the chest revealed two pulmonary nodules at the superior segment of the left lower lobe. Other relevant labs included hemoglobin and hematocrit of 13.9 g/dL and 40.6% respectively, normal range 11.5-15 and 34-44. White blood count was 11,000 per μL (4-10.5) with 58% neutrophils, 31% lymphocytes, 8% monocytes, 2% eosinophils, and 1% basophils. The C. immitis complement fixation titer (CFT) was negative at <1:1 and enzyme immunoassay (EIA) was unavailable. The patient was placed on fluconazole 400 mg per day for 6 months. Serial follow-ups revealed a decreasing size of the laryngeal mass with complete resolution of the lesion by the end of 3 months and repeat a chest x-ray demonstrated some resolution in the size of the lung nodules. Repeat serum Coccidioidomycosis CFT level remained negative.


Coccidioidomycosis spp. is a dimorphic fungus endemic to the southwestern United States and Northern Mexico [1]. Infection occurs through inhalation of the Coccidioidomycosis arthroconidium from the soil. The areas with the highest endemic sources of infection are in California and Arizona which together make up more than 50% of all cases [2]. The incidences in high-risk locations have risen from 5.3 cases per 100,000 in 1998 to 42.6 per 100,000 cases in 2011 [3]. This rise in reported cases has been in part hypothesized to the increased sensitivity of Coccidioidomycosis serological tests and industrialization into endemic areas [3,4]. The most common presentation of Coccidioidomycosis includes symptoms of chronic fatigue, malaise, and productive cough. Episodes of occasional of arthralgias are common with a primary pulmonary source as well [5]. The risk factors associated with severe disseminated infection are immunosuppression such as, AIDS, solid organ transplant recipients, lymphomas, or prolonged steroid use [6]. While the vast majority of Coccidioidomycosis infections have a primary pulmonary source, primary Coccidioidomycosis of the larynx is a very rare and unusual presentation.

A review of the literature was done using Medline and PubMed and was simplified into a table (Table 1). The following characteristics seemed consistent with patients with laryngeal Coccidioidomycosis. First, the most common complaint appeared to be vocal changes, primarily hoarseness of the voice in 50% of cases. Other presenting symptoms were cough, malaise, and a nonspecific flu-like presentation. Secondly, 50% of the patients demonstrated evidence of possible pulmonary involvement with chest imaging, however only 23% of patients underwent bronchoscopies or pulmonary biopsies. In a majority of cases, the diagnosis was confirmed with a biopsy of the larynx or sputum cultures. And thirdly, in 58% of cases the CFTs were equal or greater than 1:16. Any positive CFT is considered clinically relevant; IgG will diminish over a period of 6 months after treatment. It is for this reason that it is used as a marker of successful therapy along with clinical resolution of the initial symptoms. In addition, PCR for Coccidioidomycosis spp has been developed which has shown very high sensitivity and specificity in infected patients [7]. As well, use of enzyme immunoassay (EIA) for patients infected with Coccidioidomycosis can assist with a definite diagnosis in presence of an inconclusive or inconsistent CFT [8]. Sensitivity for patients with symptomatic infections is very high, however in immunocompromised or asymptotic patients results should be interpreted with caution [8].

The current treatment of laryngeal Coccidioidomycosis is amphotericin B or fluconazole, however the drug of choice is fluconazole [1,9-19]. To date, there have been no cases of laryngeal Coccidioidomycosis being treated with the newer azoles such as voriconazole or posaconazole. Relapse rates in isolated laryngeal infections are not known due to the scarcity of documented cases.

In this case, the usual features included multiple repeat negative CFT's, no definite evidence of pulmonary disease, and an isolated infection in an otherwise healthy female. At this point it would be difficult to speculate as to whether she would have had a positive EIA in the presence of a localized infection. However, given the laboratory and clinical data available, it would indicate the primary source of the patient's Coccidioidomycosis infection was limited only to her larynx. Due to her lack of pulmonary symptoms and negative CFT, a bronchoalveolar lavage was unnecessary and would add unneeded risks to the patient. There have been many proposed hypotheses of isolated laryngeal involvement however no proven theory has emerged as yet [20]. The most likely etiology of laryngeal involvement is from hematogenous spread of a primary pulmonary focus or dissemination from a high fungal burden in the host [20]. However, the etiology of our patient's laryngeal Coccidioidomycosis is not clear.

Due to the sporadicity of cases of laryngeal involvement of Coccidioidomycosis, morbidity and mortality is difficult to determine. With ease and mobility of travel it is very feasible for patients who live outside the expected area of infectivity to develop this illness. While cases of isolated laryngeal C. immitis are exceedingly rare, one should consider uncommon fungal etiologies like Coccidioidomycosis in patients with difficult to treat upper respiratory tract or pneumonia-like symptoms.

In summary, laryngeal Coccidioidomycosis is an unusual presentation of a common illness endemic to the southwestern United States. The diagnosis is made on the biopsy of the larynx and treatment is with fluconazole for 6-12 months.


Dr. Suresh J. Antony for his input and assistance in developing this manuscript.

12 Rosen EJ, Newlands SD, Patel J, Kalia A, Friendman, NR (2001) Reactivated laryngeal coccidioidomycosis. Otolaryngol Head Neck Surg 125: 120-1.
13 Hajare S, Rakusan TA, Kalia A, Gibson FB, Strunk CL (1989) Laryngeal coccidioidomycosis causing airway obstruction. Pediatr Infect Dis J 8: 54-6.
16 Gardner S, Seilheimer D, Catlin F, Anderson DC, Hernried L (1980) Supglottic coccidioidomycosis presenting with persistent stridor. Pediatrics 66: 623-5.
18 Galgiani JN, Ampel NM, Blair JE, Catanzaro A, Johnson RH, et al. (2005) Coccidioidomycosis. Clin Infect Dis 41: 1217-23.


Tables at a glance
Table 1
Author Year Age/Sex Presentation Diagnosis Pulmonary Cocci Serology Therapy Outcome
Allen et al. 2011 52/F 1 year history of dysphonia and cough with prior history of treated pulmonary cocciodioidomycosis Videolaryngoscopy postive for erytema and pseudomembranous exudate over epiglottis and periform fosssa. Chest CT demonstrated large peumoceles and lung damge. Biopsy of vocal cords and sputum cultures grew C. immitis. No Complement fixation titer (CFT) was 1:32 Fluconazole After 6 weeks of antifungal therapy, largyngoscopy noted complete resolution of infection.
Patel et al. 2009 28/M 1 week history of sore throat, hoarseness, dry cough, and dysphagia Contrast computed tomography scan of neck and chest positive for abscess on false vocal cord with lymphadenopathy and pulmonary nodules; culture positive for C. immitis. Yes Undetectable IgG & IgM I&D; oral fluconazole Clinically improved
Crum et al. 2004 47/M Hoarseness, neck adenopathy and increasing CF titer, history of pulmonary coccidioidomycosis FNA of neck LN was negative, laryngoscopy noted 3 nodules on vocal cords which yielded a positive diagnosis No CFT was 1:8, increased from 1:2 Fluconazole 600mg QD for 2 months Adenopathy and hoarseness resolved over 2 months. No relapses over 11 month follow-up peroid.
Rosen et al. 2001 14/M 3 week history of worsening hoarseness and dyspnea; history of disseminated coccio as a child Chest X-ray noted calcified granulomas consistent with previously treated pulmonary coccidioidomycosis. Laryngoscopy with biopsy grew C. immitis. No IgG was positive, CFT was 1:16 IV fluconazole for 3 days, switched to PO 200mg Q6hr Unknown
Boyle et al. 1991 40/F 6 week history of moderate hoarseness, lymphadenopathy of the rt side of the neck, fever, night sweats, moderate dry cough, and fatigue Contrast computed tomography scan of neck positive for supraglottic edema and near total compromise of the airway as well as bilateral lymphadenopathy and a nodule on right perijugular lymph node; biopsy and histological examination positive for caseating granulomas and multinucleated giant cells, double walled spherules with endospores indicated C. immitis. Yes IgM; CFT was 1:32 Fluconazole Clinically improved
Hajare et al. 1989 31mo/M 2 day history of respiratory distress and stridor, history of steroid-dependent nephrotic syndrome Chest X-ray was unremarkable. Laryngoscope revealed epiglottic and subglottic edema. Tracheal aspirate grew C. immitis. No CFT was 1:32 Amphotericin B for 4 weeks however d/c due to worsening renal function. Switched to fluconazole PO 6.6mg/kg/day for 1 year Compliment fixation was negative 6 months after hospital discharge
Dudley 1987 21F 3 month history of gradually increasing neck mass and reports of not feeling "up to par" Mass excised and histologic evidence of caseating granulomas. No CFT was 1:2 Excised; no antbx 3 year follow up showed no evidence of infection
Dudley 1987 13/F Neck mass and lethargy; 1 year later neck nodes developed again, bilaterally Biopsy/ histology confirmation; Biopsy showed caseating granulomas with endospore-packed spherules No CFT 1:2; CFT 1:32 Ketoconazole 6mo; ketoconazole (600mg) daily for 3mo. Cervical adenopathy disappeared; Cervical adenopathy disappeared with no signs of return over 3 year follow up
Benitz et al. 1983 5/M 5 week history of right-sided pleuritic chest pain, cough, fever, occasional night sweats; increasing stridor and intercostal retractions. Chest X-ray showed infiltrates in the middle and lower lobes of the right lung as well as mediastinal lymphadenopathy on the right. Coccidiodin skin test positive. Laryngoscopy and biopsy was performed and histologic examination showed granulomatous lympadenitis and spherules consistent with C. immitis. No CFT 1:8 initially, but up to 1:32 Amphotericin B for 10 wk (total dose of 60mg/kg) After 14 month there was no further recurrence of respiratory distress or stridor
Gardner et al. 1980 1/M 3 month history of rhinitis, stridor, and cough. Also noted were lethargy and dysphonia. Bronchoscopy/biopsy; sample obtained showed histologic evidence of granulomas and spherules suggestive of endospores consistent with C. immitis. The pathogen was also isolated from the sample; a skin C. immitis skin test showed 5mm of induration and erythema. Yes CFT was 1:8 Amphotericin B (IV) for 4 mo (total dose 65mg/kg) and tracheostomy Although CFT remined unchanged and skin test positive, no symptoms or growth recorded at 5 month follow up
Ward et al. 1977 4.5mo/M 3 month history of upper respiratory tract infection associated with wheezes and cough; subglottic stenosis with granular lessions Laryngoscope and biopsy were taken; upon histopathologic examination fibrous stroma and sphrules consistent with C. immitis were found. No CFT ranged from 1:32 to 1:128 Amphotericin B until CFT returned to 1:32 (total dose 271mg) At 6 month posttreatment follow up, patient had no signs of infection and healthy
Platt 1977 45/M Hoarseness for 1 year with associated fever, maliase, hemoptysis and weight loss; history of pulmonary coccidioidomycosis s/p lobectomy Chest X-ray showed extenstive bilateral infiltration with caviation. C. immitus was isolated in the sputum cultures. No CFT was 1:28-1:256 Amphotericin B for year intermittently, however sputum was still positive. Switched to miconazole nitrate for 10 weeks for resolution. Acute infection was resolved however bone marrow aspirate to assess anemia noted spherules of C. immitus.
Singh et al. 1956 34/M Produtive cough for 3 month with dysphagia and hoarseness for 1 month Tracheostomy with biopsy of tracheal cartilage grew C. immitus. Biopsies from cutaneous lesions also grew the fungus. No CFT was positive Dihydroxystilbamidine (IV) Q4hrs, total of 20 doses. 3 month after therapy, cutaneous lesions were healing and patient no longer required tracheostomy
Table 1: Review Literature of Coccidioidomycosis