Characteristically pathognomonic findings are only those that can be evidently measurable at quiescent phases. Adamandiades-Behçet syndrome (or Behçet's disease) is a rare idiopathic multisystem disease of uncertain nature. Although the upper hand was usually given to autoimmunity, Adamandiades- Behçet syndrome (ABS) is, paradoxically, ANA negative. Autoantibodies do not play a major role in its pathogenesis, nor do B cells. Now that autoimmunity cannot full explain ABS, it is recently considered midway between autoinflammation and autoimmunity. There are no diagnostic laboratory tests for ABS, and laboratory fndings typically refect ongoing infammation. In this study, twelve clinically quiescent cases were scheduled for incisional labial salivary gland biopsies after testing positively for the pathergy test. The biopsies were microscopically examined to describe some characteristic findings, in contrast to normal mucosa. Four immunohistochemical markers were used: Cathepsin K, CD68, CD34 and CD16b. The immunohistochemical findings could provide decisive information in differentiating Behçet's disease from Crohn's disease because Cathepsin-K, a sensitive immunohistochemical marker for detection of micro-granulomas, is natively negative. These procedures are accordingly suggested to be integrated into a diagnostic protocol of Behçet's syndrome.
Keywords: Behçet's disease, minor salivary gland biopsy, dusty acini, new diagnostic protocol