Immunological alterations in a case of Papillon-Lefèvre syndrome with recurrent cutaneous infections

An immunological study was performed in a 16-year-old boy affected with Papillon-Lefèvre syndrome (PLS) and recurrent staphylococcal cutaneous infections. A defect of neutrophil intracellular killing of Staphylococcus aureus and a decreased lymphocyte proliferative response to phytohaemagglutinin were detected. The possible role of these alterations in promoting recurrent infections in PLS is discussed.