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诊断病例。肢端角化性弹性组织变性。

Case for diagnosis. Acrokeratoelastoidosis.

作者信息

Costa Mariana Carvalho, Bornhausen Demarch Eduardo, Hertz Amanda, Pereira Francisco Burnier Carlos, Azulay David Rubem

机构信息

Prof. Rubem David Azulay Institute of Dermatology, Santa Casa de Misericórdia do Rio de Janeiro, Brazil.

出版信息

An Bras Dermatol. 2011 Nov-Dec;86(6):1222-3. doi: 10.1590/s0365-05962011000600030.

Abstract

Acrokeratoelastoidosis is a type of palmoplantar keratoderma first described by Oswaldo Gonçalves Costa, a Brazilian Dermatologist from the state of Minas Gerais. It is a rare autosomal-dominant genodermatosis; however it may occur sporadically. The disease is not congenital; rather, its onset occurs in childhood or adolescence. Clinically, the condition is characterized by multiple yellowish papules, sometimes glossy and keratotic, measuring approximately 2-4 mm in diameter, sometimes umbilicated, and located symmetrically on the sides of the hands and feet, symmetry being the most typical sign. The most common histopathological findings are hyperkeratosis, mild acanthosis and elastorrhexis, as revealed by orcein staining.

摘要

肢端角化性弹性组织病是一种掌跖角化病,最初由来自米纳斯吉拉斯州的巴西皮肤科医生奥斯瓦尔多·贡萨尔维斯·科斯塔描述。它是一种罕见的常染色体显性遗传性皮肤病;然而,它也可能散发出现。这种疾病并非先天性的;相反,它在儿童期或青春期发病。临床上,该病的特征是多个淡黄色丘疹,有时光滑且角化,直径约2 - 4毫米,有时呈脐凹状,对称分布于手足两侧,对称性是最典型的体征。经地衣红染色显示,最常见的组织病理学表现为角化过度、轻度棘层肥厚和弹性纤维离解。

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