Combined abnormality of neutrophil chemotaxis and bactericidal activity in a child with chronic skin infections

Abnormalities of neutrophil chemotaxis or bacterial killing are seen in some patients with recurrent infections. Here we report a 9-year-old boy who has chronic otitis media, periodontitis and severe, staphylococcal skin infections. The skin infections beging as painful vesicles which contain Staphylococcus aureus and very few neutrophils. These lesions rapidly ulcerate, enlarge and, in the chronic phase, resemble pyoderma gangrenosum. The patients' neutrophil chemotaxis in vitro was consistently abnormal. In addition, the patients' neutrophils were markedly defective in the intracellular killing of S. aureus, Escherichia coli, and Streptococcus faecalis. However, nitroblue tetrazolium dye reduction, hexose monophosphate shunt activity, and hydrogen peroxide generation were normal. These results suggest that the bactericidal defect may involve nonoxidative pathways. This patient raises the possibility that optimal neutrophil chemotaxis and bacterial killing both require the function of one or more common pathways. By studying patients with such combined neutrophil abnormalities, we may better understand the molecular basis of chemotaxis and bacterial killing.