Abstract:
BACKGROUND:Since publication in 2000 of the Second International Consensus Report on Diagnosis and Classification of Epidermolysis Bullosa, many advances have been made to our understanding of this group of diseases, both clinically and molecularly. At the same time, new epidermolysis bullosa (EB) subtypes have been described and similarities with some other diseases have been identified. OBJECTIVE:We sought to arrive at a new consensus of the classification of EB subtypes. RESULTS:We now present a revised classification system that takes into account the new advances, as well as encompassing other inherited diseases that should also be included within the EB spectrum, based on the presence of blistering and mechanical fragility. Current recommendations are made on the use of specific diagnostic tests, with updates on the findings known to occur within each of the major EB subtypes. Electronic links are also provided to informational and laboratory resources of particular benefit to clinicians and their patients. LIMITATIONS:As more becomes known about this disease, future modifications may be needed. The classification system has been designed with sufficient flexibility for these modifications. CONCLUSION:This revised classification system should assist clinicians in accurately diagnosing and subclassifying patients with EB.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Fine JD,Eady RA,Bauer EA,Bauer JW,Bruckner-Tuderman L,Heagerty A,Hintner H,Hovnanian A,Jonkman MF,Leigh I,McGrath JA,Mellerio JE,Murrell DF,Shimizu H,Uitto J,Vahlquist A,Woodley D,Zambruno Gdoi
10.1016/j.jaad.2008.02.004subject
Has Abstractpub_date
2008-06-01 00:00:00pages
931-50issue
6eissn
0190-9622issn
1097-6787pii
S0190-9622(08)00208-9journal_volume
58pub_type
共识发展会议,杂志文章abstract::Cicatricial pemphigoid is a chronic, presumed autoimmune, blistering disease of the mucous membranes and occasionally of the skin. The characteristic feature is scarring at the sites of healing. We report the use of thalidomide as a therapeutic agent to control previously resistant disease. ...
journal_title:Journal of the American Academy of Dermatology
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abstract::A panel of antibodies reactive in routinely fixed, paraffin-embedded tissue sections was compared with a panel of antibodies reactive in frozen sections for the immunophenotyping of cutaneous lymphoproliferative disorders. Three T cell-associated markers (UCHL1, MT-1, MT-2, six B cell-associated markers (MB-1, MB-2, L...
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