A Familial Defect of Neutrophil Chemotaxis with Asthma, Eczema, and Recurrent Skin Infections

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Abstract
Summary: A defect in chemotaxis of peripheral blood polymorphonuclear leukocytes (PMN's) was demonstrated in both parents and three of four children in a single family afflicted with varying degrees of respiratory allergy, unusual onset of severe eczema in the first month of life, and recurrent bacterial skin infections. Of great interest was the identification of HLA-B12 at the B locus in all affected members but not in the unaffected child. The two children known since infancy to be most severely affected with eczema and recurrent infections are HLA identical and homozygous for HLA-B12. The child without eczema and infections had an intermediate cellular chemotactic defect most apparent on kinetic studies. Speculation: Recently, several familial defects of neutrophil chemotaxis have been reported in association with syndromes of recurrent bacterial infections, reaginic hypersensitivity, and/or clinical atopy. Our studies extend these observations by describing the possible genetic relationships between atopy (e.g., eczema) chemotactic defects, and the HLA system. Future studies in this field should examine the association between chemotactic defects and the HLA system, and the requirements of both atopy and chemotactic defects to produce recurrent infections.