The majority of disseminated cryptococcal infections occur in patients with acquired immunodeficiency syndrome (AIDS), with only 11-14% of cases occurring in patients without AIDS. Most non-AIDS related cases (75%) occur in patients with another immune deficiency. Here, we present the first case of mucocutaneous cryptococcal disease in an immunocompetent host, review the epidemiology of risk factors associated with disseminated cryptococcal disease, and describe a rational workup for a possible acquired immunodeficiency. While rare, 25% of non-AIDS related cryptococcal disease will occur in individuals without an identifiable immunodeficiency and should prompt a work up for cell-mediated immunodeficiency and monitored for closely for progression of other opportunistic infections.
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