Clinical Perspective on Exophiala dermatitidis, A Systematic Review of Cases Reported Globally over 36 Years

Exophiala dermatitidis is an opportunistic pathogen belonging to the so-called black yeasts, a group of fungi capable of causing a wide range of infections. Here we systematically reviewed 77 global cases of E. dermatitidis infections reported between 1989 and 2024. Most cases were reported from Japan (19/77), United States (16/77) and China (10/77), with 64.9% (50/77) cases reported since 2010, indicating an increasing global awareness of this fungus. Our results indicated that reported E. dermatitidis infections occurred in both immunocompromised (54.55%; 42/77) and apparently immunocompetent hosts (45.45%; 35/77), with deep-seated infections being the most common (76.62%; 59/77). Infections are associated with a high mortality rate, especially those affecting the central nervous system (63.63%; 7/11). Leukemia, nephropathy, diabetes, cystic fibrosis and CARD9 deficiency were identified as important predisposing factors for E. dermatitidis infection. All included cases were culture-confirmed, with a subset additionally supported by histopathological examination and/or molecular identification. Although there is currently no consensus about antifungal treatment for E. dermatitidis infections, our findings suggest that voriconazole was the most frequently used antifungal agent among the reviewed cases.