Incontinentia pigmenti

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected, such as the central nervous system, eyes, hair, nails and teeth. The disease has a X-linked dominant inheritance pattern and is usually lethal to male fetuses. The dermatological findings occur in four successive phases, following the lines of Blaschko: First phase - vesicles on an erythematous base; second phase - verrucous hyperkeratotic lesions; third phase - hyperchromic spots and fourth phase - hypochromic atrophic lesions.

Saved in:
Bibliographic Details
Main Authors: Poziomczyk,Claudia Schermann, Recuero,Julia Kanaan, Bringhenti,Luana, Maria,Fernanda Diffini Santa, Campos,Carolina Wiltgen, Travi,Giovanni Marcos, Freitas,Andre Moraes, Maahs,Marcia Angelica Peter, Zen,Paulo Ricardo Gazzola, Fiegenbaum,Marilu, Almeida,Sheila Tamanini de, Bonamigo,Renan Rangel, Bau,Ana Elisa Kiszewski
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Dermatologia 2014
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962014000100026
Tags: Add Tag
No Tags, Be the first to tag this record!