Kawasaki disease (KD) is a rheumatic disease that occurs during childhood. Although T cells have been implicated as having an important role in the pathogenesis of KD, the role of B cells is unclear. To detect preferential expression of 11 amino acid complementarity determining region (CDR)3 domains, we used two-stage PCR (polymerase chain reaction) to analyze the CDR3 lengths of VkIII-derived immunoglobulin kappa light chains expressed in peripheral blood B cells during the acute, subacute, and convalescent phase of this disease. As controls, the peripheral blood B cells of age-matched normal and children with acute febrile diseases other than KD were tested. In 5 of 7 KD patients, expression of kappa light chains containing 11 amino acid codon CDR3 intervals was increased during the acute and subacute phase of KD but decreased during the convalescent phase. Two of the 7 KD patients showed the same pattern during the subacute and convalescent phase, but not during the acute phase. Two of the 5 patients with acute febrile diseases other than KD showed increased expression of kappa chains with 11 amino acid codon CDR3 intervals, but it was not a major fraction. Three of the 5 patients with acute febrile diseases other than KD and all normal control subjects showed only 9 and 10 amino acid CDR3 domains. These results strongly suggest that B cells expressing kappa light chains with the 11 amino acid CDR3 domains might be involved in the pathogenesis of KD.