Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility

Abstract: Background
Several new genes and clinical subtypes have been identified since the publication in 2014 of the report of the last International Consensus Meeting on Epidermolysis Bullosa (EB).

Objectives
We sought to reclassify disorders with skin fragility, with a focus on EB, based on new clinical and molecular data.

Methods
This was a consensus expert review.

Results
In this latest consensus report, we introduce the concept of genetic disorders with skin fragility, of which classical EB represents the prototype. Other disorders with skin fragility, where blisters are a minor part of the clinical picture or are not seen because skin cleavage is very superficial, are classified as separate categories. These include peeling skin disorders, erosive disorders, hyperkeratotic disorders, and connective tissue disorders with skin fragility. Because of the common manifestation of skin fragility, these ‘EB‐related’ disorders should be considered under the EB umbrella in terms of medical and socioeconomic provision of care.

Conclusions
The proposed classification scheme should be of value both to clinicians and researchers, emphasizing both clinical and genetic features of EB

Object Details

• Location: Deutsche Nationalbibliothek Frankfurt am Main
• Extent: Online-Ressource
• Language: Englisch
• Notes: British journal of dermatology. - 183, 4 (2020) , 614-627, ISSN: 1365-2133

Classification and Topics

• Keyword: Epidermolysis bullosa hereditaria

Contributors, Places and Time

• Event: Veröffentlichung
• (where): Freiburg
• (who): Universität
• (when): 2020
• Creator: Has, Cristina ; Bauer, Johann ; Bodemer, C. ; Bolling, Maria C. ; Bruckner-Tuderman, Leena ; Diem, Anja ; Fine, Jo-David ; Heagerty, Adrian H. ; Hovnanian, Alain ; Marinkovich, M. Peter ; Martinez, Anna E. ; McGrath, John A. ; Moss, Celia ; Murrell, Dédée F. ; Palisson, Francis ; Schwieger-Briel, Agnes ; Sprecher, Eli ; Tamai, Katsuto ; Uitto, Jouni ; Woodley, David T. ; Zambruno, Giovanna ; Mellerio, Jemima E.

Further information

• DOI: 10.1111/bjd.18921
• URN: urn:nbn:de:bsz:25-freidok-1694552
• Rights: Der Zugriff auf das Objekt ist unbeschränkt möglich.
• Last update: 25.03.2025, 1:53 PM CET