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一例 1 型节段型马赛克型 Darier 病。

A case of segmental form of type 1 mosaic Darier's disease.

机构信息

Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.

Department of Dermatovenereology, Ljubljana University Medical Centre, Ljubljana, Slovenia.

出版信息

Acta Dermatovenerol Alp Pannonica Adriat. 2022 Mar;31(Suppl):S33-S35.

Abstract

Darier's disease is a rare genetic disorder with autosomal dominant inheritance. It is characterized by hyperkeratotic papules in seborrheic areas. Associated abnormalities include nail abnormalities and changes in the mucous membranes. Exacerbation of the disease occurs with exposure to high temperatures, sun, and sweating, resulting in a worsening clinical picture in summer months. The unilateral zosteriform pattern is a rare variant that is clinically manifested by a unilateral outbreak of erythematous keratotic papules without any other associated symptoms. Here we present a 52-year-old male with a zosteriform pattern of Darier's disease. We also discuss the most important clinical and pathohistological characteristics of the disease and various treatment options.

摘要

大疱性表皮松解症是一种罕见的遗传性疾病,具有常染色体显性遗传特征。其特征是脂溢部位出现角化过度性丘疹。相关的异常包括指甲异常和粘膜变化。疾病的恶化与暴露在高温、阳光和出汗有关,导致夏季临床症状恶化。单侧带状模式是一种罕见的变异,其临床表现为单侧红斑角化性丘疹发作,无任何其他相关症状。在此,我们介绍了一名 52 岁男性的大疱性表皮松解症单侧带状模式。我们还讨论了该病最重要的临床和病理组织学特征以及各种治疗选择。

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