Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - Case report

Author:

Bittencourt Maraya de Jesus Semblano1,Dias Carolina Moraes2,Lage Thaiane Lima2,Barros Renata Silva2,Paz Otavio Augusto Gomes3,Vieira Waldonio de Brito4

Affiliation:

1. Federal University of Para, Brazil

2. Dermatologist, Private Practitioner – Belém (PA), Brazil.

3. Rheumatologist Private Practitioner – Belém (PA), Brazil.

4. Radiologist, Private Practitioner – Belém (PA), Brazil.

Abstract

Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. Different types of vessels, predominantly veins, can be affected in Behçet's disease. The frequency of vascular lesions in Behçet's disease, such as superficial and deep venous thromboses, arterial aneurysms and occlusions, ranges between 7-29%. Budd-Chiari syndrome is a rare and serious complication of Behçet's disease and implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. We report a case of a 25-year-old man with Behçet's disease that developed Budd-Chiari syndrome. The correlation of dermatological, pathological and imaging studies confirmed the diagnosis.

Publisher

FapUNIFESP (SciELO)

Subject

Dermatology

Reference10 articles.

1. Neutrophilic dermatoses: part I;Bonamigo RR;An Bras Dermatol,2011

2. Criteria for diagnosis of Behçet's disease;Lancet,1990

3. Behçet's disease as a model of venous thrombosis;La Regina M;Open Cardiovasc Med J,2010

4. Budd-Chiari syndrome associated with Behçet's disease;Ben Ghorbel I;Gastroenterol Clin Biol,2008

5. Behçet's Syndrome and Thrombosis;Seyahi E;Mediterr J Hematol Infect Dis,2011

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