IgG4-related disease presenting as central skull base osteomyelitis with deep neck abscesses: A case report

IgG4-related disease (IgG4-RD) is an immune-mediated, systemic chronic inflammatory condition that can arise in the head and neck as a tumor-like mass. We report the first case of IgG4-RD manifesting as central skull base osteomyelitis with deep neck abscesses.

A 68-year-old Malay female with diabetes presented with a 2-month history of worsening parietal headache radiating to the neck, intermittent fever, and left-sided tinnitus.

Otoscopic and cranial nerve examinations were normal, while nasoendoscopy revealed an erythematous nasopharynx with slight bilateral obliteration of the Fossa of Rosenmüller; however, biopsies showed no malignancy. Blood investigations revealed hyperglycemia and elevated inflammatory markers, while imaging revealed central skull base osteomyelitis with chronic retropharyngeal and parapharyngeal abscesses. Despite initial symptomatic improvement with intravenous ceftriaxone and glycemic control, our patient presented a month later with left facial nerve palsy (House-Brackmann Grade IV), worsening bilateral hearing loss (right moderate-to-severe mixed hearing loss and left moderate-to-profound mixed hearing loss), and hyperglycemia. A deep biopsy of the retropharyngeal lesion under general anesthesia revealed a diagnosis of IgG4-RD.

Treatment with oral prednisolone resulted in symptomatic improvement and resolution of the deep neck abscesses. Azathioprine was started after steroid was tapered over 2 months but discontinued due to the patient developing headache.

The patient made good symptomatic recovery although she developed right sigmoid sinus thrombosis at 5 months follow-up, for which she was started on warfarin. At 18 months follow-up, she remained well with slight facial weakness (House-Brackmann Grade II) and improved hearing (mild-to-moderate bilateral sensorineural hearing loss).

Our case highlights central skull base osteomyelitis with deep neck abscesses as a new clinical manifestation of IgG4-RD. IgG4-RD may not always present as a tumor-like mass de novo but instead presents with features of an infection in immunosuppressed individuals. In patients with treatment-refractory skull base osteomyelitis and deep neck abscesses, IgG4-RD should be considered as a differential diagnosis and a tissue biopsy is warranted.