Fatal Septicemia in an Infant with Keratitis, Ichthyosis, and Deafness (KID) Syndrome


Address correspondence to Mary L. Williams, M.D., Box 0316, Department of Dermatology, University of California, San Francisco, San Francisco, CA 94143-0316.


Abstract: Keratitis, ichthyosis, and deafness (KID) syndrome is a rare congenital disorder of unknown etiology in which increased susceptibility to viral, bacterial, and mycotic infections has been observed. We report an infant with KID syndrome who died from overwhelming systemic infection. To date, investigations into the immune function of patients with this syndrome have not revealed a common underlying systemic immune deficit. However, the severity of infections and multiplicity of organisms observed in this syndrome suggest that a primary immunodeficiency is present in addition to an impaired cutaneous barrier to microorganisms.