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大疱性表皮松解痣:隐性营养不良型1例的临床、皮肤镜及组织学特征

Epidermolysis bullosa nevi: clinical, dermatoscopical and histological features in a case of recessive dystrofic form.

作者信息

Melo Juliana Nakano de, Teruya Priscila Yoshie, Machado Maria Cecília Rivitti, Valente Neusa Sakai, Sotto Mirian Nacagami, Oliveira Zilda Najjar Prado de

机构信息

Departamento de Dermatologia, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, Brasil.

出版信息

An Bras Dermatol. 2011 Jul-Aug;86(4):743-6. doi: 10.1590/s0365-05962011000400017.

DOI:10.1590/s0365-05962011000400017
PMID:21987141
Abstract

Acquired melanocytic lesions may present unusual clinical features in all forms of hereditary epidermolysis bullosa. These lesions are known as "EB nevi", and often pose a diagnostic challenge for dermatologists given their resemblance - clinically, dermoscopically and histologically - to melanoma. The lesions have been reported in all types of hereditary EB, most of them in childhood. We report the case of a 6-month-old boy suffering from recessive dystrophic epidermolysis bullosa (RDEB) that presented as a large pigmented lesion on his left thigh. We decided to monitor the lesion closely since we considered that the clinical and pathological aspects of the lesion were compatible with the description of other previously reported cases of EB nevi.

摘要

在所有类型的遗传性大疱性表皮松解症中,获得性黑素细胞性损害可能呈现出不寻常的临床特征。这些损害被称为“大疱性表皮松解症痣”,鉴于其在临床、皮肤镜检查和组织学上与黑色素瘤相似,常常给皮肤科医生带来诊断挑战。所有类型的遗传性大疱性表皮松解症中均有关于这些损害的报道,其中大多数发生在儿童期。我们报告了一例6个月大患隐性营养不良型大疱性表皮松解症(RDEB)的男婴病例,该患儿左大腿出现一个大的色素沉着性损害。由于我们认为该损害的临床和病理特征与之前报道的其他大疱性表皮松解症痣病例描述相符,因此决定密切监测该损害。

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Epidermolysis bullosa nevi: clinical, dermatoscopical and histological features in a case of recessive dystrofic form.大疱性表皮松解痣:隐性营养不良型1例的临床、皮肤镜及组织学特征
An Bras Dermatol. 2011 Jul-Aug;86(4):743-6. doi: 10.1590/s0365-05962011000400017.
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Epidermolysis bullosa nevus in a patient with recessive dystrophic epidermolysis bullosa: a case report.一名隐性营养不良性大疱性表皮松解症患者的大疱性表皮松解痣:病例报告
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Hereditary epidermolysis bullosa: clinical-epidemiological profile of 278 patients at a tertiary hospital in São Paulo, Brazil.遗传性大疱性表皮松解症:巴西圣保罗一家三级医院 278 例患者的临床流行病学特征。
An Bras Dermatol. 2024 May-Jun;99(3):380-390. doi: 10.1016/j.abd.2023.06.009. Epub 2024 Feb 24.