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Darier 病:利用皮肤镜监测阿维 A 治疗的效果。

Darier disease: the use of dermoscopy in monitoring acitretin treatment.

机构信息

Department of Dermatology, Facultad de Medicina, Clínica Alemana, Universidad del Desarrollo, Santiago, Chile.

Department of Dermatology, Facultad de Medicina, Clínica Alemana, Universidad del Desarrollo, Santiago, Chile.

出版信息

An Bras Dermatol. 2022 Sep-Oct;97(5):644-647. doi: 10.1016/j.abd.2021.05.021. Epub 2022 Jul 16.

Abstract

Darier disease is an uncommon autosomal dominant inherited disease, caused by a mutation in the ATP2A2 gene. The clinical findings are hyperkeratotic papules on the trunk, scalp, face, and neck, maceration of intertriginous areas, palmar pits, whitish papules on the oral mucosa and nail abnormalities. The main histopathologic findings are acantholysis and dyskeratotic keratinocytes. Dermatoscopic features are comedo-like openings with a central polygonal yellowish/brownish structure, surrounded by a whitish halo. First-line treatment includes acitretin. Five reports have been published describing Darier disease dermatoscopic findings. Herein, we report for the first time a patient under acitretin treatment and dermatoscopic follow-up.

摘要

Darier 病是一种罕见的常染色体显性遗传性疾病,由 ATP2A2 基因突变引起。临床表现为躯干、头皮、面部和颈部的角化过度性丘疹,皱褶部位糜烂,掌部凹窝,口腔黏膜的白色丘疹和指甲异常。主要组织病理学表现为棘层松解和角化不良的角质形成细胞。皮肤镜特征为类似粉刺的开口,中央有一个多边形的黄/棕色结构,周围有一个白色晕圈。一线治疗包括阿维 A。已有 5 篇关于 Darier 病皮肤镜表现的报道。本文首次报道了 1 例接受阿维 A 治疗和皮肤镜随访的患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fcda/9453516/b8f144a8d9f1/gr1.jpg

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