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Coccidioidomycosis is a systemic mycosis that is acquired by inhalation. It is endemic in desert-like areas of the southwest USA and neighboring regions, but is becoming increasingly prevalent in other regions of the world as a result of widespread travel. The clinical spectrum is broad, ranging from inapparent infections or symptoms of acute respiratory infection with spontaneous recovery to various manifestations of disseminated coccidioidomycosis. Since the majority of patients with disseminated coccidioidomycosis present with cutaneous manifestations, early diagnosis of this potentially life-threatening disease by dermatologists is important. Atypical skin changes, pulmonary infiltrates and a history of travel to areas where the disease is endemic are indicative of coccidioidomycosis. For conclusive diagnosis, identification of the fungus by histopathologic examination or culture is desirable. Serological tests can be helpful for establishing the diagnosis and monitoring the course of the disease. The treatment of choice for cutaneous coccidioidomycosis is currently oral azole antifungal agents, such as itraconazole 400 mg daily, continued for 6 months after clinical response. Since relapses are frequent after discontinuation of the treatment, close clinical, serological and radiological follow-up is required for years.