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G55 GASTROINTESTINAL PROBLEMS IN EPIDERMOLYSIS BULLOSA
E. Freeman, A. Martinez, J. Mellerio, N. Sebire, V. Smith, K. Lindley, N. Shah.Great Ormond Street Hospital Department of Gastroenterology, London, UK
Aims: Epidermolysis bullosa (EB) is an inherited skin blistering condition. The aim of this study was to define the nature of gastrointestinal (GI) problems in children with EB.
Methods: All children in a supraregional paediatric EB centre were reviewed for GI problems.
Results: 224 cases were reviewed. 80 (31%) had epidermolysis bullosa simplex (EBS), 119 (53%) dystrophic type (RDEB), and 19 (8%) junctional type EB (JEB). GI problems were present in 139 (62%). Foregut problems of gastro-oesophageal reflux (GOR) affected 64/224 (28.6%), particularly common in RDEB (74%). Treatment was with high dose proton pump antagonists. Dysphagia from oesophageal strictures was only seen in the RDEB group 43/80 (53%) requiring treatment by repeated dilatations. Mid-gut problems were common in the JEB group with a quarter having a protein losing enteropathy that responded well to a low MCT based feed or prednisolone. Hind gut problems were most common with constipation affecting 77/224 (34.3%). Treatment was with high dose laxatives. Diarrhoea was a worrying problem as painful dressing changes were frequent. This affected 9/224 (4%) of all cases and 10% of those with RDEB. In 8/9 a non-specific colitis with mixed chronic inflammatory infiltrate and prominent apoptotic debris and pigment-laden macrophages was seen. They all responded to sulphasalazine and prednisolone.
Conclusion: GI problems in EB are very common. There is EB subtype specificity for GI problems. All symptoms require early recognition and aggressive intervention .The clinical subtype specificity and histopathological changes have not been described previously.
G56 ACUTE ERYTHRODERMAS PRESENTING TO A DISTRICT GENERAL HOSPITAL OVER A SIX MONTH PERIOD
F. Cader, H. Goodyear.Heart of England NHS Foundation Trust, Birmingham, UK
Erythroderma is a rare skin disorder caused by a variety of aetiologies depending on age. We report a case series presenting to …