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嗜酸性环形红斑是 Wells 综合征谱中的一种特殊亚型:一项多中心长期随访研究。

Eosinophilic annular erythema is a peculiar subtype in the spectrum of Wells syndrome: a multicentre long-term follow-up study.

机构信息

Department of Dermatology, Faculty of Medicine, Al-Azhar University, Cairo, Egypt.

出版信息

J Eur Acad Dermatol Venereol. 2013 Aug;27(8):973-9. doi: 10.1111/j.1468-3083.2012.04616.x. Epub 2012 Jun 25.

DOI:10.1111/j.1468-3083.2012.04616.x
PMID:22731886
Abstract

BACKGROUND/OBJECTIVE: Eosinophilic annular erythema (EAE) was proposed to describe annular skin lesions associated with tissue eosinophilia, however, its relation to Well's syndrome (WS) remains a source of controversy. We studied a series of patients to increase awareness of this entity and to clarify its relation to WS.

METHODS

A multicentre study in which the clinical records, histological findings, laboratory results, therapeutic responses and follow-up of 10 patients were demonstrated.

RESULTS

The study included seven women and three men with age ranging from 31 to 54 years. The duration of the disease ranged from 3 to 28 months. All patients showed involvement of trunk and extremities. Early lesions were manifested as erythematous plaques, which progressed to well-developed figurate lesions and ended as large annular lesions with pigmented centre and elevated border. Flame figures were only observed in well-developed and long-standing lesions. Blood eosinophilia was found in all patients with different grades. Associated disorders included chronic gastritis, diabetes mellitus, chronic hepatitis-C virus infection and chronic kidney disease. The disease showed chronic course with high relapse rate and resistance to various therapeutic modalities including systemic steroid alone and in combination with hydroxychloroquine and cyclosporine.

CONCLUSION

We believe that EAE is a peculiar clinical variant in the spectrum of WS, which is characterized by a chronic course, resistance to treatment and high relapse rate. The diagnosis and evaluation of this condition need a close monitoring with repeated clinical, histological and laboratory assessment.

摘要

背景/目的:嗜酸性环形红斑(EAE)被提出用于描述与组织嗜酸性粒细胞增多相关的环形皮肤病变,但它与 Wells 综合征(WS)的关系仍存在争议。我们研究了一系列患者,以提高对该疾病的认识,并阐明其与 WS 的关系。

方法

这是一项多中心研究,其中展示了 10 例患者的临床记录、组织学发现、实验室结果、治疗反应和随访情况。

结果

研究纳入了 7 名女性和 3 名男性,年龄在 31 至 54 岁之间。疾病的病程从 3 至 28 个月不等。所有患者均表现为躯干和四肢受累。早期病变表现为红斑斑块,进展为形态良好的图形病变,最终形成色素沉着中心和隆起边缘的大环形病变。火焰图形仅见于形态良好和长期存在的病变中。所有患者均存在不同程度的血液嗜酸性粒细胞增多。相关疾病包括慢性胃炎、糖尿病、慢性丙型肝炎病毒感染和慢性肾脏病。疾病呈慢性病程,复发率高,对各种治疗方法(包括单独使用全身类固醇以及联合羟氯喹和环孢素)均有耐药性。

结论

我们认为 EAE 是 WS 谱中的一种特殊临床变异型,其特征为慢性病程、治疗耐药和高复发率。这种疾病的诊断和评估需要密切监测,反复进行临床、组织学和实验室评估。

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