Behçet syndrome, Behçet disease

(bā′sĕt, be-chet′)

[Hulusi Behçet, Turkish dermatologist, 1889-1948]
A rare, multisystem, chronic, recurrent disease of unknown cause, marked by ulceration of the mouth and genitalia and by uveitis. The central nervous system, blood vessels, joints, and intestinal tract may be involved. It is genetically associated with HLA-B51. The disease occurs worldwide but is most common in the eastern Mediterranean and eastern Asia, where it occurs mostly in young men and is a leading cause of blindness. In the western world, where the disease is less severe, it affects men twice as frequently as it does women but is not a leading cause of blindness. The period between attacks is irregular but may be as short as days or as long as years.

Therapy depends on the severity of the clinical findings. Mild disease of skin and joints may be treated with topical steroids or nonsteroidal anti-inflammatory drugs. Involvement of the central nervous system or gastrointestinal tract may require high dose steroids or cytotoxic drugs, such as chlorambucil, cyclophosphamide, or methotrexate.

SEE: silk road disease

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