Immunoregulatory abnormalities in mucocutaneous lymph node syndrome

The immune status of 21 children in the acute phase of mucocutaneous lymph node syndrome (MCLS) was assessed and compared to that of control populations consisting of age-matched normal children and of children suffering from acute febrile nonbacterial illnesses. In contrast to the controls, 13 of 18 patients studied during the acute phase of MCLS had a significant reduction in circulating T8-positive (T8+) suppressor/cytotoxic T cells (P 0.05). Three of three patients were found to have a significantly increased number of Ia-bearing T4+ T cells, suggesting the presence of circulating activated helper cells. Furthermore, 17 of 18 patients with acute MCLS had a significantly elevated number of circulating cells spontaneously secreting IgG (P < 0.01) and IgM (P < 0.001) as determined by a reverse hemolytic plaque assay. Finally mononuclear cells from 18 of 20 patients with acute MCLS had increased cytotoxicity against 51Cr-labeled normal human skin fibroblasts (P < 0.01). Follow-up studies during the convalescence phase of MCLS indicated that most of the patients had gradual resolution of their immunologic abnormalities. These results suggested that immunoregulatory abnormalities may contribute to the pathogenesis of this syndrome.