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Anais Brasileiros de Dermatologia

On-line version ISSN 1806-4841

Abstract

POZIOMCZYK, Claudia Schermann et al. Incontinentia pigmenti. An. Bras. Dermatol. [online]. 2014, vol.89, n.1, pp.26-36. ISSN 1806-4841.  https://doi.org/10.1590/abd1806-4841.20142584.

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.

Keywords : Genetic diseases, X-Linked; Incontinentia pigmenti; Pigmentation disorders.

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