Mucormycosis in a Patient With AIDS Receiving Systemic Steroids

Presentation and History

A 39-year-old woman with AIDS and no known history of diabetes mellitus was admitted to the emergency department in late September 2012 for weakness, malaise, and dizziness. In August 2012, the patient had been admitted to the emergency department for seizures and a 1.2-cm enhancing calcified lesion with vasogenic edema in the frontal lobe. At that time, neurocysticercosis with cyst rupture was empirically diagnosed after work-up did not reveal any other cause of symptoms. She was discharged with an ongoing treatment regimen of albendazole (200 mg twice daily), dexamethasone (4 mg twice daily), levetiracetam (750 mg twice daily), and insulin (70% human insulin isophane suspension and 30% human insulin, 40 U twice daily). Medications included highly active antiretroviral therapy (combined zidovudine and lamivudine and combined lopinavir and ritonavir) for AIDS. Her last CD4 cell count (taken earlier that month before presentation) was 139 cells/mm³, with an undetectable viral load. Other notable medical history included deep vein thrombosis and gastroesophageal reflux disease.

Physical Examination

At admission, the patient's vital signs included a temperature of 97.7°F, a pulse of 116 beats/min, a respiration rate of 18 beats/min, and a blood pressure of 98/60 mm Hg. Examination revealed left facial swelling and a protruding left eye that exhibited afferent papillary defect. The patient had vision loss and her left eye was limited in abduction and adduction. The rest of her physical examination was unremarkable.

Laboratory Test Results

At admission, the patient's metabolic panel revealed the following findings (reference ranges appear in parentheses): blood urea nitrogen, 36 mg/dL (5-20 mg/dL); creatinine, 1.00 mg/dL (0.60-1.00 mg/dL); serum glucose, 250 mg/dL (65-100 mg/dL); chloride, 109 mEq/L (98-107 mEq/L); magnesium, 1.6 mEq/L (1.8-2.4 mEq/L); total protein, 6.0 g/dL (6.4-8.2 g/dL); albumin,1.6 g/dL (3.4-5.0 g/dL); lactate dehydrogenase, 664 U/L (81-234 U/L); alkaline phosphatase, 149 U/L (50-136 U/L); alanine aminotransferase, 83 U/L (12-78 U/L); and aspartate aminotransferase, 54 U/L (10-37 U/L). Hemoglobin A_1c was 8.8% (4.5%-6.2%).

Thyroid-stimulating hormone level was 0.133 mIU/L (0.510-6.270 mIU/L), total triiodothyronine uptake was 47.53% (22.5%-37.0%), and total thyroxine was 5.20 ng/dL (4.50-10.90 ng/dL).

White blood cell count was 3.9 × 10³ μL (4.5 × 10³ μL to 11.0 × 10³ μL); hemoglobin, 10.5 g/dL (12.0-16.0 g/dL); hematocrit, 31.3% (37.0%-47.0%); and platelet count, 28 × 10³ μL (150 × 10³ μL to 400 × 10³ μL). Differential blood cell count revealed 87.1% neutrophils and 6.2% lymphocytes. Manual differential count showed 87 segmented neutrophils (33-66) and 2 bands (0-10). Absolute neutrophil count was 3855 cells/μL. Total serum iron level was 144 μg/dL (50-175 μg/dL), with a ferritin level of 246 ng/mL (10-291 ng/mL).

Findings from the patient's urinalysis and blood cultures were unremarkable.

Imaging

One month after admission (October 2012), a chest radiograph revealed no abnormalities. A magnetic resonance image showed new, expansible fluid collection in the left infraorbital fat and opacification of the left maxillary sinus not present at admission (Figure 1). A few opacified left ethmoid air cells were also present. The right frontal, maxillary, and sphenoid sinuses remained clear. No changes were observed in the calcified lesion.

Figure 1.

Magnetic resonance image showing (A) opacification of the left sphenoid sinus and (B) increased intensity of the left orbit suggesting edema in the infraorbital fat.

Treatment

The patient was hospitalized and treated for hyperglycemia and thrombocytopenia. She continued to receive steroids and highly active antiretroviral therapy and was evaluated by specialists in the hematology, neurology, ophthalmology, and otolaryngology departments. Bone marrow biopsy findings revealed no evidence of increased blasts, eosinophilia, monocytosis, or a lymphoproliferative disorder. Subconjunctival biopsy findings showed a minute fragment of conjunctiva containing a capillary hemangioma with mild acute conjunctivitis.

Endoscopic evaluation of the maxillary sinus and middle turbinate revealed necrotic blackened tissue (Figure 2). The necrotic tissue of the middle turbinate was removed and sent to the pathology department for evaluation. Fluid was drained out of the maxillary sinus, and the necrotic tissues were débrided. Findings from endoscopic evaluation of the left frontal, sphenoid, and right-sided sinuses were unremarkable.

Figure 2.

Endoscopic image showing necrosis of the middle turbinate.

At microscopic examination, the biopsy specimen showed fungal forms infiltrating into bone. The sinus fluid showed necrotic tissue and a blood clot containing fungal forms that resembled mucormycosis and bone fragments with fungal invasion (Figure 3). The patient received amphotericin B (50 mg/d intravenously).

Figure 3.

Microscopic image from the left-middle turbinate showing broad, irregular, nonseptate hyphae, which are characteristic of mucormycosis.

Clinical Course

Despite treatment, the patient's condition deteriorated. In December 2012, a magnetic resonance image showed multiple small lesions with vasogenic edema involving the supratentorial areas bilaterally and the cerebellum and brainstem. Air-fluid levels were present in the maxillary sinuses bilaterally. Mucosal thickening was present in the left maxillary sinus, ethmoid air cells, and sphenoid and frontal sinuses. The patient died at the end of December 2012.