Please use this identifier to cite or link to this item:
Title: Papillon-Lefèvre syndrome
Other Titles: ABD - Anais Brasileiros de Dermatologia
Authors: Silva, Thadeu Santos
Lacerda, Priscila Neri
Rêgo, Fernanda Pedreira de Almeida
Rêgo, Vitória Regina Pedreira de Almeida
Issue Date: 1-Feb-2018
Abstract: Papillon-Lefèvre syndrome is a rare, autosomal recessive genodermatosis with an estimated prevalence of one to four cases per million people, without sexual or racial predominance.1 It is clinically characterized by diffuse palmaoplantar keratoderma, associated with a severe periodontopathology, with premature loss of deciduous and permanent teeth. The cutaneous changes commonly begin during the first four years of life, and the severe destruction of periodontal regions results in loss of the primary teeth within these first four years, as well as premature loss of the permanent teeth.1 The syndrome is caused by a mutation in the gene that encodes the protein cathepsin C, a lysosomal protease known as dipeptidyl-peptidase 1, present in the epithelial regions commonly affected by the syndrome, as well as in cells of the immune system, including polymorphonuclear leukocytes, macrophages and their precursors
ISSN: 1806-4841
Appears in Collections:Artigos Completos Publicados em Periódicos

Files in This Item:
File Description SizeFormat 
ARTIGO - THADEU SILVA - 2018.pdf559,67 kBAdobe PDFView/Open

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.