Papillon-Lefevre Synydrome Periodontitis: 1 Year Follow-up A Clinical Report

Abstract

Papillon-Lefevre Syndrome PLS is a rare autosomal recessive disease with a very low prevalence. Characteristic symptoms are diffuse palmar and plantar hyperkeratosis combined with a rapidly progressive periodontal breakdown affecting both dentitions, resulting in premature loss of deciduous and permanent teeth. This paper reports the treatment planning of the severely affected periodontal component of a patient with Papillon-Lefevre Syndrome. Clinical examination which included conventional periodontal measurements and radiographic analysis showed severe inflamation and bone loss. Total blood count, neutrophil function tests and alkaline phosphatase, CD 2, 3, 4, 5, 8, 11b, 16, HLA-DR and immunoglobulin analysis were made. Occurence of major suspected periodontopathic bacteria which included Porphyromonas gingivalis, Bacteriodes forsythus and Trepenoma denticola was determined by BANA N-benzoyl-DL-argininenaphthylamide test. As the result of clinical, immunological, microbiological and hematological findings, the patient was diagnosed as having PLS. The treatment consisted of extraction of all primary teeth and systemic antibiotic therapy. After an edentulous period complete dentures were inserted 3 months after the extractions. The patient is still under control after one year.

Keywords

• Papillon-LeFevre Syndrome, periodontitis, periodontal treatment.

PAPILLON-LEFEVRE SENDROMU İLE İLİŞKİLİ PERİODONTİTİS OLGUSU: 1 YILLIK TAKİP

Abstract

Papillon-Lefevre sendromu, PLS çok nadirgözlenen otozomal resesif bir hastalıktır. Süt vedaimi dişlerde erken kayıplar, hızlı ilerleyen periodontal doku kayıpları ile birlikte gözlenen, avuç içive ayak tabanında yaygın hiperkeratotik alanlarınvarlığı karakteristik bulgularındandır. Bu vaka raporu, PLS’ye bağlı olarak ileriderecede etkilenmiş periodontal dokularındeğerlendirilmesini amaçlamıştır. Rutin periodontal ölçümleri ve radyografik değerlendirmeleri kapsayan klinik incelemelerde, şiddetli inflamasyon vekemik kaybı tespit edilmiştir. Hastaya tam kansayımı, nötrofil fonksiyon testi, alkalen fosfataz,CD2,3,4,5,8,11b,16, HLA-DR ve immünglobulinanalizleri yapılmıştır. Ayrıca Porphyromonas gingivalis, Bacteriodes forsythus ve Trepenoma denticola’yı kapsayan periodonto-patojenler BANA Nbenzoyl-DL-arginine-naphthylamide testi ile tespitedilmiştir. Klinik, immünoljik, mikrobiyolojik vehematolojik bulguların sonucunda, hastaya PLSteşhisi konmuştur. Tedavi olarak, sistemik antibiyotik uygulaması altında bütün dişlerin çekimigerçekleştirilmiştir. Dişsiz periodu takiben 3 aysonra hastaya tam protez uygulaması yapılmıştır vehasta halen kontrol altındadır

Keywords

• Papillon-LefevreSendromu, periodontitis, periodontal tedavi

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