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嗜酸性环状红斑:多中心队列的临床病理分析及治疗结果

Eosinophilic Annular Erythema: Clinicopathologic Analysis and Therapeutic Outcomes from a Multicenter Cohort.

作者信息

Bettolini Luca, Maione Vincenzo, Bighetti Stefano, Venturini Marina, Incardona Paolo, Calzavara-Pinton Piergiacomo, Brunetti Antonio Podo, Stabile Giorgio, Rongioletti Franco

机构信息

Dermatology Department, University of Brescia, ASST Spedali Civili di Brescia, Brescia, Italy.

Faculty of Medicine and Surgery, University of Brescia, Brescia, Italy.

出版信息

Dermatol Pract Concept. 2025 Apr 1;15(2):5143. doi: 10.5826/dpc.1502a5143.

DOI:10.5826/dpc.1502a5143
PMID:40401866
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12090946/
Abstract

INTRODUCTION

Eosinophilic annular erythema (EAE) is a rare dermatosis characterized by persistent pruritic erythematous annular plaques with dermal eosinophilic infiltrates. It typically has a chronic, relapsing course with variable treatment responses and frequent refractory cases.

OBJECTIVES

This retrospective multicenter study reports the clinical and histopathological features of EAE and the treatment outcomes in a case series of 10 patients.

METHODS

Ten patients with a confirmed clinical and histopathological diagnosis of EAE were referred to the Dermatology Departments of the University of Brescia and the University San Raffaele of Milan for evaluation and treatment.

RESULTS

The cohort included six females and four males, all Caucasian, with a median age of 56 years. Time from lesion onset to diagnosis ranged from two days to seven years. Patients exhibited annular, figurate, or polycyclic plaques with erythematous borders-dashed in 80% and centrally pigmented in 70% of cases. Intense itching was reported by 90%. Histopathology displayed dermal infiltrate primarily composed of lymphocytes with various numbers of eosinophils, ranging from scattered (10%) to abundant (40%) and numerous (50%). Treatment responses were variable, oral corticosteroids, either alone or in combination with hydroxychloroquine, being the most used therapies. However, flares frequently occurred following discontinuation of treatment. Dupilumab has shown promise in achieving long-term remission.

CONCLUSION

Most patients exhibited pruritic lesions with dashed borders and central pigmentation, strongly suggesting a diagnosis of EAE. The positive response to dupilumab in refractory cases, along with long-term follow-up, reinforces the growing body of scientific evidence from case reports documented in the literature.

摘要

引言

嗜酸性粒细胞性环状红斑(EAE)是一种罕见的皮肤病,其特征为持续性瘙痒性红斑环状斑块伴真皮嗜酸性粒细胞浸润。它通常病程慢性、易复发,治疗反应不一,难治性病例常见。

目的

这项回顾性多中心研究报告了10例EAE患者的临床和组织病理学特征及治疗结果。

方法

10例经临床和组织病理学确诊为EAE的患者被转诊至布雷西亚大学和米兰圣拉斐尔大学皮肤科进行评估和治疗。

结果

该队列包括6名女性和4名男性,均为白种人,中位年龄56岁。从皮损出现到诊断的时间为2天至7年。患者表现为环状、地图状或多环状斑块,80%的病例边界呈虚线状,70%的病例中央色素沉着。90%的患者报告有剧烈瘙痒。组织病理学显示真皮浸润主要由淋巴细胞组成,伴有数量不等的嗜酸性粒细胞,从散在(10%)到丰富(40%)再到大量(50%)。治疗反应不一,最常用的治疗方法是单独使用口服糖皮质激素或与羟氯喹联合使用。然而,停药后常出现病情复发。度普利尤单抗在实现长期缓解方面显示出前景。

结论

大多数患者表现为边界呈虚线状和中央色素沉着的瘙痒性皮损,强烈提示EAE的诊断。难治性病例对度普利尤单抗的阳性反应以及长期随访,加强了文献中病例报告的科学证据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/6428c7b5fa78/dp1502a5143g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/2a8475394b75/dp1502a5143g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/47ec605f9758/dp1502a5143g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/6428c7b5fa78/dp1502a5143g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/2a8475394b75/dp1502a5143g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/47ec605f9758/dp1502a5143g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/64de/12090946/6428c7b5fa78/dp1502a5143g003.jpg

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本文引用的文献

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Dupilumab Treatment Efficacy and Impact on Clinical Scores, Serum Biomarkers, and Itch in Adult Patients with Atopic Dermatitis: A Retrospective Analysis.度普利尤单抗治疗成人特应性皮炎的疗效及其对临床评分、血清生物标志物和瘙痒的影响:一项回顾性分析
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Treatment of Eosinophilic Annular Erythema With Benralizumab.使用贝那利珠单抗治疗嗜酸性环状红斑。
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Dupilumab in Adults and Adolescents with Eosinophilic Esophagitis.
度普利尤单抗在成人和青少年嗜酸性食管炎中的应用。
N Engl J Med. 2022 Dec 22;387(25):2317-2330. doi: 10.1056/NEJMoa2205982.
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Effect of Dupilumab on Blood Eosinophil Counts in Patients With Asthma, Chronic Rhinosinusitis With Nasal Polyps, Atopic Dermatitis, or Eosinophilic Esophagitis.度普利尤单抗对哮喘、伴鼻息肉的慢性鼻-鼻窦炎、特应性皮炎或嗜酸性食管炎患者血液嗜酸性粒细胞计数的影响。
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Eosinophilic Annular Erythema: A New Entity of Eosinophilic Dermatosis.嗜酸性环状红斑:一种嗜酸性皮肤病的新类型。
Cureus. 2022 Feb 27;14(2):e22657. doi: 10.7759/cureus.22657. eCollection 2022 Feb.
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Treatment of Eosinophilic Annular Erythema: Retrospective multicenter study and literature review.嗜酸性环状红斑的治疗:回顾性多中心研究及文献复习。
Ann Dermatol Venereol. 2022 Jun;149(2):123-127. doi: 10.1016/j.annder.2021.07.007. Epub 2021 Oct 27.
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Eosinophilic annular erythema.嗜酸性环状红斑
Proc (Bayl Univ Med Cent). 2021 May 17;34(5):606-607. doi: 10.1080/08998280.2021.1922253. eCollection 2021 Sep.
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Refractory eosinophilic annular erythema treated successfully with dupilumab.使用度普利尤单抗成功治疗难治性嗜酸性粒细胞性环状红斑。
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Mepolizumab Therapy for Recalcitrant Eosinophilic Annular Erythema in an Adult: A Case Report and Review of Treatment Options.美泊利珠单抗治疗成人顽固性嗜酸性环状红斑:一例报告及治疗选择综述
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