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[局限性硬皮病:92例回顾性研究]

[Localized scleroderma: a retrospective study about 92 cases].

作者信息

El Fékih Nadia, Réjaibi Iménc, Kamoun Hajer, Zéglaoui Faten, Fazaa Bécima, Kharfi Monia, Kamoun Mohamed Ridha

机构信息

Service de dermatologie Hôpital Charles Nicolle Tunis Tunisie.

出版信息

Tunis Med. 2009 Sep;87(9):573-8.

Abstract

BACKGROUND

Sclerodermas are rare affections which can be located or generalized. Localized form is the most frequent.

AIM

The purpose of this study was to describe epidemiologic, clinics, biological, immunological, therapeutic, evolutionary characteristics of the localized scleroderma through a personal series and the data of the literature.

METHOD

We have performed a retrospective study on all patients followed in the department of dermatology of the Hospital Charles Nicole during 14 years period.

RESULTS

Our study was about 92 cases of localized scleroderma (73 were females and 19 males). The mean age was 35 years (between 2 and 72 years). The majority of localised sclerodermas (66.2% of the cases) appeared before 40 years with a maximum of frequency between 10 and 30 years (41.6%). Only 11.9% of the cases were observed before 10 years. They were 51 cases (55%) of morphea, 35 cases (38%) of scleroderma in bands including 32 linear scleroderma and 3 scleroderma en coup de sabre, 5 cases (5.5%) of generalized morphea and 1 case (0.15%) of deep morphea. Average therapeutic was specified among 63 patients (87%), and the evolution could be appreciated among 45 patients.

CONCLUSION

The epidemiologic data observed in our series are comparable with those reported in the literature. Therapeutic difficulties and risks of functional after-effects, particular in scleroderma in bands, remain the principal concern for all the authors.

摘要

背景

硬皮病是一种罕见疾病,可局限或泛发。局限性硬皮病最为常见。

目的

本研究旨在通过个人病例系列及文献数据,描述局限性硬皮病的流行病学、临床、生物学、免疫学、治疗及演变特征。

方法

我们对在查尔斯·尼科尔医院皮肤科随访14年的所有患者进行了回顾性研究。

结果

我们的研究涉及92例局限性硬皮病患者(女性73例,男性19例)。平均年龄为35岁(2至72岁)。大多数局限性硬皮病(66.2%的病例)出现在40岁之前,发病频率最高在10至30岁之间(41.6%)。10岁之前仅观察到11.9%的病例。其中硬斑病51例(55%),带状硬皮病35例(38%),包括32例线状硬皮病和3例剑伤样硬皮病,泛发性硬斑病5例(5.5%),深部硬斑病1例(0.15%)。63例患者(87%)明确了平均治疗情况,45例患者可评估病情演变。

结论

我们系列研究中观察到的流行病学数据与文献报道的数据具有可比性。治疗困难以及功能后遗症的风险,尤其是带状硬皮病,仍然是所有作者主要关注的问题。

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