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营养不良性大疱性表皮松解症的上呼吸道和外生殖器受累情况。

Upper airway and external genital involvement in epidermolysis bullosa dystrophica.

作者信息

Shackelford G D, Bauer E A, Graviss E R, McAlister W H

出版信息

Radiology. 1982 May;143(2):429-32. doi: 10.1148/radiology.143.2.7071344.

Abstract

Radiographic findings in two patients with uncommon manifestations of epidermolysis bullosa dystrophica are described. A girl with recurrent urinary tract infections had scarring of the external genitalia producing chronic vaginal and uterine reflux and retention of urine. A second patient with long-standing stridor was found to have subglottic narrowing due to localized subglottic edema associated with an inflammatory membrane. The second case illustrates the occasional involvement of columnar epithelial surfaces which can occur in both epidermolysis bullosa dystrophica and epidermolysis bullosa hereditaria letalis.

摘要

描述了两名患有营养不良性大疱性表皮松解症罕见表现患者的影像学检查结果。一名反复发生尿路感染的女孩,其外生殖器有瘢痕形成,导致慢性阴道和子宫反流以及尿潴留。另一名长期喘鸣的患者被发现存在声门下狭窄,这是由于局部声门下水肿伴有炎性膜所致。第二个病例说明了柱状上皮表面偶尔会受累,这种情况在营养不良性大疱性表皮松解症和遗传性致死性大疱性表皮松解症中都可能发生。

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