Beh？et's disease (BD) is a multisystemic, relapsing inflammatory disorder with an obscure etiology and pathogenesis. Diagnosis depends on the clinician's ability to identify a group of nonspecific mucocutaneous lesions, which also manifest in a number of other diseases. In recent years, there has been an increase in the studies focusing on the histopathological aspects of Beh？et's disease diagnostic mucocutaneous lesions. Their results emphasize the value of histopathology and direct immunofluorescence (DIF) in the differential diagnosis of Beh？et's disease. 1. Introduction After seventy-four years, since Dr. Hulusi Beh？et had published his classic paper describing the three major signs [1], BD are still an enigma for clinicians and researchers. Almost all aspects of BD is a source of debate and even its diagnostic criteria, classification, and pathogenesis are controversial [2–4]. In the last two decades, extensive studies have been conducted to reveal the nature of BD. In the light of these studies, BD is now recognized as a chronic, multisystemic vasculitis [2, 5–7]. Whether this vasculitis is a result of autoimmunity is controversial [2, 3], but there is increasing evidence indicating the possible role of immunologic mechanisms in the pathogenesis. Evaluation of lesion-free skin and mucocutaneous lesions of patients with BD patients by DIF reveals immunoreactant deposits on the vessel walls [8–11]. Also, elevated serum levels of several proinflammatory cytokines (IL-1, IL-4, IL-6, TNF-α, etc.) have been reported in these patients [12]. A special subgroup of T lymphocytes (γδ T lymphocytes), which play an important role in mucosal immunity, are found to be present in the increased numbers in circulation and mucosal lesions of patients [13]. Several research groups found out that cultured γδ T lymphocyte cells proliferate when stimulated with mycobacterial heat shock proteins and products of several oral pathogen microorganisms [13, 14]. Correlation between the hyperactive state of neutrophils and BD activity is another well-known fact. The underlying mechanism is unknown. Antigen-presenting cell and T-lymphocyte-derived cytokines and chemokines are believed to be responsible for neutrophiles hyperreactivitiy [15]. BD is mostly encountered in the Mediterranean and Middle and Far East countries, Turkey having the highest prevalence with 80–420？cases/100.000 [3, 16]. Genetic studies show statistically significant association with HLA-B51 [17, 18]. Antiendothelial antigens are another immunological anomaly found in BD patients, but there is no solid
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