IgG4-RD-Associated Mikulicz Syndrome Without Classic Systemic Involvement-A Case Report.

ABSTRACT: Background: IgG4-related disease is a rare, chronic inflammatory disorder characterized by lymphoplasmacytic infiltration, 'storiform' fibrosis, and elevated IgG4 levels in affected tissues. This disease has a broad and heterogeneous clinical spectrum that includes four main phenotypes: pancreatic-hepatobiliary disease, retroperitoneal/aortic fibrosis, head and neck disease, and Mikulicz syndrome. Case Description: An 85-year-old male patient with a clinical presentation, which is unusual outside Asia, of IgG4-related disease phenotype Mikulicz syndrome, characterized by bilateral dacryoadenitis, orbital pseudotumor, and no evidence of significant systemic participation. Despite extensive involvement in the orbital and glandular region, the patient did not develop serious org

SUBMITTER: Mendoza-Vargas LA 

PROVIDER: S-EPMC11818687 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature