PMC6734004_01
An itchy rash on the left side of patient's face (A); A brown mold cultured in Sabouraud Dextrose Agar (B); Histological features of the lymph node biopsy (C-D); Hematoxylin-eosin H-E staining shows fungi surrounded by an epithelioid and giant cell granuloma (original magnification x200) (C); H-E staining demonstrates infiltration of fungi without granuloma to another area of the biopsy sample (original magnification x400) (D).
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F1
The dark red patches observed on his face (Figure 1A) were about the size of an egg, soft, and well defined, with no ulceration. The culture of the neck lymphoid tissue yielded a black yeast-like fungus (Figure 1B); the pathological report indicated fungal granulomatous inflammation with necrosis (Figures 1C,D), and the strain was later identified as E. dermatitidis by DNA sequencing of the ribosomal DNA ITS domains.
Radiological examination of the patient: T1 weighted axial and T2-flair brain MR imaging showing pachymeningitis and hydrocephalus (A,B); The lung CT on the first day of his second admission shows no obvious signs of inflection (C); The chest radiograph (D); and lung CT (E) show pulmonary infection, pleural effusion, and partial atelectasis; Head CT on the first day after surgery (F) and a week after surgery (G) show rapid accumulation of cerebrospinal fluid (CSF).
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F2
The brain MRI (Figures 2A,B) presented with noticeable meningeal thickening, large edema in the corpus callosum and white matter in both the hemispheres, and obvious ventricular expansion. The lung CT (Figure 2C) showed no significant change as compared to the previous imaging. Streptococcus F group were cultured from his sputum. Serious pulmonary infection, pleural effusion and partial atelectasis (Figures 2D,E) resulted in repeated resuscitation from respiratory failure. However, deteriorating intracranial hypertension, ventricle enlargement, compression of brain stem, extensive intracranial infarction, and edema (Figures 2F,G) predicted a poor prognosis.
Pedigree of the patient with Exophiala dermatitidis infection and a CARD9 mutation: Square indicates male; circle indicates female; filled symbol indicates affected individual; diagonal line across the symbol indicates deceased individual; arrow indicates the proband (A); A novel c.759dup (p. Lys254fs) mutation was identified in the exon 5 of CARD9
(B); Histological features of the brain tissue biopsy (C-E): Hematoxylin-eosin H-E staining shows fungal granuloma (C), magnification x200] and mycelia [(D), magnification x400]. PSA staining shows mycelia (E). Abbreviations are as follows: +, mutation; -, wild-type.
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F3
A brain tissue specimen was collected during surgery for histopathological assessment, which showed mycelia and fungal granuloma (Figures 3C-E).