Issue: Vojnosanit Pregl 2016; Vol. 73 (No. 4)

Case report of Mikulicz`s disease – a modern concept of an old entity

Authors:
Ksenija Božić, Branislava Glišić, Olga Radić-Tasić, Bojana Knežević

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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.