Case report of Mikulicz's disease - a modern concept of an old entity.

Introduction. Modern knowlegde defines Mikulicz's disease as a part of immunoglobulin G4-related disease. The main feature is the presence of lymphoplasmacytic infiltrates, immunoglobulin G4 plasma cells positivity, distinctive storiform fibrosis and moderate eosinophilia. Case report. A 59-years old male presented with a mild keratoconjuctivitis sicca and enlarged lacrimal and salivary glands during the last two years. Althought clinical presentation of the patient was typical, earlier testing did not pinpoint Mikulicz 's disease. By typical clinical presentation, elevated serum immunoglobulin G4 level and histopathological finding of lacrimal glands tissue we diagnosed Mikulicz's disease successfully treated with corticosteroid therapy. Conclusion. We reported the first case of IgG4-related Mikulicz's disease in Serbia. Our report highlights IgG4-related Mikulicz`s disease as an important differential diagnosis with Sjögren`s syndrome and lymphoproliferative disease in rheumatological practice. [ABSTRACT FROM AUTHOR]