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Anais Brasileiros de Dermatologia

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SUCCI, Isabella Brasil; ROSMAN, Fernando Colonna  and  OLIVEIRA, Elisa Fontenelle de. Do you know this syndrome?. An. Bras. Dermatol. [online]. 2011, vol.86, n.3, pp. 608-610. ISSN 0365-0596.

Bloch-Sulzberger syndrome (incontinentia pigmenti) is a rare genodermatosis that affects predominantly females, since it is generally lethal to male fetuses in utero. It is characterized principally by skin lesions, but may also involve dental, ophthalmological and neurological abnormalities. The skin lesions are present in four different phases: vesicular, verrucous, hyperpigmented and atrophic/hypopigmented. Their sequence is irregular and overlapping of stages is common

Keywords : Incontinentia pigmenti; Pigmentation disorders; Skin diseases, genetic.

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