Distribution:
(1) Latin America
(2) Australia
(3) Southeast Asia
Clinical syndromes:
(1) cutaneous
(2) encephalitis
Usually the patient presents with a cutaneous lesion but eventually develops the encephalitis, despite maximal antifungal and antiamebic therapy. Most patients who develop encephalitis will die.
Features of cutaneous lesions:
(1) The lesions typically involve the central face (nose and cheeks) but may occur elsewhere.
(2) The lesions usually start as an erythematous, nonulcerated plaque with a consistency that ranges from rubbery to indurated.
(3) A biopsy will show a chronic granulomatous infiltrate. Trophozoites are usually present but may be confused with histiocytes. Cysts may or may not be present.
Features of the encephalitis:
(1) The patient develops seizures, focal neurologic deficits, elevated intra-cranial pressure and eventually coma.
(2) A brain biopsy shows areas of necrosis with trophozoites.
Differential diagnosis:
(1) leishmaniasis
(2) paracoccidiomycosis or other fungal infections
(3) Wegener's granulomastosis
(4) T or NK cell lymphoma
(5) sarcoidosis
