Disseminated infection and pulmonary embolization of Cunninghamella bertholletiae complicated with hemophagocytic lymphohistiocytosis.

A 22-year-old Japanese woman was diagnosed with hemophagocytic lymphohistiocytosis and subsequently was treated with etoposide and cyclophosphamide. On Day 22, multiple nodular lesions appeared in the bilateral lungs. Neither the administered antibiotics nor the antifungal agent were effective, and she died suddenly of respiratory failure on Day 35. An autopsy revealed disseminated zygomycosis and a pulmonary infarction due to the embolization of an angioinvasive fungus, which was later identified as Cunninghamella bertholletiae using in situ hybridization of 18S rRNA. C. bertholletiae is aggressive as well as resistant to antifungal agents. This rare species should therefore be taken into consideration as a potential causative agent of zygomycosis.