Peer Reviewed
Cutaneous, Pulmonary, and Cardiac Invasion in a Young Woman
AUTHOR:
Paul Aronowitz, MD
CITATION:
Aronowitz P. Cutaneous, pulmonary, and cardiac invasion in a young woman. Consultant. 2014;54(9):737-738.
A 19-year-old woman without history of previous medical problems presented to the emergency department complaining of dry cough and dyspnea. Her symptoms started 3 months prior and acutely worsened on the day of admission. She reported frequent subjective fevers as well as drenching night sweats, but had never measured her temperature. She noted a 30-pound weight loss over the prior 3 months, despite a good appetite and the addition of high-calorie protein milkshakes to her diet.
The patient smoked 4 cigarettes and 2 to 3 marijuana joints daily. She was a resident of San Francisco, CA and her only recent travel had been to nearby Oakland. She initially reported that she had not been taking any medications.
Physical examination. The patient was afebrile with a heart rate of 124 beats per minute, blood pressure of 123/79 mm Hg, respiratory rate of 24 breaths per minute, and oxygen saturation of 91% on room air.
She was uncomfortable and in moderate respiratory distress. She had obvious facial plethora, as well as blistering, weeping skin lesions on her lower lip (Figure 1), and nose. Auscultation of the chest revealed crackles and coarse breath sounds throughout both lung fields. Examination of her extremities revealed nonpitting, upper extremity edema, and an additional blistering skin lesion under the nail bed of the left fifth finger (Figure 2).
Figure 1. Right lower lip lesion
Figure 2. Left fifth finger lesion
Laboratory tests. A chest radiograph (Figure 3) demonstrated mild diffuse, bilateral, interstitial infiltrates. Empiric treatment for community-acquired pneumonia was begun. An HIV-1/HIV-2 test was nonreactive. Due to concern for the possibility of a pulmonary embolism, a CT angiogram of the chest was performed which demonstrated extensive diffuse, bilateral pulmonary infiltrates, with marked mediastinal enlargement due to an infiltrating process, as well as pneumomediastinum (Figure 4). The superior vena cava was completely obliterated by the mediastinal infiltration with a contiguous mass protruding into the right atrium (Figure 5). The patient underwent a transesophageal echocardiogram, which demonstrated a well-defined, mobile mass protruding from the superior vena cava down into the right atrium (Figure 6).
Figure 3. Chest radiograph showing diffuse bilateral interstitial infiltrates.
Figure 4. CT angiogram of the chest showing extensive diffuse mediastinal infiltration (star) and pneumomediastinum (arrow).
Figure 5. CT angiogram of the chest showing filling defect in right atrium (arrow).
Figure 6. Transesophageal echocardiogram showing a lobulated, mobile, fungating mass with central necrosis (arrow) protruding through the superior vena cava (SVC) into the right atrium (RA). The left atrium (LA) and intra-atrial septum (IAS) are also shown in this image.
Subsequently, she underwent a CT-guided biopsy of the anterior mediastinal mass. The biopsy revealed chronic active mediastinitis containing a large numbers of spherules with endospores (Figure 7). This pathology, along with her sputum culture, left fifth finger wound culture, and serum antibodies, confirmed severe, disseminated infection with Coccidioides immitis. Upon further questioning, she reported that she had lived in California’s Central Valley for several months 2 years prior to admission. She also reported she had occasional shortness of breath during those previous 2 years, which she treated with a beta-2 agonist metered dose inhaler given to her by a friend.
Figure 7. Mediastinal biospy (hematoxylin and eosin stain) showing chronic active mediastinits with a large number of coccidioides spherules containing endospores (arrows).
Treatment. The patient was treated with intravenous amphotericin-B as well as intravenous fluconazole. She was subsequently intubated and ventilated for respiratory failure, subsequently requiring a tracheostomy. After a prolonged ICU and hospital course, she was transferred to a long-term rehabilitation facility for ventilator weaning, where she continued to recover and was eventually discharged home off the ventilator.
Discussion. Coccidioidomycosis is an infection caused by the dimorphic fungus Coccidioides. In 2011, there were over 20,000 cases of coccidioidomycosis with 75% of cases occurring in Arizona and 25% in California. Most infections are caused by the inhalation of spores. Sixty percent of infected persons remain asymptomatic.
Approximately 1 in 200 infected people develop disseminated disease, which can develop several years after initial infection. The diagnosis is often delayed due to the rarity of disseminated disease. Coccidioidal pneumonia most commonly presents with chest pain, cough, and fever. Other symptoms include fatigue, dyspnea, headache, sore throat, and arthralgias. Less common symptoms are hemoptysis and erythema nodosum.1-4 Our patient most likely contracted the infection while living in Fresno, CA, which is in the California Central Valley. She presented with clinical evidence of disseminated disease as characterized by her symptoms of chest pain, cough, fevers, dyspnea, upper extremity edema due to cardiac and superior vena cava invasion, and skin lesions.
For unclear reasons, certain populations, including Asians and African Americans,5 are at increased risk for developing disseminated disease. Other groups at increased risk for severe disease include pregnant women, infants, patients who have undergone thymectomy, patients with malignancy, diabetes mellitus, or those who are immunocompromised. AIDS patients with a CD4 count below 250 cells/mm3 are at particularly increased risk.6
Literature. We believe this to be the only reported case of an adult with a right atrial and anterior mediastinal coccidioidal mass of this scale. However, 1 case has previously been reported of a 10-year-old Mexican boy, with a pedunculated, highly mobile vegetation originating from the right atrial wall due to coccidioidomycosis. This occurred after a peripherally inserted central catheter line was placed with its terminus in the right atrium, which was thought to be a possible etiology of this complication.7 Additionally, there are 2 case reports of dogs developing masses at the bases of their hearts, found to be due to Coccidioides.8
Coccidioidomycosis can involve all layers of the heart. Fibrogranulomatous pericarditis, miliary granulomas and cardiac muscle abscesses can occur.9 Cardiac involvement due to Coccidioides infection is very rare and usually presents as pericarditis or myocarditis. Coccidioides endocarditis is extremely rare. In the few reported cases of Coccidioides, endocarditis pathology demonstrated granulomatous inflammatory nodules, but not the typical destructive reaction seen with bacterial endocarditis. In 1 report looking at 22 patients with clinical and pathological evidence of extrapulmonary coccidioidomycosis infections, only 6 of them were found to have cardiac involvement. Dyspnea and orthopnea were present in each of these patients. Symptomatic coccidioidal pericarditis with pericardial effusion was present in 3 of the 6 patients and severe myocarditis was present in all 6 patients.
Autopsy studies suggest 5% to 15% of patients with disseminated coccidioidomycosis have evidence of infection in the myocardium.10 A retrospective meta-analysis of case reports ranging over 30 years of fungal endocarditis found that almost all patients had a single fungal pathogen. The most common among these pathogens were Aspergillus species, Candida albicans, non-albicans species of Candida, and histoplasmosis. There were no reported fungal endocarditis cases with Coccidioides species, further suggesting that it is a remarkably rare presentation.11
In this case, the patient presented with severe disseminated coccidioidomycosis involving the lungs, mediastinum, heart, and skin. She had been having mild symptoms for 2 years, which she was apparently self-treating with bronchodilators. Shortly after presentation, her clinical status rapidly deteriorated, but she went on to survive a prolonged stay in the critical care unit, hospital, and rehabilitation unit. This case is unique due to the patients’ late presentation with very advanced disease, as well as the involvement of not only the mediastinum, but also the heart.
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