Journal of the European Academy of Dermatology and Venereology ELSEVIER 6(1996)246-251 Case report Papillon-Lefevre syndrome associated with albinism. Clinical and immunological study Mohammed Amer ^•% Fawzia Farag Mostafa ', Zenab Tosson \ Ahmmed Hegazi ^ From the Department of Dermalology. Unirersity of Zagazig. Faculty of Medicine. Zagazig. Egypt ' National Research Center Dokki, Cairo, Egypt Abstract Background: We report the first association of Papillon-Lefevre syndrome (PLS) with tyrosinase-nega- tivc albinism in two brothers. Also, we studied T-lymphocytes and mononuclear celi activity in them and in two other cases of PLS not associated with albinism. Objective: To recognize clinical and immunological correlation. Methods: Four PLS patients from 3 different families were studied clinically and immunologically. Two brothers (albino) in one family had recurrent infections. The other two patients from two other families had no infection. The patients' healthy parents were also included in the study. Results: A decreased lymphocyte proliferative response to phytohaemaglutinin and in mononuclear cell activities were detected not only in the PLS patients with recurrent pyogenic infection, but also in their healthy parents. Further investigations of these patients showed impaired humoral and cellular immune response. PLS patients with no infection and their parents showed normal immunoiogic parameters. Conclusion: We suggest dividing PLS on clinical and immunological bases into two variants: PLS with pyogenic infections and the other variety without such infection. PLS with pyogenic infections is associated with immunologic dysfunction. Periodontosis could not be explained on an immunological basis. Keywords: Papillon-lefevre; Keratosis palmoplantaris 1. Introduction and Lefevre [1] in 1924. The syndrome is inher- ited as an autosomal recessive trait and its frc- The syndrome of keratosis palmoplantaris with quency has been estimated at approximately one periodontopathy, was first described by Papillon to four per million persons in the general popula- tion [2,3]. There is evidence that this syndrome is associated with increased susceptibility to infec- tions in about 20% of patients [3]. No cases of • Corresponding author. pigment abnormalities have been reported in the 0926-9959/96/513,00 £ 1996 Eisevier Science B.V. All rights reserved SSDI 0926-9959(95)00165-4