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Published Online First: 17 October 2006. doi:10.1136/jcp.2005.035766
Journal of Clinical Pathology 2007;60:843-844
Copyright © 2007 by the BMJ Publishing Group Ltd & Association of Clinical Pathologists.

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SHORT REPORT

Histopathological features of gastrointestinal mucosal biopsy specimens in children with epidermolysis bullosa

N Shah1, E Freeman1, A Martinez2, J Mellerio2, V V Smith3, K J Lindley4, N J Sebire5

1 Department of Paediatric Gastroenterology, Great Ormond Street Hospital, London, UK
2 Department of Paediatric Dermatology, Great Ormond Street Hospital, London, UK
3 Department of Histopathology, Great Ormond Street Hospital, London, UK
4 Department of Paediatric Gastroenterology, Great Ormond Street Hospital, London, UK
5 Department of Histopathology, Great Ormond Street Hospital, London, UK

Correspondence to:
Dr N Shah
Consultant Paediatric Gastroenterologist, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH; ShahN@gosh.nhs.uk 5 January 2006

Keywords: epidermolysis bullosa; inflammation; apoptosis

The first 150 words of the full text of this article appear below.

Epidermolysis bullosa (EB) is a term used to represent a group of conditions characterised by cutaneous blistering due to abnormalities of ultrastructural components anchoring epithelial cells to either each other or to the basement membrane.1–3 There are a range of subtypes, which were traditionally classified on the basis of the level of the epidermal separation, more recently delineated on the basis of the underlying genetic defect.4 The vast majority of the clinical literature in these conditions is based around the cutaneous complications. There is a reported association between some subtypes of epidermolysis bullosa and pyloric stenosis,4 but there are no previous reports of mucosal histopathological features, despite these patients sometimes exhibiting significant gastrointestinal symptoms.

We report the gastrointestinal mucosal histopathological features in a series of patients with epidermolysis bullosa who underwent endoscopic evaluation for gastrointestinal symptoms at a single specialist centre.


METHODS
Since 2003 it has been our policy to investigate . . . [Full text of this article]







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Copyright © 2007 by the BMJ Publishing Group Ltd & Association of Clinical Pathologists.