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网状红斑黏蛋白病:25 例患者特征、相关疾病、治疗和预后的回顾性研究。

Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases.

机构信息

Section of Dermatology, Department of Health Sciences (DISSAL), University of Genoa, Viale Benedetto XV, 16132, Genoa, Italy.

出版信息

Br J Dermatol. 2013 Dec;169(6):1207-11. doi: 10.1111/bjd.12577.

Abstract

BACKGROUND

Reticular erythematous mucinosis (REM) is an uncommon disease, the nosology and specific characteristics of which are controversial because most reports deal with single cases or small series.

OBJECTIVES

To describe the characteristics of patients with REM regarding demographics, clinical and pathological features, comorbidities, treatment and course.

METHODS

A retrospective and prospective study was conducted on 25 patients diagnosed with REM in the setting of university-affiliated dermatology departments and dermatopathology centres.

RESULTS

Of the 25 patients with REM, 16 were women (sex ratio 2 : 1) and the mean age was 46 years. The roles of sun exposure and oral contraceptives were ambiguous. Associated diseases included hypertension (n = 4), malignancies (n = 3), autoimmune diseases (n = 3) and Borrelia infection (n = 1). Immunological studies (including serology and direct immunofluorescence) were noncontributory. The response to antimalarial treatment was good in > 80% of cases. Worsening or recurrence of the lesion after treatment discontinuation, or in the course of the disease, occurred in 31% of patients.

CONCLUSIONS

We present the largest REM case series to date. The reticular pattern with involvement of the midline of the chest and back, the predilection for middle-aged women, the controversial relationship with photosensitivity and the possible association with other conditions such as malignancies and thyroid dysfunctions are the main characteristics that makes REM a recognizable disease.

摘要

背景

网状红斑性黏蛋白病(REM)是一种罕见的疾病,其分类学和具体特征存在争议,因为大多数报道都是针对单个病例或小系列的。

目的

描述 REM 患者在人口统计学、临床和病理特征、合并症、治疗和病程方面的特点。

方法

对在大学附属皮肤科和皮肤病理中心诊断为 REM 的 25 例患者进行回顾性和前瞻性研究。

结果

在 25 例 REM 患者中,有 16 例为女性(男女比例为 2:1),平均年龄为 46 岁。阳光暴露和口服避孕药的作用不明确。相关疾病包括高血压(n=4)、恶性肿瘤(n=3)、自身免疫性疾病(n=3)和伯氏疏螺旋体感染(n=1)。免疫研究(包括血清学和直接免疫荧光)无意义。超过 80%的患者对抗疟治疗有良好的反应。在治疗停止或疾病过程中,31%的患者出现病变恶化或复发。

结论

我们目前提出了最大的 REM 病例系列。网状模式伴胸部和背部中线受累、中年女性的偏好、与光敏感性的争议关系以及与恶性肿瘤和甲状腺功能障碍等其他疾病的可能关联,是使 REM 成为一种可识别疾病的主要特征。

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