Papillon-Lefevre syndrome, Genetic aspects of host susceptibility & treatment modalities- A case report

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.