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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.
Insights
Mikulicz's disease is now understood as part of immunoglobulin G4-related disease. This case report successfully diagnosed and treated a patient with this condition, highlighting its importance in differential diagnosis.
Area of Science:
- Rheumatology
- Immunology
- Ophthalmology
Background:
- Mikulicz's disease is characterized by lymphoplasmacytic infiltrates, IgG4 plasma cell positivity, storiform fibrosis, and eosinophilia.
- It is increasingly recognized as a manifestation of immunoglobulin G4-related disease (IgG4-RD).
Observation:
- A 59-year-old male presented with keratoconjunctivitis sicca and enlarged lacrimal and salivary glands.
- Despite a typical clinical presentation, previous tests did not confirm Mikulicz's disease.
Findings:
- Diagnosis was established through typical clinical signs, elevated serum IgG4 levels, and histopathological examination of lacrimal gland tissue.
- The patient was successfully treated with corticosteroid therapy.
Implications:
- This case represents the first reported instance of IgG4-related Mikulicz's disease in Serbia.
- It underscores the significance of considering IgG4-related Mikulicz's disease in the differential diagnosis of Sjögren's syndrome and lymphoproliferative disorders in rheumatological practice.