Immunoglobulin G4-related disease of the orbital cavity, cervical lymph nodes and greater auricular nerve: case report.

IgG4-related disease (IgG4-RD) is a novel clinicopathological entity characterised by elevated tissue levels of IgG4-positive plasma cells. It can present in almost every organ systems. We present a case of a 48year-old man with recurrent intra-orbital and cervical lymph node swelling and found to have greater auricular nerve involvement intraoperatively during open surgical biopsy. Histopathological evaluation of biopsied specimens from these lesions yielded IgG4-positive plasma cell infiltration on immunohistochemistry. Key pathological features such as prominent lymphoplasmacytic population, storiform fibrosis and obliterative phlebitis were also seen. A diagnosis of IgG4-RD was made. Oral prednisone therapy ameliorated the symptoms and patient remained in remission at followup. Literature review indicated that IgG4-RD is a rare condition that seldom occurs concurrently in the orbital cavity, cervical lymph nodes and involving the greater auricular nerve. The condition may often masquerade as malignancy or infection due to formation of tumefactive lesions but tend to respond favourably to glucocorticoid or immunosuppressants. The differential diagnosis of unusual mass lesions in these locations should include IgG4-RD. The otolaryngologist, as well as other health professionals, should be familiar with this novel disease to ensure timely diagnosis and treatment.