Abstract
BACKGROUND: Medicopsis romeroi only rarely causes infection in immunocompromised individuals. We report a rare case of invasive infection in a specific primary immunodeficiency not arising from primary cutaneous infection. CASE SUMMARY: A 16-year-old male with chronic granulomatous disease (CGD) developed progressively enlarging diffuse pulmonary nodules in the setting of admission with obstructive hydrocephalus. A transthoracic biopsy grew a dematiaceous mold, identified as Medicopsis romeroi through DNA sequencing of the internal transcribed spacer (ITS) region of ribosomal DNA (rDNA). The nodules progressed despite a combination of posaconazole, isavuconazole, terbinafine, and liposomal amphotericin B. A course of HLA-compatible granulocytes was initiated, resulting in stabilization of the infection. CONCLUSION: This is a rare case of M. romeroi causing an invasive fungal infection of a visceral organ, the second documented M. romeroi infection in a CGD patient, and a report of a novel treatment regimen for this pathogen.