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Acta Odontológica Venezolana

versión impresa ISSN 0001-6365

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FREIRE DE CASTILHO, Aline Rogeria; MARCHI DE CARVALHO, Izabel Maria  y  FERRAZ VANIN SORACE, Catherine. Incontinentia pigmenti  en una niña brasileña. Acta odontol. venez [online]. 2005, vol.43, n.2, pp.151-154. ISSN 0001-6365.

Incontinentia Pigmenti is one of the rare conditions that affect organs and tissues of ectodermal and mesodermal origin, and is inherited as an X-linked dominant trait. It may be noticed at birth or soon after, by means of skin erythematous lesions. It may also affect hair, nails, eyes, central nervous system and teeth. Dental manifestations are similar to those observed on ectodermal dysplasia and congenital syphilis: hypodontia, microdontia, delayed tooth eruption, malformations and impacted teeth. The dental alterations presented on this case report are typical and include tooth agenesis, peg-shaped teeth and eruption disturbances. A clinical case of a girl is presented in that observes the cutaneous hiperpigmentation of the superior members and the back; and the clinical absence of several temporary teeth.

Palabras clave : Incontinentia Pigmenti; skin lesions; dental manifestations; ectodermal dysplasia.

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