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Revista chilena de pediatría

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ENEI G, Mª LEONOR et al. Mother and daughter incontinentia pigmenti: Case report. Rev. chil. pediatr. [online]. 2011, vol.82, n.3, pp.225-230. ISSN 0370-4106.

Incontinentia pigmenti (IP) is a rare genodermatosis linked to the X chromosome. It affects variably all tissues derives from neuroecthoderm such as skin, hair, nails, eyes and central nervous system. Early diagnosis allows the study of eventual multisystem involvement. Clinical case: We describe a 6 m.o. girl, controlled from the first week of life for a dermatological feature characterized by linear lesions, which were vesicular, then verrucous, and finally hyperpigmented. IP diagnostic family, determined by maternal history of similar lesions.

Keywords : X-linked dominant; incontinentia pigmenti; genodermatosis; neurocutaneous syndromes.

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