Incontinentia pigmenti
AUTOR(ES)
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
FONTE
An. Bras. Dermatol.
DATA DE PUBLICAÇÃO
2014-01
RESUMO
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.
