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奥姆斯特德综合征

Olmsted syndrome.

作者信息

Elise Tonoli Renata, De Villa Damiê, Hübner Frainer Renata, Pizzarro Meneghello Luana, Ricachnevsky Nelson, de Quadros Maurício

机构信息

Department of Dermatology, Brazilian Society of Dermatology, Santo Antônio Hospital, Santa Casa de Midericórdia de Porto Alegre, Porto Alegre, RS, Brazil.

出版信息

Case Rep Dermatol Med. 2012;2012:927305. doi: 10.1155/2012/927305. Epub 2012 Dec 23.

Abstract

Olmsted syndrome is a rare congenital, sharply circumscribed transgredient palmoplantar keratoderma. It was first described by Olmsted in 1927. The diagnosis of this rare disease depends on clinical features like symmetrical involvement of keratoderma of the palms and soles and the symmetrical hyperkeratotic plaques around the body orifices. It starts in the neonatal period or in childhood. The disease has a slow but progressive and extremely disabling course. Treatment of Olmsted syndrome is often based on topical therapy with retinoic acid, corticosteroid, emollients, and keratolytics. The present paper describes a case of Olmsted syndrome and its treatment.

摘要

奥姆斯特德综合征是一种罕见的先天性、边界清晰的进行性掌跖角化病。它于1927年由奥姆斯特德首次描述。这种罕见疾病的诊断取决于临床特征,如手掌和足底角化病的对称性受累以及身体孔口周围的对称性角化过度斑块。它始于新生儿期或儿童期。该疾病病程缓慢但呈进行性发展,且致残性极强。奥姆斯特德综合征的治疗通常基于外用维甲酸、皮质类固醇、润肤剂和角质剥脱剂。本文描述了一例奥姆斯特德综合征病例及其治疗情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a491/3540698/5c73888466b2/CRIM.DM2012-927305.001.jpg

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