Incontinentia pigmenti

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...

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Bibliographic Details
Main Authors: Claudia Schermann Poziomczyk, Julia Kanaan Recuero, Luana Bringhenti, Fernanda Diffini Santa Maria, Carolina Wiltgen Campos, Giovanni Marcos Travi, Andre Moraes Freitas, Marcia Angelica Peter Maahs, Paulo Ricardo Gazzola Zen, Marilu Fiegenbaum, Sheila Tamanini de Almeida, Renan Rangel Bonamigo, Ana Elisa Kiszewski Bau
Format: Article
Language:English
Published: Sociedade Brasileira de Dermatologia 2014-01-01
Series:Anais Brasileiros de Dermatologia
Subjects:
Genetic diseases, X-Linked
Incontinentia pigmenti
Pigmentation disorders
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962014000100026&tlng=en
Description
Summary: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.
ISSN:0365-0596

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