Definition

Mucormycosis is the common name given to several different diseases caused by fungi of the class Zygomycetes, order Mucorales. Organisms of the class Zygomycetes were first noted to cause disease in humans in publications from the 1800s. Platauf is credited with the first description of zygomycosis in the human nasopharynx in his 1885 paper entitled "Mycosis Mucorina" (1). His descriptions, in German, are detailed enough to suggest that this first case of disseminated disease in a cancer patient was caused by Absidia corymbifera. It was over 65 years later that Harris reported the first case of successful cure in a young girl who improved after correction of her DKA (2). She was the only known survivor until 1961, when Gass reported the successful management of a patient with amphotericin B, which became available in the 1950s (3).

The information that emerged over the next several decades was based predominantly on tissue morphology and rarely confirmed by culture. As a result, many of the early cases, and some of the cases still reported today, relied on the morphologic tissue findings of coenocytic, angioinvasive hyphae suggesting infection with one of the Mucorales. The majority of cases reported had no culture identification; instead, the infection was identified as a "mucormycosis" or Mucor infection, despite this lack of culture confirmation. Even with the poor showing with culture results, it soon became obvious that Rhizopus species, and not Mucor species, were the predominant organisms causing disease. Other important information was also being collected by astute clinicians and researchers regarding the association of zygomycosis with cancer, antibiotic or prednisone use, diabetes, deferoxamine and desferrioxamine therapy, transplantation, and the associated forms of immunosuppressive therapies.

With the development of diagnostic tools that allowed earlier diagnosis, with better surgical and antifungal interventions, and with more sophisticated laboratory methods for identifying these agents, more patients are surviving these previously fatal infections. The variety of organisms causing disease has also expanded. In addition to Rhizopus, Mucor, and Absidia, human diseases due to Rhizomucor, Apophysomyces, Saksenaea, Cunning-hamella, Cokeromyces, and Syncephalastrum species have all been confirmed. The manifestations of disease have also evolved from primarily rhinocerebral, pulmonary, and disseminated disease to include gastrointestinal (GI), cutaneous/subcutaneous, allergic disease, and even asymptomatic colonization.

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