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

Ksenija Svetozar Bozic; Branislava Glišić; Olga Radić-Tasić; Bojana Knežević

doi:10.2298/7523

Ksenija Svetozar Bozic Clinic of Rheumatology, Military Medical Academy, Belgrade, Serbia;
Branislava Glišić Clinic of Rheumatology, Military Medical Academy, Belgrade, Serbia, Faculty of Medicine of the Military Medical Academy, University of Defence, Belgrade, Serbia
Olga Radić-Tasić Institute of Pathology, Military Medical Academy, Belgrade, Serbia
Bojana Knežević Clinic of Rheumatology, Military Medical Academy, Belgrade, Serbia

DOI: https://doi.org/10.2298/7523

Keywords: mikulicz' disease, diagnosis, differential, lacrimal apparatus, salivary glands, immunoglobulin G, histological techniques, glucocorticoids, treatment outcome,

Abstract

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.

Author Biography

Ksenija Svetozar Bozic, Clinic of Rheumatology, Military Medical Academy, Belgrade, Serbia;

Clinic of Rheumatology

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