A case report: Papillon Lefevre Syndrome
Istanbul University, Turkey
: Dent Health Curr Res
Aim: This article aims to present an advanced therapeutic approach based entirely on adhesive dentistry.
Objective: Papillon Lefevre Syndrome (PLS) is an autosomal recessive inherited genetic disorder characterized by palmoplantar hyperkeratosis and premature loss of deciduous and permanent teeth. Mutation of cathepsin C gene has been detected in the patient with PLS. In this case report, oral and radioghaphic findings of one PLS patient were discussed.
Case: Fourteen year old male patient diagnosed with PLS after radiographic, dermatologic and dental examination was referred to our clinic due to severe gingival inflammation and mobility. Severe gingival inflammation, alveolar bone resorption and multiple teeth loss due to periodontal reasons were observed. Mild palmar and moderate plantar hyperkeratosis was observed but no other systemic problems were detected. After non-surgical periodontal treatment and antibiotherapy severe gingival inflammation was reduced but not eliminated completely, mobility score of some teeth reached to zero score; patient was motivated about oral hygiene and was taken into maintenance 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 periodontal view, it is possible to be able to identify the disease in its very early stages. Early diagnosis of the disease and institution of an appropriate periodontal and antimicrobial treatment might improve the prognosis.
Rumeysa Cihangir was graduated from Istanbul University, Faculty of Dentistry and she is a PhD student at Istanbul University, Faculty of Dentistry, Department of Periodontology. Her PhD dissertation will be about neutrophil enzyme alterations of gingival crevicular fluid at smoker gingivitis patients. This study is still on progress. She has participated in many domestic congresses.