A Case Report: Papillon Lefevre Syndrome

Study Background: Papillon Lefevre Syndrome (PLS) is an autosomal recessive inherited genetic disorder characterized bypalmoplantar hyperkeratosis and premature loss of deciduous and permanent teeth. Mutation of cathepsin C gene has beendetected in the patient with PLS. In this case report, oral and radioghaphic findings of one PLS patient were discussed. 14 yearold male patient diagnosed with PLS after radiographic, dermatologic and dental examination was referred to our clinic dueto severe gingival inflamation and mobility. Severe gingival inflamation, alveolar bone resorption and multiple teeth loss dueto periodontal reasons were observed. Mild palmar and moderate plantar hyperkeratosis were observed but no other systemicproblems were detected.Methods: Non-surgical periodontal treatment was perfomed among multiple sessions and in each session subgingival tissueshad been irrigated with %2 chlorhexidine irrigation solution. To reduce severe gingival inflammation antibiotherary has beenprescribed. Hopeless teeth were extracted.Results: After non-surgical periodontal treatment and antibiotherary severe gingival inflammation was reduced but not eliminatedcompletely, mobility score of some teeth reached to zero score, patient was motivated about oral hygiene and was taken intomaintanence phases. After having extracted hopeless teeth patient was directed for prosthodontic rehabilitation.Conclusion: PLS is a rare autosomal recessive disorder. With PLS-specific dermatologic findings and characteristic periodontalview, it is possible to be able to identify the disease in its very early stages. Early diagnosis of the disease and institution of anappropriate periodontal and antimicrobial treatment might improve the prognosis.