A 4-year-old Japanese boy with Rothmund-Thomson syndrome suffered from seve
re herpes encephalitis at 5 months of age. The serum level of immunoglobuli
n G was low and the responsiveness of peripheral blood mononuclear cells to
bacterial superantigens was poor. It was suggested that these immunologica
l abnormalities, possibly associated with Rothmund-Thomson syndrome, led to
severe infection with herpes simplex virus in our patient.