Papillon-Lefevre syndrome treated by acitretin: case report and cytokine profile.

Papillon-Lefevre Syndrome (PLS) is (OMIM 245'000) a rare genodermatosis, transmitted in an autosomal recessive mode. The disease results from mutations in the CTSC gene (11q14.2) encoding cathepsin C or dipeptidyl peptidase1 . The clinic is marked by recurrent gingivitis, tooth loss and palmoplantar hyperkeratosis. We report the case of a 19-year-old patient who presented with palmoplantar hyperkeratosis and erythemato-squamous plaques (figure 1), with involvement of knees and elbows. The patient had recurrent abscesses mainly on inguinal and axillary areas.