Image of the Month—Quiz CaseImage of the Month—Quiz Case FREE

A 53-year-old African American man presented with abdominal bloating and pain over 3 weeks. Intermittent nausea and vomiting was associated with the pain and he lost about 11 kg over the past 3 months despite good appetite. Three months prior, he had several weeks of dry cough and was diagnosed with walking pneumonia. This was treated with antibiotics and resolved. The patient had no medical problems and was human immunodeficiency virus (HIV) negative. He had an umbilical hernia repaired many years ago and did not take any medications. He did not smoke. He worked doing home construction in central California.

On examination, the patient had normal vital signs and appeared well. His abdomen was distended with a fluid wave and he had mild right upper quadrant tenderness but no rebound, guarding, or masses. Chemistry, complete blood cell count, and liver function test findings were significant only for mild anemia. Results of an upper endoscopy were normal. Computed tomography of his abdomen and pelvis demonstrated studding of his peritoneal lining (Figure 1) and large ascites as well as a 2-cm nodule in his right lower lobe. Chest computed tomography demonstrated no further abnormalities and the lesion was biopsied under computed tomographic guidance with findings of “granulomatous disease.” A paracentesis aspirated 1.4 L of serous ascitic fluid that showed numerous lymphocytes but pathology and culture results were negative. The patient then underwent diagnostic laparoscopy with peritoneal biopsy with findings as shown in Figure 2.

A.  Adenocarcinomatosis of unknown primary origin

B.  Disseminated coccidioidomycosis

C.  Peritoneal tuberculosis

D.  Primary peritoneal mesothelioma

Answer

Coccidioidomycosis refers to the spectrum of disease caused by Coccidioides species and clinical manifestations vary. Coccidioides are soil-dwelling fungi that exist solely in semiarid to arid areas and the incidence of infection has increased in recent years owing to population growth and accompanying construction within the southwestern United States.^1- 2 Individuals whose occupations involve the frequent aerosolization of soil are at particularly high risk of contracting coccidioidomycosis. Additionally, the increased number of immunosuppressed patients due to the use of immune-modulating drugs,³ transplants,^4- 5 and HIV⁶ have also contributed to the increased incidence.

An ethnic predisposition to coccidioidal infection causes Filipino and African American patients to have a disproportionate burden of disseminated disease with a 10- to 175-fold higher relative risk. Extrapulmonary disease most commonly manifests in the skin, joints, or central nervous system. Peritoneal coccidioidomycosis is an unusual extrapulmonary manifestation of infection affecting less than 1% of all patients with coccidioidomycosis. Most present with abdominal pain, swelling, or the new onset of an inguinal hernia.⁷ Rare cases have presented with abdominal or pelvic mass lesions, including 1 case presenting with vaginal prolapse.^7- 8 Despite peritoneal disease representing disseminated infection, fever is uncommon, found at presentation in only 5 of 26 prior cases.⁷ Diagnosis was established in 4 of 26 cases by nonsurgical means using culture of the ascitic fluid. Interestingly, 8 of 26 cases had the diagnosis incidentally discovered during herniorrhaphy after thick edematous tissue or granulomas were noted within the hernia sac. All patients demonstrated Coccidioides -specific antibody titers 1:16 or more, a value consistent with disseminated disease.⁹ Three patients ultimately died of disseminated coccidioidomycosis despite only 14 of 26 receiving antifungal therapy. Of these 3 mortalities, only 1 had been treated with amphotericin B (an HIV-positive patient with a CD4 cell count of 10); the other 2 did not receive treatment for unclear reasons. One other patient was HIV positive (CD4 cell count <200) and was treated with amphotericin B, eventually clearing the infection. Ultimately, 19 of 26 exhibited a complete response with no evidence of ongoing infection at follow-up. Of these 19, 5 cleared their infection without any antifungal treatment. These observations suggest the role of host immunogenetics in the ultimate control of invasive fungal infection.

Based on these findings, peritoneal coccidioidomycosis has a better prognosis than other extrapulmonary manifestations and is one of the few coccidioidal infections that may be cured. In our experience, during acute presentation, high-dose triazoles such as fluconazole or a lipid amphotericin B formulation should be administered. Following stabilization of disease, treatment with fluconazole or another triazole should be initiated until the patient returns to baseline and serologic titers have declined to normal values. At this time, discontinuation of antifungal therapy may be appropriate.

This case illustrates the potential for coccidioidal infection to mimic other pathology. Similar to the approach to pulmonary nodules within Coccidioides endemic regions where Coccidioides- specific serologies are obtained in an attempt to avoid unnecessary procedures, we advocate considering coccidioidal infection in all patients residing in or traveling to an endemic area if clinical parameters suggest the possibility of this diagnosis.

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