Introduction Immunoglobulin G4-related disease (IgG4-RD) is a systemic, immune-mediated fibroinflammatory condition characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and storiform fibrosis (1). While it can affect nearly any organ system, the ocular adnexa are involved in up to 25–40% of
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