Epidermolysis bullosa: diagnosis and therapy

McMillan, J. R., Long, H.A., Akiyama, M., Shimizu, H. and Kimble, R. M. (2009) Epidermolysis bullosa: diagnosis and therapy. Wound Practice & Research, 17 2: 62-70.

Author McMillan, J. R.
Long, H.A.
Akiyama, M.
Shimizu, H.
Kimble, R. M.
Title Epidermolysis bullosa: diagnosis and therapy
Formatted title
Epidermolysis Bullosa (EB) - Diagnosis and Therapy
Journal name Wound Practice & Research   Check publisher's open access policy
ISSN 1323-2495
Publication date 2009-05
Year available 2009
Sub-type Critical review of research, literature review, critical commentary
Volume 17
Issue 2
Start page 62
End page 70
Total pages 9
Editor Allison Cowin
Michael Woodward
Place of publication West Leederville, WA
Publisher Cambridge
Collection year 2010
Language eng
Subject 110304 Dermatology
920117 Skin and Related Disorders
Abstract The hemidesmosome (HD)-anchoring filament complex comprises a multiprotein complex that aids the attachment of epidermal keratinocytes to the underlying basal lamina and dermis. The importance of the HD and its components is highlighted by genetic defects that cause congenital blistering skin diseases that are categorised under the epidermolysis bullosa (EB) group of disorders. EB disorders can be subcategorised into three main subtypes by the level of epidermal separation – within the basal keratinocyte (EB simplex – EBS), between the keratinocyte and underlying basal lamina (junctional EB – JEB), and separation beneath the basal lamina (dystrophic EB – DEB). HD-anchoring filament-related components – including keratins 5 and 14, plectin, a6ß4 integrin, collagen XVII, laminin 332 and collagen VII – have been demonstrated to harbour defects leading to EB disease. We summarise here the current understanding of the biological function of these HD-components and their involvement in EB in light of their functions in keratinocyte adhesion and also describe putative future therapeutic avenues that hold promise to alleviate the morbidity suffered by EB patients over the coming decades.
Keyword Epidermolysis bullosa
Q-Index Code C1
Q-Index Status Confirmed Code

Document type: Journal Article
Sub-type: Critical review of research, literature review, critical commentary
Collections: Excellence in Research Australia (ERA) - Collection
2010 Higher Education Research Data Collection
School of Medicine Publications
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Created: Thu, 26 Nov 2009, 09:39:35 EST by Michael Affleck on behalf of Paediatrics & Child Health - RBWH