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大疱性表皮松解症:克什米尔山谷的12例患者系列研究

Epidermolysis bullosa: a series of 12 patients in kashmir valley.

作者信息

Qayoom Seema, Masood Qazi, Sultan Javeed, Hassan Iffat, Jehangir Majid, Bhat Yasmeen J, Bhat Taseer, Chisti Muzamil

机构信息

Department of Dermatology, STD & Leprosy, SKIMS Medical College, Bemina, India.

出版信息

Indian J Dermatol. 2010 Jul-Sep;55(3):229-32. doi: 10.4103/0019-5154.70668.

Abstract

BACKGROUND

Epidermolysis Bullosa (EB) is a genetically determined mechano-bullous disorder of the skin encompassing a group of conditions that share skin fragility as a common feature.

MATERIALS AND METHODS

Twele patients with Epidermolysis Bullosa from Kashmir valley are reported.

RESULTS

Our series included 12 patients, 5 males and 7 females. Features were consistent with EB simplex in 8 patients, EB pruriginosa in 2 patients, generalized atrophic benign EB in one patient and EB acquista in one patient.

CONCLUSION

EB is a rare, genetically determined, blistering disorder affecting both males and females with predominant involvement of hands and feet. In the absence of specific therapy, treatment mainly involves avoidance of provoking factors, prevention and treatment of complications.

摘要

背景

大疱性表皮松解症(EB)是一种由基因决定的机械性大疱性皮肤病,包括一组以皮肤脆弱为共同特征的病症。

材料与方法

报告了来自克什米尔山谷的12例大疱性表皮松解症患者。

结果

我们的系列研究包括12例患者,5例男性和7例女性。8例患者的特征与单纯性EB一致,2例患者为痒疹性EB,1例患者为泛发性萎缩性良性EB,1例患者为获得性EB。

结论

EB是一种罕见的、由基因决定的水疱性疾病,影响男性和女性,主要累及手足。在缺乏特异性治疗的情况下,治疗主要包括避免诱发因素、预防和治疗并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/de0b/2965906/a89ebf409820/IJD-55-229-g001.jpg

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