Behçet Disease

  • Erdal Sag
  • Yelda Bilginer
  • Seza Ozen
Part of the Rare Diseases of the Immune System book series (RDIS)


Behçet disease (BD) is a multisystemic autoinflammatory disorder of unknown etiology. BD is most common along the historical Silk Road from Far East Asia to the Mediterranean region. The disease is characterized by recurrent oral/genital ulcers and ocular, gastrointestinal, musculoskeletal, and nervous system involvement. BD is also a unique vasculitis affecting both arteries and veins of all sizes.

There are several sets of classification criteria for BD; however, the International Study Group (ISG) diagnostic/classification criteria and the recently proposed pediatric BD (PEDBD) criteria are the most widely used. There are no specific guidelines for the management of pediatric BD; however, very recently, the new European League Against Rheumatism (EULAR) recommendations for adult BD have been proposed.


Behçet Vasculitis Oral ulcer Genital ulcer Uveitis Sinus thrombosis 


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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Erdal Sag
    • 1
  • Yelda Bilginer
    • 1
  • Seza Ozen
    • 1
  1. 1.Department of Pediatric RheumatologyHacettepe University Faculty of MedicineAnkaraTurkey

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