Summary

With the increasing prevalence of diabetes and immunosuppressive conditions, zygomy-cosis has emerged as an important fungal infection. The increasing number of organ transplantations and new immunosuppressant therapies also may be changing the demographics of this disease. In the setting of diabetes mellitus, hematological or solid-organ malignancies, transplantation, neutropenia, steroid therapy, and other immunocompromising conditions, the Mucorales tend to produce angioinvasive disease. Approximately 12% of fungal infections in patients with hematologic diseases have been found to be caused by the order Mucorales, the best-known pathogens being of the family Mucoraceae, including the genera Rhizopus, Absidia, Mucor, and Rhizomucor. The disease manifestation differs tremendously, depending upon the organism causing the disease and upon the underlying risk factor for acquiring the disease. The full spectrum of disease seen with these agents includes rhinocerebral, pulmonary, cutaneous, abdominal-pelvic and gastric, and disseminated presentations. Diagnosis of mucormycosis remains a major problem. Even in the presence of hematogenous dissemination of fungi, blood cultures are negative, and the detection of specific antibodies in patients with mucormycosis has revealed poor sensitivity and specificity. Therefore, tissue biopsy and histologic identification of fungi remain the gold standard of diagnosis. Because the diagnosis is often made after death, the real incidence of mucormycosis may be underestimated. A high degree of suspicion, careful clinical examination, precise radiologic localization, and early aggressive treatment of mucormycosis are essential for the outcome of patients with an otherwise very poor prognosis. Therapy should begin immediately upon suspicion of diagnosis. Optimal treatment of mucormycosis involves both aggressive surgical debridement and appropriate systemic antifungal therapy. In particular, early surgical intervention has been associated with improved prognosis and survival rate. Overall, disease with the Mucorales tends to be fulminant and is fatal in all cases if not aggressively treated.

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