Gastrointestinal manifestations in the Hallopeau-Siemens variant of recessive dystrophic epidermolysis bullosa*
作者:
期刊:
The British Journal of Radiology
(WILEY Available online 1993)
卷期:
Volume 66,
issue 789
页码: 788-793
年代: 1993
DOI:10.1259/0007-1285-66-789-788
出版商: The British Institute of Radiology
数据来源: WILEY
摘要:
AbstractEpidermolysis bullosa encompasses a group of rare disorders typified by blister formation following minor trauma to the skin. Gastrointestinal tract involvement may occur in the extremely rare recessive dystrophic variants. The gastrointestinal manifestations present in 25 patients with the Hallopeau-Siemens variant of recessive dystrophic epidermolysis bullosa are reviewed. In the oesophagus both anatomical and motility abnormalities were observed. Features seen included a generally shortened oesophagus, strictures including those resembling webs, hiatus herniae, decreased peristalsis, oesophageal atony and pseudodiverticulum formation. These patients also had faecal impaction.
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