Behc ¸et Disease in the Developing World Ilknur Tugal-Tutkun, MD Behc ¸et disease is a multisystem inflammatory disorder named after the Turkish dermatologist, Professor Hulusi Behc ¸et, who described the triple-symptom complex of the disease, including recurrent oral ulcers, genital ulcers, and iritis; as a distinct entity in 1937. 1 Earliest descriptions of a disease that bares similarities to Behc ¸et description date back to the writings of Hippocrates. 2 In addition to the classical triad, Behc ¸et disease is now recognized as a systemic vasculitis leading to a wide spectrum of disease manifestations. 3 Although much has been learned about the clinical characteristics of Behc ¸et disease, the etiopathogenesis of this disorder has not been fully clarified. It is generally accepted that in immunogenetically susceptible individuals, environmental agents may trigger an enhanced and dysregulated immune response resulting in inflammatory vascular injury in many organ systems. 4 An infectious etiology has long been suspected. In his original article, Prof Behc ¸et proposed an unknown virus. 1 Besides herpes simplex virus and hepatitis viruses, a number of bacteria have been implicated as potential infectious triggers. 5 Microbial heat shock proteins bearing a homology with the human mitochondrial heat shock proteins may trigger a cross-reactive immunopathologic response. 6 Abnormalities of toll-like receptors have been shown in Behc ¸et patients. 7 These receptors are involved in the recognition of microbial products and regulation of innate and adaptive immune systems. A dysregulation of both of these immune systems is implicated in the pathogenesis of Behc ¸et disease. 6 Whereas a dysregulated innate immune system is held responsible for the proinflammatory state and unprovoked recurrent inflammatory episodes bearing similarities to the newly designated autoinflammatory syndromes, 4 a dysregulated www.internat-ophthalmology.com | 87 INTERNATIONAL OPHTHALMOLOGY CLINICS Volume 50, Number 2, 87–98 r 2010, Lippincott Williams & Wilkins