Eponym Papillon–Lefevre syndrome
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Buket Dalgc & Aysegul Bukulmez & Sinan Sar
- چاپ و سال / کشور: 2011
Description
Papillon–Lefevre Syndrome (PLS) is a very rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and severe early onset periodontitis, affecting the primary and permanent dentition. The syndrome was first described by Papillon and Lefevre in 1924. Genetic, immunologic, and microbiologic factors are suggested as responsible for the initiation and progression of the disease. A point mutation of cathepsin C gene has recently been detected in PLS. A multidisciplinary approach is important for management .The prognosis has improved with the early recognition of the syndrome, effective professional supervision, and home care.
Eur J Pediatr (2011) 170:689–691 Received: 6 October 2010 / Accepted: 24 November 2010 / Published online: 17 December 2010
