Atypical Pediatric‐Onset Behçet's Disease Presenting With Superior Vena Cava Thrombosis in an Adolescent Male: A Case Report

Behçet's disease is a chronic, relapsing multisystem vasculitis known for its highly variable clinical spectrum. We report the case of an 18-year-old male who presented with progressive headache and facial swelling. Imaging revealed extensive thrombosis involving the dural venous sinuses, superior vena cava, and left brachiocephalic vein. Despite the absence of ocular involvement or genital ulcers, a diagnosis of Behçet's disease was established based on recurrent oral ulcers, cutaneous findings, vascular thrombosis, and a positive pathergy test, fulfilling the International Criteria for Behçet's Disease. The patient was treated with corticosteroids, azathioprine, colchicine, and rivaroxaban, resulting in clinical and radiologic improvement. This case highlights a rare and aggressive vascular manifestation of Behçet's disease in an adolescent, emphasizing the need for early diagnosis and immunosuppressive management to prevent long-term complications.

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Publication Details

Journal
Clinical Case Reports
Published
2025-12-01
DOI
https://doi.org/10.1002/ccr3.71616
Primary Topic
Ocular Diseases and Behçet’s Syndrome
Type
article
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article

Atypical Pediatric‐Onset Behçet's Disease Presenting With Superior Vena Cava Thrombosis in an Adolescent Male: A Case Report

Shoaib Bin Aleem, Aizaz Ali, Tahmina Begum, Jibran Ikram et al.
Clinical Case Reports
Ocular Diseases and Behçet’s Syndrome
article

Atypical Pediatric‐Onset Behçet's Disease Presenting With Superior Vena Cava Thrombosis in an Adolescent Male: A Case Report

Shoaib Bin Aleem, Aizaz Ali, Tahmina Begum, Jibran Ikram, Abdul Moeez, T.B. Lal, Ubaid Ullah, Tahreem Mari
article en

Abstract

Behçet's disease is a chronic, relapsing multisystem vasculitis known for its highly variable clinical spectrum. We report the case of an 18-year-old male who presented with progressive headache and facial swelling. Imaging revealed extensive thrombosis involving the dural venous sinuses, superior vena cava, and left brachiocephalic vein. Despite the absence of ocular involvement or genital ulcers, a diagnosis of Behçet's disease was established based on recurrent oral ulcers, cutaneous findings, vascular thrombosis, and a positive pathergy test, fulfilling the International Criteria for Behçet's Disease. The patient was treated with corticosteroids, azathioprine, colchicine, and rivaroxaban, resulting in clinical and radiologic improvement. This case highlights a rare and aggressive vascular manifestation of Behçet's disease in an adolescent, emphasizing the need for early diagnosis and immunosuppressive management to prevent long-term complications.

Clinical Case ReportsVol. 13(12)
Cleveland Clinic (US), Khyber Medical College (PK), Lady Reading Hospital (PK), Dow University of Health Sciences (PK)
Openalex Percentile: Top 46%
Ocular Diseases and Behçet’s Syndrome
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