Papillon-Lefèvre Syndrome Case Report
نویسندگان
چکیده
1 Assistant professor, Oro-maxillofacial developmental diseases research center, Department of oral medicine, Guilan University of Medical Sciences, Dental school, Rasht, Iran 2 Associate professor, Dental Research Center, Mashhad University of Medical Sciences, Dental School, Mashhad, Iran 3 Assistant professor, Department of Oral and Maxillofacial Radiology, Mashhad University of Medical Sciences, Dental School, Mashhad, Iran
منابع مشابه
Papillon-Lefèvre Syndrome: a case report
Introduction: Papillon-Lefèvre syndrome(PLS) characterized by palmoplantar hyperkeratosis is a rare autosomal recessive genetic disorder with rapidly progressive periodontitis and premature loss of both deciduous and permanent teeth. In this study, we report the clinical and radiographic features of Papillon-Lefèvre syndrome in an 11- year-old girl and we also discuss the history ...
متن کاملPapillon-Lefevre syndrome: A report of two cases in a family
Palmoplantar keratodermas are a heterogenous group of diseases, one of them is Papillon-Lefevre Syndrome (PLS). This rare disease is inherited as autosomal recessive and characterized by focal hyperkeratotic plaques on elbows and knees, severe periodontal disease resulting in premature loss of teeth. We report two brothers with PLS who did not have a history of this disease in their famil...
متن کاملOriginal articles Haim-Munk syndrome and Papillon-Lefèvre syndrome are allelic mutations in cathepsin C
Of the many palmoplantar keratoderma (PPK) conditions, only Papillon-Lefèvre syndrome (PLS) and Haim-Munk syndrome (HMS) are associated with premature periodontal destruction. Although both PLS and HMS share the cardinal features of PPK and severe periodontitis, a number of additional findings are reported in HMS including arachnodactyly, acroosteolysis, atrophic changes of the nails, and a rad...
متن کاملPAPILLON-LEFE\'VRE SYNDROME: NEUTROPHIL MOTILITY AND KILLING DEFECT IN A CHILD WITH RECURRENT SEVERE INFECTIONS
A case of palmoplantar hyperkeratosis with periodontosis and a history of recurrent severe pyoderma, pneumonia and multiple liver abscesses is described in a 12 year old girl. The patient demonstrated neutrophil dysfunction characterized by decreased random migration and chemotaxis and defective bactericidal activity. The exact immunopathological mechanism for susceptibility to infections i...
متن کاملPapillon–Lefevre Syndrome: A Case Report with Review of Literature
papillon-Lefèvre Syndrome (PLS) Is A Very Rare Autosomal Recessive Disorder Characterized By Palmoplantar Hyperkeratosis And Severe Early Onset Of Destructive Periodontitis Leading To Premature Loss Of Both Primary And Permanent Dentitions. Here We Are Presenting Case Report Of Siblings Who Presented With Palmoplantar Hyperkeratosis And Aggressive Periodontitis.
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