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丘疹性弹性纤维离解:临床视角

Papular elastorrhexis: clinical perspectives.

作者信息

Sezer Engin, Öztürk Emel, Şahin Durmaz Sedef

机构信息

Department of Dermatology, Acibadem University School of Medicine, Istanbul, Turkey,

出版信息

Clin Cosmet Investig Dermatol. 2018 Oct 26;11:541-544. doi: 10.2147/CCID.S151020. eCollection 2018.

Abstract

First described by Bordas in 1987, papular elastorrhexis (PE) is a rare elastic fiber disorder of the skin characterized by multiple, discrete, asymptomatic, firm, nonfollicular, monomorphous, 1-5 mm, circumscribed, hypopigmented, oval to round papules, symmetrically distributed on the chest, abdomen, back, shoulders, arms, and thighs. The onset of the condition is usually in the first or second decade of life. PE appears to be an exceedingly rare entity, with 33 cases reported in the literature until now. However, the disorder might be underestimated probably because of its subtlety, asymptomatic course, and benign nature of clinical alterations, which can easily be confused with other dermatoses such as acne scars. Clinical and histopathological differential diagnosis of PE is broad and includes papular acne scars, eruptive collagenoma, disseminated lenticular dermatofibrosis (as a component of Buschke-Ollendorff syndrome), white fibrous papulosis of the neck, pseudoxanthoma elasticum, pseudoxanthoma elasticum-like papillary dermal elastolysis, middermal elastolysis, and perifollicular elastolysis. Treatment of PE is a matter of debate and no reliable curative option exists.

摘要

丘疹性弹力纤维松解症(PE)于1987年由博尔达斯首次描述,是一种罕见的皮肤弹性纤维疾病,其特征为胸部、腹部、背部、肩部、手臂和大腿上出现多个、离散、无症状、坚实、非毛囊性、形态单一、1 - 5毫米、边界清晰、色素减退、椭圆形至圆形的丘疹,呈对称分布。该病通常在生命的第一个或第二个十年发病。PE似乎是一种极其罕见的病症,迄今为止文献中报道了33例。然而,这种疾病可能被低估了,可能是因为其症状不明显、病程无症状以及临床改变的良性性质,这很容易与其他皮肤病如痤疮瘢痕相混淆。PE的临床和组织病理学鉴别诊断范围广泛,包括丘疹性痤疮瘢痕、发疹性胶原瘤、播散性豆状皮肤纤维瘤(作为布希克 - 奥伦多夫综合征的一个组成部分)、颈部白色纤维丘疹病、弹性假黄瘤、弹性假黄瘤样乳头真皮弹力纤维松解症、真皮中层弹力纤维松解症和毛囊周围弹力纤维松解症。PE的治疗存在争议,目前尚无可靠的治愈方法。

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