The current study tested the hypothesis that the gastrointestinal tract could be one of the primary sites of entry for etiologic agents in Kawasaki disease (KD). In an attempt to elucidate the pathogenic role of certain superantigenic agents in KD, T cell receptor V beta expression by T cells in the small intestinal mucosa of KD patients was investigated using MAb on frozen tissue sections. Twelve Japanese patients with KD and eight controls were enrolled in the study. The numbers of cells stained by an immunofluorescence from each study group were counted and analyzed statistically by the t test. The occurrence of V beta 2+ T cells was found to be selectively increased in the small intestinal mucosa of patients in the acute phase of KD compared with controls (p < 0.01). In our previous study, five kinds of streptococci and two kinds of staphylococci, not detected in control patients, were isolated from the lumen of the jejunum of KD patients. These data suggest that the increased occurrence of V beta 2+ T cells in the jejunal mucosa of KD patients may be caused by exotoxins acting as superantigens produced by bacteria colonizing the small intestinal mucosa of these patients.