Abstract
Introduction: Chronic granulomatous disease (CGD) is a primary hereditary immunodeficiency condition caused by a defect in the nicotinamide adenine dinucleotide phosphate (NADP) oxidase complex. Recurrent infections and inflammatory complications with a narrow bacterial and fungal spectrum characterize CGD. Acute inflammatory and infectious complications are vexing and fatal for them. Therefore, managing CGD with prophylactic antibiotics and antifungal agents is essential. Case Presentation: We present a 23-month-old infant with a chief complaint of recent lymphadenopathy diagnosed with CGD. Two months later, she was hospitalized with uncontrolled fever and lung involvement while receiving prophylactic treatment with trimethoprim/sulfamethoxazole. A mass-like consolidation was seen in her radiography evaluation. Based on the biopsy and bronchoalveolar lavage (BAL) pathology report, pulmonary mucormycosis was confirmed. Two weeks after starting the standard treatment regimen with amphotericin B, the patient’s symptoms improved. She was discharged in good general condition with oral voriconazole and trimethoprim/sulfamethoxazole and referred to do hematopoietic stem cell transplantation (HSCT). Conclusions: Mucormycosis in CGD is not a common infection and usually infects patients with impaired cell-mediated immunity, hematological malignancies, steroid treatment, and diabetes. Patients with CGD are vulnerable when treated with a high dose of immunosuppressant medications. In our case report, the patient was infected with mucormycosis despite not receiving immunosuppressant medications and having no history of hematologic malignancies or diabetes. Our patient also had a mass-like consolidation in her radiological result, a rare feature of pulmonary mucormycosis involvement in CGD patients.
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