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获得性多发性毛细血管扩张症:中国人群中的一个新实体及相关因素分析。

Telangiectasia macularis multiplex acquisita: a new entity in Chinese populations and an analysis of associated factors.

机构信息

Departments of Dermatology and Gastroenterology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taipei, Taiwan.

出版信息

Int J Dermatol. 2013 Apr;52(4):426-31. doi: 10.1111/j.1365-4632.2011.05331.x. Epub 2012 Aug 22.

Abstract

BACKGROUND

Telangiectasia macularis multiplex acquisita (TMMA) is a rarely documented entity with distinctive clinical manifestation. Most patients are middle-aged adults. It may be associated with other diseases such as hepatitis, diabetes, or cardiovascular diseases.

OBJECTIVES

To assess the clinical presentation of TMMA and its association to other systemic diseases, especially the correlation to liver disease, in Taiwan.

METHODS

We identified and retrospectively analyzed clinical criteria-matched and skin biopsy-proven patients from 2002 to 2010 at a single medical center. The clinical criteria for diagnosis of TMMA included: (i) crops of telangiectasia superimposed on erythematous macules symmetrically on bilateral upper arms, possibly extending to the forearms, shoulders, V-shaped area of the anterior chest, back or thighs; (ii) no mucosal or systemic involvement; (iii) not associated with autoimmune diseases, such as lupus erythematosus, dermatomyositis or systemic scleroderma that may induce cutaneous telangiectasia; and (iv) no ataxia or unsteady gait.

RESULTS

Twenty-five patients were enrolled in this study. The male-to-female ratio was 19:6. The age of onset was variable, mostly between the third to sixth decades. The V-shaped area of the anterior chest was commonly involved (68%), in addition to the arms (100%). Viral hepatitis was diagnosed in 56.5% of the patients (13/23); 38.9% (7/18) and 50% (9/18) of the patients had diabetes and hypertension, respectively. In addition, 64.7% of the patients (11/17) had dyslipidemia. The pathological reports of most cases showed only mild perivascular lymphocytic infiltration with or without telangiectasia.

CONCLUSIONS

TMMA should be assigned to a distinct entity with unique clinical manifestation. It has not been well recognized and mostly misdiagnosed as other diseases with telangiectasia. It is important to evaluate the complete liver function tests when TMMA is suspected.

摘要

背景

多发性局灶性毛细血管扩张症(TMMA)是一种罕见的疾病,具有独特的临床表现。大多数患者为中年成年人。它可能与其他疾病如肝炎、糖尿病或心血管疾病有关。

目的

评估 TMMA 的临床表现及其与其他系统性疾病的关联,特别是与肝病的相关性,在台湾。

方法

我们从 2002 年至 2010 年在一家医疗中心确定并回顾性分析了临床标准匹配和皮肤活检证实的患者。TMMA 的临床诊断标准包括:(i)双侧上臂对称性红斑上叠加的毛细血管扩张症,可能延伸至前臂、肩部、前胸 V 形区、背部或大腿;(ii)无黏膜或系统性受累;(iii)与可能引起皮肤毛细血管扩张的自身免疫性疾病如红斑狼疮、皮肌炎或系统性硬皮病无关;(iv)无共济失调或步态不稳。

结果

本研究共纳入 25 例患者。男女比例为 19:6。发病年龄不定,多在第三至第六个十年。前胸 V 形区常见受累(68%),其次是手臂(100%)。病毒性肝炎诊断为 56.5%的患者(13/23);38.9%(7/18)和 50%(9/18)的患者分别患有糖尿病和高血压。此外,64.7%的患者(11/17)有血脂异常。大多数病例的病理报告仅显示轻度血管周围淋巴细胞浸润,伴有或不伴有毛细血管扩张。

结论

TMMA 应归为具有独特临床表现的独特实体。它尚未得到充分认识,并且大多被误诊为其他具有毛细血管扩张的疾病。当怀疑 TMMA 时,重要的是要评估完整的肝功能检查。

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