Behçet's disease as a systemic disease

Clin Dermatol. 2014 May-Jun;32(3):435-42. doi: 10.1016/j.clindermatol.2013.11.012. Epub 2013 Nov 23.


Behçet's disease usually begins with cutaneous manifestations, such as recurrent aphthous stomatitis, genital ulcers, erythema nodosum-like lesions, papulopustular findings, and pathergy phenomenon. Recurrent aphthous stomatitis is generally the first sign, and other findings may develop in the course of the disease. There is no specific diagnostic available for Behçet's disease. It is most prevalent among patients along the ancient Silk Road. The high frequency of HLA-B51 among a wide range of ethnic populations favors the role of genetic factors. Behçet's disease usually appears in the third to fourth decade of life, and is rarely seen in children and adults over 50 years of age. It affects both genders equally, but the course of the disease is more severe in men. Eye involvement leading to loss of vision, plus vascular, articular, and central nervous system involvement are more commonly observed among men. Behçet's disease is a systemic inflammatory disorder. A complex genetic background, coupled with innate and adaptive immune system activation, causes the diverse clinical manifestations that characterize the clinical picture.

Publication types

  • Review

MeSH terms

  • Arthralgia / complications
  • Arthritis / complications
  • Behcet Syndrome / complications*
  • Behcet Syndrome / epidemiology
  • Behcet Syndrome / genetics
  • Behcet Syndrome / pathology*
  • Central Nervous System Diseases / complications
  • Humans
  • Inflammation / complications*