A boy with recurrent infections, impaired PMN-chemotaxis, increased IgE concentrations and cranial synostosis--a variant of the hyper-IgE syndrome?

A patient with coarse facies, craniosynostosis, recurrent staphylococcal infections with pneumatocele formation is described. Laboratory features included moderately elevated serum IgE, cutaneous anergy, decreased numbers of T-suppressor cells and variable inhibition of neutrophil chemotaxis. The combination of clinical findings suggests the diagnosis hyper-IgE syndrome, though the total IgE serum concentration (800 U/ml) and the level of IgE-specific antibodies to staphylococci (9.4%, normal less than 5%) were only slightly elevated.