جستجوی مقالات مرتبط با کلیدواژه « Lefevre syndrome » در نشریات گروه « پزشکی »

Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder. Clinical manifestations of this disease are hyperkeratosis of the palms and soles and rapidly progressive periodontitis, which result in premature loss of deciduous and permanent teeth. Early diagnosis and correct treatment of this discover could prevent the involvement of permanent teeth and promote periodontal condition, which leads to longer maintenance of deciduous teeth and prevention of alveolar ridge breakdown and prosthetic problems.
Case report: Our case was a 4-year-old boy presented with PLS, hyperkeratosis of the palms and soles and rapidly progressive periodontitis, which had led to the loosening of deciduous teeth. The case has been followed for four years. Evidence-based treatment of the patient resulted in stabilized periodontal condition and the possibility of partial removable prosthodontics for aesthetic and functional reasons. In addition, periodontal condition of permanent teeth of the patient was favorable after the eruption.
Due to periodontal disease, dentists are often the first to diagnose this syndrome. Early diagnosis and treatment of PLS is of paramount importance due to the possibility of preventing the involvement of permanent teeth.

Due to the lack of a clear and consistent protocol for periodontal therapy in patients with Papillon-Lefèvre syndrome, periodontal treatment is still a challenge for dentists in such patients. The aim of this study was to investigate the causes of success and failure in periodontal treatment of patients with Papillon-Lefèvre syndrome reported in the literature based on the treatment protocols offered. In addition, this article describes the periodontal treatment of a child with this syndrome during a 6-year follow-up.Case report: A 6-year-old boy, with clinical symptoms of hyperkeratosis of the palms, soles and knees and generalized aggressive periodontitis in primary dentition, was referred to Isfahan Faculty of Dentistry. The disease was confirmed by clinical and radiological evaluations. The patient was treated with a combination of non-surgical and anti-microbial therapy and placed on a monthly recall. Despite severe bone resorption and inflammatory symptoms on follow-up visits, during the last visit the patient exhibited radiographic signs of bone formation, which can enhance the idea that it might be possible to control the patient’s periodontal disease with continuous follow-ups.

Papillon-Lefèvre syndrome is a rare autosomal recessive disorder. This syndrome is characterized by palmoplantar hyperkeratosis، severe periodontal destruction and premature loss of primary and permanent teeth. The teeth erupt normally but due to the severe alveolar bone loss in both deciduous and permanent dentitions teeth are exfoliated within two or three years after eruption and by the age of 15 or 17 patients are usually edentulous. The identified genetic defect in this syndrome involves a mutation in the gene encoding cathepsin C. An increased susceptibility to infection has also been reported in patient with this syndrome. Case Report: A 15-year-old girl was referred to the dental clinic، complaining of the mobility of permanent teeth. The patient had lost all the permanent teeth except for teeth 13، 14، 17، 23، 27، 37، 43، 44 and 47. The third molars were impacted. The patient had advanced periodontal disease and all the teeth had severe mobility. There was hyperkeratosis at the palms and soles. She did not report a history of recurrent skin infections and liver abscesses. Complete blood count and liver tests were within the normal limits. Due to periodontal disease the dentists are often the first to diagnose this syndrome. Early diagnosis of Papillon-Lefèvre syndrome can help preserve the permanent teeth by early institution of treatment، using a multidisciplinary approach. Dental treatment includes extraction of all deciduous teeth، professional prophylaxis، conventional periodontal therapy، systemic antibiotics، oral retinoids، complete dentures and implants.

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.