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Do you know this syndrome?

作者信息

Succi Isabella Brasil, Rosman Fernando Colonna, Oliveira Elisa Fontenelle de

机构信息

Hospital Municipal Jesus (HMJ), Rio de Janeiro, Brazil.

出版信息

An Bras Dermatol. 2011 May-Jun;86(3):608-10. doi: 10.1590/s0365-05962011000300037.

Abstract

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.

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