Behçet syndrome

Also known as: Behçet disease; Silk Road disease

Variable-vessel vasculitis with recurrent oral and genital ulcers and uveitis.

Medical specialty: Rheumatology
Subspecialty: Rheumatology / Vasculitis
Sex & age distribution: Male: affected, more severe; Female: affected; Children: yes, rare
Gene(s) involved: CCR1, ERAP1, HLA-B*51, IL10, IL12A, IL23R, STAT4

Etiology & mechanism

Autoinflammatory-autoimmune process with HLA-B51 link.

Gene(s) involved (7)

CCR1ERAP1HLA-B*51IL10IL12AIL23RSTAT4

Symptoms & signs (8)

arthritisgenital ulcersneuro-behçetpathergyrecurrent oral ulcersskin lesionsuveitisvenous thrombosis

SyndromeScope — educational reference. Content is model-generated and not clinically verified; always confirm against OMIM, Orphanet and GeneReviews before clinical use.