Räßler F, Lukács J, Elsner P
Klinik für Hautkrankheiten, Universitätsklinikum, Jena, Deutschland.
Department of Dermatology, University Hospital, Jena, Deutschland.
J Eur Acad Dermatol Venereol. 2016 Sep;30(9):1465-79. doi: 10.1111/jdv.13706. Epub 2016 Jun 29.
Eosinophilic cellulitis (Wells syndrome) is a rare inflammatory skin disease defined by erythematous, tender, sometimes urticarial plaques, possibly with vesicles and bullae, and granulomatous eosinophilic infiltrates in the dermis. Usually the disease has a benign course with spontaneous remission within a few weeks. Nevertheless, recurrences are quite frequent and may occur for several years. The objective of this study was to review the so far reported treatment options for Wells syndrome in a systematic manner. This systematic review is based on a search on Medline, Embase and Cochrane Central Register for English and German articles from 1970 to 2015. Advices on the treatment of Wells syndrome are limited predominately to case reports or to small case series. There are no randomized controlled trials, and control groups are missing. A variety of treatment options for Wells syndrome were reported including topical and systemic corticosteroids, antihistamines, cyclosporine, dapsone, azathioprine, griseofulvin, doxycycline, minocycline, antimalarial medications, oral tacrolimus/topical tacrolimus, sulfasalazine, interferon alpha and gamma, TNF alpha inhibitors, colchicine and PUVA therapy. As well-designed, randomized controlled trials are missing, no guidelines for the treatment of this disease can be given. Due to the small number of patients and the frequent misdiagnosis of this clinical entity, the aim of this systematic overview is to call attention to this rare condition and to help clinicians to diagnose and treat Wells syndrome effectively. Due to the good prognosis and tendency to resolve, systemic treatment should be limited to cases resistant to local therapy or with widespread lesions.
嗜酸性蜂窝织炎(韦尔斯综合征)是一种罕见的炎症性皮肤病,其特征为红斑、压痛,有时出现风团样斑块,可能伴有水疱和大疱,真皮内有肉芽肿性嗜酸性粒细胞浸润。通常该病病程呈良性,数周内可自发缓解。然而,复发相当常见,且可能持续数年。本研究的目的是以系统的方式回顾迄今为止报道的韦尔斯综合征的治疗选择。本系统评价基于对1970年至2015年期间Medline、Embase和Cochrane中央注册库中英语和德语文章的检索。关于韦尔斯综合征治疗的建议主要限于病例报告或小病例系列。没有随机对照试验,也没有对照组。报道了多种韦尔斯综合征的治疗选择,包括局部和全身用皮质类固醇、抗组胺药、环孢素、氨苯砜、硫唑嘌呤、灰黄霉素、多西环素、米诺环素、抗疟药物、口服他克莫司/外用他克莫司、柳氮磺胺吡啶、α和γ干扰素、肿瘤坏死因子α抑制剂、秋水仙碱和补骨脂素紫外线疗法。由于缺乏设计良好的随机对照试验,无法给出该病的治疗指南。由于患者数量少且该临床实体常被误诊,本系统综述的目的是引起对这种罕见疾病的关注,并帮助临床医生有效诊断和治疗韦尔斯综合征。鉴于预后良好且有自行消退的趋势,全身治疗应限于对局部治疗耐药或有广泛病变的病例。