Encephalitozoon (Septata) intestinalis: cytologic, histologic, and electron microscopic features of a systemic intestinal pathogen.

Encephalitozoon (Septata) intestinalis affects AIDS patients with CD4 counts <100/microL, causing intestinal and disseminated disease. It must be distinguished from the more common intestinal microsporidian, Enterocytozoon bieneusi, and from other microsporidia of extraintestinal tissues, such as Encephalitozoon hellem and E cuniculi, because clinical manifestations and treatment differ. In this report, the authors describe the diagnostic features of E intestinalis and illustrate all stages of its life cycle as exemplified by a case studied in detail. Spores can be detected by light microscopy in feces, urine, or nasal secretions, but not identified to species. A presumptive tissue diagnosis of E intestinalis can be made if 20 to 50 organisms 1.2-2.5 microm in diameter are seen within vacuoles in enterocytes. The diagnosis is substantiated if organisms also are present in stromal cells. On electron microscopy, the septate parasitophorous vacuole is pathognomonic. E bieneusi occurs only in intestinal and biliary epithelial cells, and never within a vacuole. E hellem and E cuniculi, which are not intestinal pathogens, may cause systemic infection but develop in a nonseptate vacuole.