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儿童获得性大疱性表皮松解症。三例报告及文献复习。

Childhood epidermolysis bullosa acquisita. Report of three cases and review of literature.

作者信息

Arpey C J, Elewski B E, Moritz D K, Gammon W R

机构信息

Department of Dermatology, University Hospitals of Cleveland, Case Western Reserve University School of Medicine, OH 44106.

出版信息

J Am Acad Dermatol. 1991 May;24(5 Pt 1):706-14.

PMID:1869641
Abstract

Epidermolysis bullosa acquisita is an acquired subepidermal blistering disease with variable clinical, pathologic, and immunologic features. The disease has been reported infrequently in adults and only rarely in children. We describe three new cases of childhood epidermolysis bullosa acquisita, review three previously reported cases, and contrast the features of the disease in children with those in adults. The results suggest that both children and adults with epidermolysis bullosa acquisita have variable clinical and pathologic features that may mimic other bullous diseases. Epidermolysis bullosa acquisita is characterized by a chronic course, poor response to therapy, and occasional clinical remissions.

摘要

获得性大疱性表皮松解症是一种获得性表皮下大疱性疾病,具有多样的临床、病理和免疫特征。该疾病在成人中报道较少,在儿童中更是罕见。我们描述了三例儿童获得性大疱性表皮松解症的新病例,回顾了之前报道的三例病例,并对比了儿童和成人患者的疾病特征。结果表明,儿童和成人获得性大疱性表皮松解症均具有多样的临床和病理特征,可能会与其他大疱性疾病相似。获得性大疱性表皮松解症的特点是病程慢性、治疗反应不佳且偶尔会出现临床缓解。

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