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Impaired Cytotoxicity in Papillon-Lefèvre Syndrome

¹ King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia;
² current address, Department of Periodontics, Loma Linda University, 24860 Taylor Street, Loma Linda, CA 92354, USA;
³ University of Kristianstad, Kristianstad, Sweden; and
⁴ Section of Periodontology, College of Dentistry, The Ohio State University, Columbus, OH, USA;

^* corresponding author, tlundgren{at}sd.llu

Papillon-Lefèvre syndrome (PLS), palmoplantar hyperkeratosis with periodontitis, has been genetically characterized. However, suspected associated immune dysfunctions remain elusive. The purpose of this study was to evaluate peripheral blood lymphocyte levels and natural killer (NK) cell cytotoxicity in PLS. Twenty patients and 20 healthy controls were examined. Peripheral blood lymphocytes were analyzed by flow cytometry for surface markers. NK cell cytotoxicity against K562 cells was determined by means of a ⁵¹Cr release assay. White blood cell differential and proportions of B-, T-, T-helper, T-suppressor, and NK cells revealed only sporadic borderline variations from control values. In contrast, NK cell cytotoxicity was consistently and severely depressed (32–53% of control values) in all patients. To the best of our knowledge, this newly described impairment of NK cell cytotoxic function is the first consistent immune dysfunction reported in PLS. This suggests that the impaired NK cell cytotoxicity might contribute to the pathogenesis of PLS-associated periodontitis.

KEY WORDS: natural killer cells • cytotoxicity • Papillon-Lefèvre syndrome

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