Backgroung/Aim: Papillon Lefévre syndrome is a rare autosomal recessive genodermatosis. The characteristic findings of the disease are early loss of primary and permanent teeth and palmoplantar keratoderma. Notwithstanding that many etiologic factors like genetic mutations, bacterial agents, immunologic changes have been identified, the pathogenesis has not been fully understood. Although dentists play an important role in the diagnosis and treatment of Papillon Lefévre syndrome, it is appropriate to treat the disease with a multidisciplinary approach.
Case Report: In this case report, the clinical, radiological and genetic examination of the patient with Papillon Lefévre syndrome who has a homozygous mutation in the CTSC gene will be presented.
Conclusions: Dentists should have knowledge about treatment management of these patients. Teeth can be preserved longer with early diagnosis and appropriate treatment of the disease.