Myeloperoxidase deficiency with extensive candidal osteomyelitis of the base of the skull.

A 6-year-old girl had a 7-month history of headaches and painful torticollis. A CT scan of the mastoids showed extensive bone destruction of the base of the skull and C-1. Biopsies of the retropharyngeal area and of the anterior aspect of C-1 were performed: histopathologic findings were suggestive of mycotic infection and cultures were positive for Candida albicans. The child was treated successfully with amphotericin B. The immunologic evaluation demonstrated the absence of myeloperoxidase in the neutrophils.