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泛发性道林-德戈斯病:病例报告

Generalized dowling-degos disease: case reports.

作者信息

Wititsuwannakul Jade, Noppakun Nopadon

机构信息

Division of Dermatology, Department of Medicine, Faculty of Medicine, Chulalongkorn University and King Chulalongkorn Memorial Hosptial, Thai Red Cross Society, Bangkok, Thailand.

出版信息

Ann Dermatol. 2013 Aug;25(3):360-4. doi: 10.5021/ad.2013.25.3.360. Epub 2013 Aug 13.

Abstract

Dowling-Degos disease (DDD) is a rare autosomal dominant trait characterized by numerous, symmetrical, progressive and pigmented macules over the axillae, groins, face, neck, arms and trunk as well as scattered comedo-like lesions (dark dot, follicles) and pitted acneiform scars. Histopathology is diagnostic testing using a distinctive form of acanthosis, characterized by an irregular elongation of thin branching rete ridges, with a concentration of melanin at the tips. We report cases of generalized DDD in a single family with autosomal dominant penetrance. DDD can be presented in a generalized form with hypopigmented lesions instead of reticulate hyperpigmentation confined to the flexor areas. This form can be differentiated from DUH by histopathology.

摘要

道林-德戈斯病(DDD)是一种罕见的常染色体显性遗传病,其特征为腋窝、腹股沟、面部、颈部、手臂和躯干出现大量对称、进行性色素沉着斑,以及散在的粉刺样损害(黑点、毛囊)和凹陷性痤疮样瘢痕。组织病理学检查是诊断的关键,其特征为一种独特的棘皮症形式,表现为细分支状 rete 嵴不规则延长,尖端有黑色素聚集。我们报告了一个具有常染色体显性遗传外显率的单一家族中发生的全身性 DDD 病例。DDD 可表现为全身性形式,出现色素减退性损害,而非局限于屈侧部位的网状色素沉着。这种形式可通过组织病理学与弥漫性掌跖角化病相鉴别。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/542a/3756204/02b4afe01fbf/ad-25-360-g001.jpg

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