Report of a case of Papillon-Lefevre syndrome

Abstract

Papillon-Lefevre Syndrome is a rare, autosomal recessive disorder. It is characterized by dermatological and periodontal changes that include palmar-plantar hyperkeratosis and generalized aggressive periodontitis in both primary and permanent dentition. Hiam-Munk syndrome and hypophosphatasia are some of its differential diagnoses. Dentists and dermatologists both should be aware of its characteristics which help to have an early diagnosis, a multidisciplinary approach and better prognosis. Here, we present a case of Papillon-Lefevre Syndrome in a 4-year-old girl who was brought to a private dental office with the chief complaint of tooth mobility.