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一项回顾性多中心研究中100例类脂质渐进性坏死患者临床特征及治疗选择的更新结果

Updated results of 100 patients on clinical features and therapeutic options in necrobiosis lipoidica in a retrospective multicentre study.

作者信息

Erfurt-Berge Cornelia, Dissemond Joachim, Schwede Katharina, Seitz Anna-Theresa, Al Ghazal Philipp, Wollina Uwe, Renner Regina

机构信息

Department of Dermatology, University Hospital Erlangen, Ulmenweg 18, 91054 Erlangen, Germany.

Department of Dermatology, Venerology and Allergology, University Hospital Essen, Germany.

出版信息

Eur J Dermatol. 2015 Nov-Dec;25(6):595-601. doi: 10.1684/ejd.2015.2636.

Abstract

BACKGROUND

Necrobiosis lipoidica (NL) is a rare granulomatous disorder of unknown aetiology which can manifest in ulcerative skin lesions and is widely recognised in association with diabetes. We evaluated in total 100 patients to improve knowledge about the epidemiology and clinical course of NL. Additionally we focused on treatment options according to evidence level and expert opinion.

OBJECTIVE

To examine the clinical features of patients with necrobiosis lipoidica and to give recommendations for topical and systemic treatment.

MATERIALS & METHODS: This multicentre retrospective study covers data from 100 patients with the diagnosis of NL of the lower leg, treated during the period between 01/2006 and 06/2014. Data from 5 German dermatological centres with 23 men and 77 women with a median age of 52 years were evaluated. We also asked 15 dermatological experts for their opinions about ulceration, associated diseases and their preferences for treatment modalities of NL.

RESULTS

Patients' ages ranged from 15 to 95 years (median age 52 years, mean age 49.8 years). NL was significantly more common in women (77%). Ulceration of the NL was observed in 33% of cases, in particular in male patients and in patients with concomitant diabetes mellitus. Thyroidal disorders were found in 15% of all cases.

CONCLUSION

Our data confirm that female sex, middle age and endocrine disorders like diabetes mellitus or thyroid dysfunction are characteristics of patients suffering from NL. Existing therapeutic options were very variable and - according to the opinion of our experts - only a few highly effective.

摘要

背景

类脂质渐进性坏死(NL)是一种病因不明的罕见肉芽肿性疾病,可表现为溃疡性皮肤病变,并且与糖尿病相关已得到广泛认可。我们共评估了100例患者,以增进对NL流行病学和临床病程的了解。此外,我们根据证据水平和专家意见重点关注了治疗选择。

目的

研究类脂质渐进性坏死患者的临床特征,并给出局部和全身治疗的建议。

材料与方法

这项多中心回顾性研究涵盖了2006年1月至2014年6月期间接受治疗的100例诊断为小腿NL的患者的数据。对来自5个德国皮肤科中心的23名男性和77名女性患者的数据进行了评估,患者中位年龄为52岁。我们还询问了15位皮肤科专家对NL溃疡、相关疾病及其治疗方式偏好的意见。

结果

患者年龄在15至95岁之间(中位年龄52岁,平均年龄49.8岁)。NL在女性中更为常见(77%)。33%的病例观察到NL溃疡,尤其在男性患者和伴有糖尿病的患者中。15%的所有病例发现有甲状腺疾病。

结论

我们的数据证实,女性、中年以及糖尿病或甲状腺功能障碍等内分泌疾病是NL患者的特征。现有的治疗选择差异很大,并且——根据我们专家的意见——只有少数几种非常有效。

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