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The typical appearance and CBCT images of the patient with Papillon-Lefevre syndrome: A case report
(University of Dicle, 2015) Tumen D.S.; Tumen E.C.; Gunay A.; Lacin N.; Cetin S.G.
Papillon-Lefevre syndrome is a rare autosomal recessive genetic disorder, which is transmitted with an estimated frequency of one to four per million individuals. It is characterized by palmar-plantar hyperkeratosis, and rapid destruction of the alveolar bone and periodontium of both the primary and permanent dentitions, commencing at the time of tooth eruption. Early diagnosed and rehabilitation of children with Papillon-Lefevre syndrome will go a long way in helping them interact normally and integrate with their peers. However, the rarity with which this entity appears, along with its complex characteristics, often make it difficult to treat. The aim of this case report is to present the early diagnosed of a 5-year-old boy with Papillon-Lefevre syndrome associated with severe periodontitis in which the premature exfoliation of primary maxillary and mandibular incisor teeth and maxillary primary first molars are observed, and with hyperkeratosis of the palms, knees and soles.