Incontinentia pigmenti* * Work performed at Porto Alegre Health Sciences Federal University (UFCSPA) - Porto Alegre (RS), Brazil

Cláudia Schermann Poziomczyk Júlia Kanaan Recuero Luana Bringhenti Fernanda Diffini Santa Maria Carolina Wiltgen Campos Giovanni Marcos Travi André Moraes Freitas Marcia Angelica Peter Maahs Paulo Ricardo Gazzola Zen Marilu Fiegenbaum Sheila Tamanini de Almeida Renan Rangel Bonamigo Ana Elisa Kiszewski Bau About the authors

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.

Genetic diseases, X-Linked; Incontinentia pigmenti; Pigmentation disorders


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