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.
