Recurrent pyoderma in a family with a defect in leucocyte locomotion

Granulocyte functions including leucocyte locomotion and chemoluminescence were studied in three generations of a family in which all male members had presented with recurrent pyoderma. While parameters of humoral immunity including serum concentrations of IgG, IgA, IgM, and IgE and of complement components C3 and C4 as well as the response of mononuclear leucocytes to mitogens proved to be within the normal range, leucocyte locomotion was found to be severely impaired in all affected subjects. Moreover, granulocyte dysfunction in male members was associated with the occurrence of a single haplotype (HLA-A2, B13, DR7). These findings suggest that the defect in leucocyte locomotion and the pyoderma might not only have been inherited in an X linked manner but might also have been linked to a gene within the inherited HLA haplotype.