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Published on: April 13, 2018
Behçet's disease presenting as deep venous thrombosis and priapism
A Ateş1, O Tiryaki Aydintuğ, N Düzgün
1Departments of Clinical Immunology and Rheumatology, Ankara University Faculty of Medicine, Ankara, Turkey. ates@medicine.ankara.edu.tr
Insights
Behçet
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Behçet's disease (BD) is a systemic inflammatory condition affecting multiple organs, including the vasculature.
- Current diagnostic standards rely on the International Study Group (ISG) criteria.
- Vascular manifestations occur in 25-50% of BD patients, sometimes as the sole presenting feature.
Observation:
- A case report details a patient presenting with deep venous thrombosis and priapism.
- This patient did not meet the established ISG criteria for Behçet's disease diagnosis.
Findings:
- Large vessel disease, a significant vascular manifestation, is notably absent from the ISG diagnostic criteria.
- The case highlights diagnostic challenges when vascular symptoms are prominent but do not align with current criteria.
Implications:
- The findings suggest a potential limitation in the ISG criteria for diagnosing Behçet's disease, particularly in cases with significant vascular involvement.
- Revising diagnostic criteria to include major vascular events may improve early identification and management of Behçet's disease.
Abstract:
Behçet's disease (BD) is a multi-system inflammatory disorder which may involve the vascular system. Currently, it is general practice to use the International Study Group (ISG) criteria for the diagnosis of BD. However, even though vascular involvement may be seen in one-fourth to one-half of BD patients, and occasionally is the presenting and only manifestation of BD, large vessel disease is not included among the ISG criteria. In this report we describe a patient who had deep venous thrombosis and priapism, but who does not fulfill ISG criteria for the diagnosis of BD.
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