A fatal case of Exophiala dermatitidis meningoencephalitis in an immunocompetent host: A case report and literature review

Abstract

Background: Central nervous system (CNS) infection due to Exophiala dermatitidis is rare and fatal, and primarily reported in immunocompromised patients or those with caspase recruitment domain-containing protein 9 deficiency. Herein, we describe a case of an otherwise healthy person (without underlying disease or gene deficiency) diagnosed with Exophiala dermatitidis meningoencephalitis. The patient achieved clinical remission under high-dose antifungal therapy in the first 14 months but died after 2 years of the therapy. Case presentation: A 15-year-old student with headache and fever was admitted to our department. Lumbar puncture showed increased cerebrospinal fluid (CSF) pressure, moderately high CSF protein levels and cell counts, and a remarkable decrease in CSF glucose and chloride. Magnetic resonance imaging of the brain revealed multiple lesions and cerebral pia mater enhancement. CSF culture confirmed E. dermatitidis infection. We administered 4-week antifungal therapy of amphotericin B, but his CSF culture remained positive. After receiving the 12-week standard dose of voriconazole (200 mg q12h), the patient's CSF culture became negative, but his condition deteriorated with intracranial lesion enlargement. We administered a high-dose voriconazole therapy (600-800 mg per day) for 12 months, which led to clinical remission. The voriconazole dose was reduced due to adverse effects including hepatic dysfunction and hypokalemia, and the disease progressed with high intracranial pressure and epileptic seizures. Conclusions: CNS infection caused by E. dermatitidis is fatal and the most serious form of fungal infection. Initially, high-dose and long-term antifungal therapy could be effective. Gene defect and related antifungal immunodeficiency may be the most important pathogenic and lethal factor.

Distribution of Yeast Species and Risk Factors of Oral Colonization after Oral-Care Education among the Residents of Nursing Homes

“…There were seven residents colonized by C. pararugosa , which was first reported in 2017 to cause bloodstream infections [ 27 ]. Hence, it is worth noting that in addition to those common species that cause diseases in humans, some rare species that were recovered in the present study such as Candida norvegensis [ 28 ], C. utilis [ 29 ], Cystobasidium minutum ( Rhodotorula minute ) [ 30 ], Exophiala dermatitidis [ 31 , 32 , 33 ], Lodderomyces elongisporus [ 34 , 35 ], Magnusiomyces capitatus [ 36 ], and Trichosporon mucoides [ 37 ] have been reported to cause invasive infections in humans.…”

Multidisciplinary management of disseminated Exophiala dermatitidis mycosis in an infant with mixed phenotype acute leukemia: a case report

“…To date, the treatment of E. dermatitidis has not been standardized. Based on previous reports, we administered L-AMB and VRCZ [ 25 , 26 ]. Although posaconazole is also a therapeutic choice for phaeohyphomycosis, it is recommended only for children 2 years and above.…”

Fungal corneal abscess caused by Exophiala dermatitidis

“…Reviewing the literature, we have only found a few cases of eye infection by E. dermatitidis [ 1 , 3 - 6 ]. However, this yeast is involved in diverse pathologies such as central line infection [ 7 ], pneumonia [ 8 ], meningoencephalitis [ 9 ] or skin and soft-tissue infections [ 10 ] among others.…”