PAPILLON LEFEVRE SYNDROME: A CASE CHALLENGE AND LITERATURE REVIEW

A rare autosomal recessive disease Papillon lefevre syndrome is caused by mutation in the cathepsin C gene which is positioned at chromosome 11q14.1-q14.3 and other etiological factors such as endocrinopathy, generalized epithelial dysplasia and vitamin A deficiency, genetic, immunologic and microbiologic. It is presented with early loss of permanent and primary teeth, hyperkeratosis of hands and feet and aggressive periodontitis. Diagnosis is made on basis of history, clinical examination, urine analysis and genetic mapping. The cases discussed have esthetic and functional concerns. Our cases were presented with initial loosening and then loss of some of the erupted permanent teeth, aggressive periodontitis and excessive bone loss which was seen by radiographic examination. Treatment required a multi-disciplinary approach. Extraction of the teeth with grade 3 mobility was advised in all these cases and they were recalled after 3 weeks of healing for impression taking to form removable partial denture and complete denture. Maintenance of oral hygiene by brushing twice daily and use of mouth wash was recommended and for hyperkeratosis of hands and feet, the patients were referred to a competent dermatologist. The patients agreed to the treatment plan except our 3rd case who was 20 years old female. Conclusion: Early diagnosis and management with the early extraction of teeth having grade 3 mobility with prophylactically use of antibiotics can prevent the excessive bone loss and for periodontitis oral retinoid can be given. Key words: Papillon lefevre syndrom