Pulmonary Infiltrates, Eosinophilia, and a Facial Skin Nodule: DISCUSSION
Coccidioidomycosis is a geographically limited infection with a wide spectrum of illness, ranging from mild, self-limited, and clinically unrecognized infection to widely disseminated and potentially life-threatening disease.
Coccidioides immitis is a dimorphic fungus, found in the soil as an arthrospore and in human tissue as a round-walled spherule. It is found in semi-arid climates with short, intense rainy seasons, such as central California, Arizona, West Texas, New Mexico (the Lower Sonoran life zone), Mexico, and Central and South America. Except for rare cases of direct skin innoculation, infection in humans is by inhalation of the highly infectious arthrospores. Infectivity is enhanced by dry and windy conditions or activity that stirs up the soil (construction, archaeologic digging, and agricultural work). While infection is most common in these endemic areas, cases have been reported thousands of miles away, presumably through contaminated fomites. The diagnosis should be considered even in nonendemic areas.
Primary pulmonary infection is asymptomatic in at least 60 percent of cases; most often the illness is so mild that positive skin testing or chest roentgenogram changes are serrendipitous findings. The remaining 40 percent of cases have symptoms varying from mild flu-like illness to severe pneumonia. Eosinophilia is common, with counts usually from 3 to 26 percent of the total white blood cell count. Skin findings occur in 5 to 25 percent of patients. Erythema nodosum is characterized by tender, red nodules on the lower legs (“desert bumps”). Erythema multiforme is seen less frequently and occurs as a maculopapular rash on the extremities, often with vesicles or bullae. “Valley fever” comprises a constellation of cough, malaise, erythema nodosum, and arthralgias. This form of disease is thought to indicate a hypersensitivity reaction to the fungus; it usually lasts two to six weeks and has a good prognosis. Most cases of primary pulmonary infection show complete resolution of the chest roentgenogram findings, though some infiltrates form coin lesions, or coccidioidomas. A small number of cases progress to a chronic form, with cavity formation, lung volume loss, and a slow relapsing course over many years.
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Roentgenographically, primary pulmonary coccidioidomycosis most often shows nonspecific alveolar infiltrates. These can be distinguished from other pneumonias by three specific findings: (1) hilar lymphadenopathy, often bilateral, is seen in about 20 percent of cases; (2) “phantom infiltrates” that resolve in one lung segment and reappear in another; and (3) thin- or-thick-walled peripheral lung cavities that develop over a period of days.
Disseminated coccidioidomycosis occurs rarely, but is a serious complication that may involve many organ systems, especially the skin, bones, joints, meninges, and genitourinary system. Dissemination appears to be more likely in male subjects, dark skinned races (Mexicans, Blacks, Filipinos, and Native Americans), children, the elderly, immunosuppressed patients, and, possibly, pregnant women. Dissemination occurs early in the disease course, within a few months of pulmonary infection, and may be invoked by immunosuppressive agents (such as corticosteroids, chemotherapy, and HIV infection).
