2011
DOI: 10.1590/s0365-05962011000300037
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Você conhece esta síndrome?

Abstract: A Síndrome de Bloch-Sulzberger (Incontinência Pigmentar) é uma genodermatose rara, que afeta, principalmente, o sexo feminino, pois costuma ser letal em pacientes do sexo masculino intraútero. Caracteriza-se, principalmente, pelas manifestações dermatológicas, podendo também apresentar anomalias dentárias, oftalmológicas e neurológicas. As lesões cutâneas apresentam 4 fases distintas: vesiculosa, verrucosa, pigmentar e atrófica; que podem seguir uma sequência irregular, havendo até sobreposição das mesmas

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Cited by 5 publications
(16 citation statements)
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(21 reference statements)
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“…The cause of IP was attributed to a mutation that inactivates the IKK-gamma gene (IKBKG; OMIM 300248), also known as NEMO, localized in the region 28 of the X chromosome long arm. A positive family history for the disease is found in about 50% of cases (1,6) . The skin manifestations are present in almost all patients with IP and appear in 90% of cases during the second week of life.…”
Section: Introductionmentioning
confidence: 99%
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“…The cause of IP was attributed to a mutation that inactivates the IKK-gamma gene (IKBKG; OMIM 300248), also known as NEMO, localized in the region 28 of the X chromosome long arm. A positive family history for the disease is found in about 50% of cases (1,6) . The skin manifestations are present in almost all patients with IP and appear in 90% of cases during the second week of life.…”
Section: Introductionmentioning
confidence: 99%
“…The skin manifestations are present in almost all patients with IP and appear in 90% of cases during the second week of life. They are characterized by having four phases: vesicular, warty, pigmentary and atrophic phases, which may follow an irregular sequence and have variable duration (6) . These are usually the first signs observed in IP.…”
Section: Introductionmentioning
confidence: 99%
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