title: Epidermolysis bullosa. Part 1: causes, presentation and complications.
abstract: This article is the first in a series of three focusing on the causes, clinical presentation, complications and care of adult patients affected by epidermolysis bullosa (EB), a group of rare genetic skin fragility disorders. Although the condition is rare, in some cases it presents extreme challenges both to those affected and those involved in the care of the EB patient; therefore, these articles may have relevance for other long-term disorders. While there is a wealth of information regarding the 'science' of EB there is dearth of information regarding the care of the adult EB patient, and this series of articles will endeavour to fill that gap. This article focuses mainly on those patients affected with the most severe form of EB found in the adult group, recessive dystrophic epidermolysis bullosa; with the part two looking at the care of the adult with EB from the nursing perspective, including wound management, and the experiences of a specialist EB psychotherapist being presented in the final article of the series. Readers will thus have an opportunity to gain an overall view of this difficult condition.
authors.affiliation: DebRA Adult Nursing Team, DebRA UK.
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Pillay
mesh_terms: D000740:Anemia / Q000209:etiology; D002294:Carcinoma, Squamous Cell / Q000209:etiology; D002311:Cardiomyopathy, Dilated / Q000209:etiology; D015984:Causality; D002921:Cicatrix / Q000209:etiology; D003320:Corneal Ulcer / Q000209:etiology; D004820:Epidermolysis Bullosa / Q000145:classification / Q000453:epidemiology / Q000235:genetics / Q000523:psychology; D005767:Gastrointestinal Diseases / Q000209:etiology; D006801:Humans; D007674:Kidney Diseases / Q000209:etiology; D024802:Nurse's Role; D010024:Osteoporosis / Q000209:etiology; D010146:Pain / Q000209:etiology; D035583:Rare Diseases; D013222:State Medicine; D006113:United Kingdom / Q000453:epidemiology