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Id:
18148
Author:
Alvares, Valeria Brega
Title:
Paquioniquia congênita(Sindrome de Jadassohn Lewandowsky) Pachyonychia Congenita (Syndrome of Jadassohn Lewandowsky)-
Source:
s.l; s.n; 1997. 3 p. ilus, tab.
Abstract:
A sin drome de jadassohn lewandowsky ou paquioniquia conjenita (PC) hereditaria autossomica dominante de ocorrencia rara com expressividade fenotipica altamente variavel. Caracteriza-se pela formação de quantidades excessivas de queratina nas unhas pele e mucosas, manifestada por hiperceratose ungueal simetrica hiperceratose palmoplantar e leucoceratose folicular. Apresenta-se o caso de um jovem portador dessa anomaliacom onicodistrofa generalizada de mâos e pes, hiperceratose palmoplantar dentes natis maculas no dorso comedões e cistos multiplos pelo corpo paquitriquia e placas alopecias ocipitais. Neste artigo citam-se aspectos geneticos compilam-se manifestações clinicas revisam-se propostas de classificação descreve-se o quadro histologico e aventam-se as possibilidades terapeiticas da PC.(AU).
Descriptors:
CERATOSE FOLICULAR/diag
CERATOSE FOLICULAR/fisiopatol
CERATOSE FOLICULAR/reabil
CERATOSE FOLICULAR/terap
UNHAS/anorm
UNHAS/les
UNHAS/microbiol
UNHAS/fisiopatol
Limits:
RELATO DE CASO
HUMANO
Location:
BR191.1; 08974/s
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