Literature DB >> 26029334

Recessive dystrophic epidermolysis bullosa: a review of disease pathogenesis and update on future therapies.

Luis Soro1, Cynthia Bartus2, Stephen Purcell3.   

Abstract

OBJECTIVE: Review the pathogenesis of recessive dystrophic epidermolysis bullosa and provide an update on research currently underway that is aimed at treating and potentially curing this severe skin disorder.
DESIGN: Review article.
SETTING: Private practice and large teaching hospital. PARTICIPANTS: None. MEASUREMENTS: N/A.
RESULTS: Currently, patients with recessive dystrophic epidermolysis bullosa are managed with only supportive care. However, there are several promising new treatment avenues that may help patients in the future. These include gene therapy, cell therapy, and protein-based therapy. Each approach offers distinct advantages and disadvantages.
CONCLUSIONS: The advances in understanding the molecular basis for epidermolysis bullosa over the last few decades has led to significant progress in devising new treatment options. Though many of these approaches remain several years away from regular implementation, it is an exciting time for research in the field.

Entities:  

Year:  2015        PMID: 26029334      PMCID: PMC4445895     

Source DB:  PubMed          Journal:  J Clin Aesthet Dermatol        ISSN: 1941-2789


  20 in total

1.  Epidermolysis bullosa. II. Type VII collagen mutations and phenotype-genotype correlations in the dystrophic subtypes.

Authors:  Roslyn Varki; Sara Sadowski; Jouni Uitto; Ellen Pfendner
Journal:  J Med Genet       Date:  2006-09-13       Impact factor: 6.318

2.  Fibroblasts show more potential as target cells than keratinocytes in COL7A1 gene therapy of dystrophic epidermolysis bullosa.

Authors:  Maki Goto; Daisuke Sawamura; Kei Ito; Masataka Abe; Wataru Nishie; Kaori Sakai; Akihiko Shibaki; Masashi Akiyama; Hiroshi Shimizu
Journal:  J Invest Dermatol       Date:  2006-04       Impact factor: 8.551

3.  Gene therapy of inherited skin adhesion disorders: a critical overview.

Authors:  M De Luca; G Pellegrini; F Mavilio
Journal:  Br J Dermatol       Date:  2009-04-20       Impact factor: 9.302

Review 4.  Dystrophic epidermolysis bullosa: pathogenesis and clinical features.

Authors:  Leena Bruckner-Tuderman
Journal:  Dermatol Clin       Date:  2010-01       Impact factor: 3.478

5.  Bone marrow transplantation for recessive dystrophic epidermolysis bullosa.

Authors:  John E Wagner; Akemi Ishida-Yamamoto; John A McGrath; Maria Hordinsky; Douglas R Keene; David T Woodley; Mei Chen; Megan J Riddle; Mark J Osborn; Troy Lund; Michelle Dolan; Bruce R Blazar; Jakub Tolar
Journal:  N Engl J Med       Date:  2010-08-12       Impact factor: 91.245

6.  Potential of fibroblast cell therapy for recessive dystrophic epidermolysis bullosa.

Authors:  Tracy Wong; Luke Gammon; Lu Liu; Jemima E Mellerio; Patricia J C Dopping-Hepenstal; John Pacy; George Elia; Rosemary Jeffery; Irene M Leigh; Harshad Navsaria; John A McGrath
Journal:  J Invest Dermatol       Date:  2008-04-03       Impact factor: 8.551

7.  Normal and gene-corrected dystrophic epidermolysis bullosa fibroblasts alone can produce type VII collagen at the basement membrane zone.

Authors:  David T Woodley; Gerald G Krueger; Cynthia M Jorgensen; Janet A Fairley; Tom Atha; Yi Huang; Lawrence Chan; Douglas R Keene; Mei Chen
Journal:  J Invest Dermatol       Date:  2003-11       Impact factor: 8.551

8.  Adhesive stripping to remove epidermis in junctional epidermolysis bullosa for revertant cell therapy.

Authors:  A Gostynski; F C L Deviaene; A M G Pasmooij; H H Pas; M F Jonkman
Journal:  Br J Dermatol       Date:  2009-03-26       Impact factor: 9.302

9.  Correction of junctional epidermolysis bullosa by transplantation of genetically modified epidermal stem cells.

Authors:  Fulvio Mavilio; Graziella Pellegrini; Stefano Ferrari; Francesca Di Nunzio; Enzo Di Iorio; Alessandra Recchia; Giulietta Maruggi; Giuliana Ferrari; Elena Provasi; Chiara Bonini; Sergio Capurro; Andrea Conti; Cristina Magnoni; Alberto Giannetti; Michele De Luca
Journal:  Nat Med       Date:  2006-11-19       Impact factor: 53.440

10.  Two novel mutations on exon 8 and intron 65 of COL7A1 gene in two Chinese brothers result in recessive dystrophic epidermolysis bullosa.

Authors:  Ying Lin; Xue-Jun Chen; Wei Liu; Bo Gong; Jun Xie; Jun-Hao Xiong; Jing Cheng; Xi-Ling Duan; Zhao-Chun Lin; Lu-Lin Huang; Hui-Ying Wan; Xiao-Qi Liu; Lin-Hong Song; Zheng-Lin Yang
Journal:  PLoS One       Date:  2012-11-30       Impact factor: 3.240

View more
  9 in total

Review 1.  Pathogenetic Therapy of Epidermolysis Bullosa: Current State and Prospects.

Authors:  I I Ryumina; K V Goryunov; D N Silachev; Yu A Shevtsova; V A Babenko; N M Marycheva; Yu Yu Kotalevskaya; V V Zubkov; G T Zubkov
Journal:  Bull Exp Biol Med       Date:  2021-05-29       Impact factor: 0.804

2.  Pretibial dystrophic epidermolysis bullosa.

Authors:  Elisabeth de Albuquerque Cavalcanti Callegaro; Flavio Nappi; Rosana Lazzarini; Rute Facchini Lellis
Journal:  An Bras Dermatol       Date:  2017       Impact factor: 1.896

3.  End-stage kidney disease in patient with epidermolysis bullosa - what are the treatment options? - case report.

Authors:  Michał Małecki; Maciej Domański; Kazimierz Ciechanowski
Journal:  BMC Nephrol       Date:  2017-06-14       Impact factor: 2.388

Review 4.  A rare case of skin blistering and esophageal stenosis in the course of epidermolysis bullosa - case report and literature review.

Authors:  Agata Michalak; Halina Cichoż-Lach; Beata Prozorow-Król; Leszek Buk; Monika Dzida
Journal:  BMC Gastroenterol       Date:  2018-04-13       Impact factor: 3.067

Review 5.  Gene editing in dermatology: Harnessing CRISPR for the treatment of cutaneous disease.

Authors:  Catherine Baker; Matthew S Hayden
Journal:  F1000Res       Date:  2020-04-23

Review 6.  A systematic literature review of the disease burden in patients with recessive dystrophic epidermolysis bullosa.

Authors:  Jean Yuh Tang; M Peter Marinkovich; Eleanor Lucas; Emily Gorell; Albert Chiou; Ying Lu; Jodie Gillon; Dipen Patel; Dan Rudin
Journal:  Orphanet J Rare Dis       Date:  2021-04-13       Impact factor: 4.123

7.  Detection of Novel Biallelic Causative Variants in COL7A1 Gene by Whole-Exome Sequencing, Resulting in Congenital Recessive Dystrophic Epidermolysis Bullosa in Three Unrelated Families.

Authors:  Fozia Fozia; Rubina Nazli; May Mohammed Alrashed; Hazem K Ghneim; Zia Ul Haq; Musarrat Jabeen; Sher Alam Khan; Ijaz Ahmad; Mohammed Bourhia; Mourad A M Aboul-Soud
Journal:  Diagnostics (Basel)       Date:  2022-06-23

8.  Clinical characteristics, healthcare use, and annual costs among patients with dystrophic epidermolysis bullosa.

Authors:  James A Feinstein; Anna L Bruckner; Benjamin Chastek; Amy Anderson; Juan Roman
Journal:  Orphanet J Rare Dis       Date:  2022-09-29       Impact factor: 4.303

9.  An RNA-targeted therapy for dystrophic epidermolysis bullosa.

Authors:  Patricia Peking; Ulrich Koller; Blanca Duarte; Rodolfo Murillas; Susanne Wolf; Tobias Maetzig; Michael Rothe; Thomas Kocher; Marta García; Gabriele Brachtl; Axel Schambach; Fernando Larcher; Julia Reichelt; Johann W Bauer; Eva M Murauer
Journal:  Nucleic Acids Res       Date:  2017-09-29       Impact factor: 16.971

  9 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.