musculoskeletal and connective tissue disorders
Behçet Disease
Introduction
Behçet disease is a multisystem, relapsing, chronic vasculitic disorder with mucosal inflammation. Common manifestations include recurrent oral ulcers, ocular inflammation, genital ulcers, and skin lesions. The most serious manifestations are blindness, neurologic or gastrointestinal manifestations, venous thromboses, and arterial aneurysms. Diagnosis is clinical, using international criteria. Treatment is mainly symptomatic but may involve corticosteroids with or without other immunosuppressants for more severe manifestations. (See also Overview of Vasculitis .) Behçet disease is an inflammatory disorder that can include a vasculitis of small and large arteries and/or veins. Arterial and venous thrombosis may occur as well. The disease occurs nearly equally in men and women but tends to be more severe in men, typically beginning during their 20s. Occasionally, the disease develops in children. Incidence varies by location. Behçet disease is most common along the silk route from the Mediterranean to China; it is uncommon in the US. The cause of Behçet disease is unknown. Immunologic (including autoimmune) and viral or bacterial triggers have been suggested, andHLA-B51is a major risk factor. Prevalence of an HLA-B51 allele is > 15% among people from Europe, the Middle East, and the Far East but is low or absent among people from Africa, Oceania, and South America. Neutrophil infiltration is detected in biopsy specimens from oral aphthous ulcers and erythema nodosum and pathergy lesions, but no histologic changes are pathognomonic. 