Papillon–Lefevre Syndrome: A Case Report with Review of Literature
نویسنده
چکیده
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.
منابع مشابه
Papillon-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 کاملAnalysis of Human Leukocyte Antigen class II Gene Polymorphism in Iranian Patients with Papillon-Lefevre Syndrome: a Family Study
Background: Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and early development of aggressive pe-riodontitis. Although cathepsin C (CTSC) gene mutations have been established in about 70-80% of PLS patients, it is assumed that the patients may have dysfunctioning of immune defense mechanisms. Objective: To assess the associat...
متن کاملHLA Class I Gene Polymorphism in Iranian Patients with Papillon-Lefevre Syndrome
Background: Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive disorder characterized by diffused palmoplantar keratoderma and severe periodontitis. Increased susceptibility to infections due to impairment of the immune system is considered to be involved in pathoetiology of this disease. Objective: According to the crucial function of HLA molecules in immune responses and associati...
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-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 ...
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