Showing 3 results for Lefevre Syndrome
G. rad Afshar ,
Volume 15, Issue 3 (6-2002)
Abstract
Diagnosis and treatment of patients with periodontitis as a manifestation of systemic diseases is of especial concern to the periodontist, especially those associated with genetic disorders, which have poor prognosis. With aggressive progression of periodontal bone and attachment loss, a patient could be a partial or total edentulous early in life.The aim of this article was to report a case of Papillon-Lefevre syndrome (PLS) with generalizedprepubertal periodontitis (GPPP). A ten-year old boy for whom active periodontal treatment and subsequent maintenance recalls was performed for five years since the diagnosis of PLS. Treatment procedures included: precise mechanical instrumentation at several visits, periodontal surgery, adjunctive serial systemic antibiotic therapy, professional irrigation of pockets with 0/2% chlorhexidine solution and periodic maintenance recall visits. In spite of all of these, progressive course of the disease continued until the patient was fifteen and edentulous. Unfortunately association of GPPP with systemic unmanageable condition or diseases has caused refractory periodontitis, which yet has no proven and reliable treatment protocol. Besides, this article has discussed more successful treatment modalities for PLS with GPPP and the genetic aspects of host susceptibility, which is a complicated and challenging field.
Mehrdad Lotf Azar, B. Gharesi Fard , Sh . Fargadian ,
Volume 15, Issue 4 (1-2003)
Abstract
Papillon Lefevre Syndrome (PLS) is a rare disease, associated with the early onset periodontal break down in deciduous and permanent dentition.The etiology of PLS is not exactly determined but recently, mutation in catespin C gene, as a genetic basis for the disease, has been established. The aim of this study was to investigate the HLA status in patients affected with PLS. In this research, the frequency of HLA-I and HLA-DR in 9 PLS patients (Belonged to seven unrelated families) were compared with 37 healthy people (controls). Fisher exact test was used for data analysis. The results indicated that the frequency of HLA-B18 was significantly higher in the patients’ group, compared to the control group(P<0.05). The present study, based on the serological methods, is the only existing controlled study regarding PLS. However, in order to get accurate results, a molecular technology with more samples is required.
M. Lotfazar ,
Volume 15, Issue 4 (1-2003)
Abstract
Papillon Lefevre Syndrome (PLS) is a rare recessive autosomal disease, which is associated with palm and sole hyperkeratosis and early periodontium break down of deciduouse and permanent dentition. In the differential diagnosis of PLS, congenital form of palm and sole hyperkeratosis should be considered.Recently, mutation in catepesin C gene has been recognized as a genetic basis for PLS disease. In this disease, complete lack of catepesin C enzyme activity, due to the mutations on both allels, are observed. Actinobacillous actinomycetemcomitans (A.a) bacteria are presented as the main periodontal pathogene in PLS disease. The treatment results have been contradictory and no agreement has been obtained in this regard. Elimination of A.a bacteria and administration of synthetic retinoids are reported as a suitable treatment.
