Papillon-Lefèvre syndrome. Analysis of peripheral blood lymphocyte subsets

We have studied the peripheral blood lymphocyte populations in our 6 patients (2 female and 4 male) with a mean age of 11.19 with Papillon-Lèfevre Syndrome (PLS) using adequate monoclonal antibodies and double coloured flow cytometry. Total B, T, CD4, CD8, CD29, CD45RA, NK, HLA-DR cells were studied. Total B, T, CD4 and CD8 lymphocytes were within normal limits. We have observed an increase in the CD29 lymphocytes and NK cells and a decrease in CD45RA lymphocytes. We think that these findings are important in explaining B lymphocyte activation and in the pathogenesis of the PLS.