%0 Journal Article
%T Genetics of Beh£¿et's Disease
%A Tamer £¿rfan Kaya
%J Pathology Research International
%D 2012
%I Hindawi Publishing Corporation
%R 10.1155/2012/912589
%X Beh£¿et's disease (BD) is a systemic inflammatory disorder characterized mainly by recurrent oral and genital ulcers and eye involvement. Although the pathogenesis remains poorly understood, a variety of studies have demonstrated that genetic predisposition is a major factor in disease susceptibility. Peculiar geographical distribution of BD along the ancient Silk Road has been regarded as evidence supporting genetic influence. The observed aggregation of BD in families of patients with BD is also supportive for a genetic component in its etiology. HLA-B51 (B510101 subtype) is the most strongly associated genetic marker for BD in countries on the Silk Road. In recent years, several genome-wide association studies and genetic polymorphism studies have also found new genetic associations with BD, which may have a supplementary role in disease susceptibility and/or severity. The author reviewed the HLA and non-HLA genetic association studies. 1. Introduction Beh£¿et¡¯s disease (BD) is a complex syndrome characterized mainly by recurrent oral aphthous ulcers, genital ulcerations, and ocular involvement. This triple-symptom complex was first described by Hulusi Beh£¿et, a Turkish dermatologist, as a separate disease entity. Later, other associated clinical features were described [1, 2]. Although the aetiology is still obscure, BD is believed to be triggered by environmental factors such as microbial agents in individuals with a particular genetic background. Interactions of genetic and environmental factors in BD patients may underlie pathogenic processes, which may influence the development of the disease and modify its course. The prevalent distribution in a specific geographical area, the close association with HLA-B51 in different ethnic groups, and the familial clustering of BD are hallmarks accounting for the strong contribution of a genetic background [2, 3]. Here we review the current knowledge of the genetic basis of BD and their implications in pathogenesis of BD and summarize the important findings in Table 1. Table 1: Findings supporting the genetic background of Beh£¿et¡¯s disease. 2. Geographical Distribution The prevalence of BD is known to be high in Japan, China, Turkey, and the Mediterranean and the Middle Eastern countries. It is hypothesized that this geographical distribution of BD from Japan to the Middle East and the Mediterranean basin correlates with the distribution of HLA-B51 by old nomadic or Turkish tribes via the ancient Silk Road. This peculiar geographical distribution have been regarded as evidence supporting genetic influence on
%U http://www.hindawi.com/journals/pri/2012/912589/