Literature DB >> 32039456

Skin Fragility: Perspectives on Evidence-based Therapies.

Leena Bruckner-Tuderman1.   

Abstract

The term skin fragility disorders describes a group of conditions in which the structural integrity of the skin is compromised and its resistance to external shear forces diminished. Skin fragility can have different causes, ranging from genetic variations to inflammatory or physical phenomena. The genetic skin fragility disorders, collectively called epidermolysis bullosa, serve as a paradigm for the study of causes and mechanisms of skin fragility. Recent biomedical research has revealed substantial genetic heterogeneity of the epidermolysis bullosa group, delivered ample new knowledge on its pathophysiology, and facilitated the design of evidence-based therapeutic strategies. The therapy development process extends from in vitro testing to preclinical validation in animal models, and clinical trials. This article reviews different approaches to curative and symptom-relief therapies, and appraises their status and perspectives for clinical implementation.

Entities:  

Keywords:  genodermatosis; molecular therapy; symptom-relief; skin blistering

Mesh:

Year:  2020        PMID: 32039456      PMCID: PMC9128950          DOI: 10.2340/00015555-3398

Source DB:  PubMed          Journal:  Acta Derm Venereol        ISSN: 0001-5555            Impact factor:   3.875


  58 in total

1.  High Local Concentrations of Intradermal MSCs Restore Skin Integrity and Facilitate Wound Healing in Dystrophic Epidermolysis Bullosa.

Authors:  Tobias Kühl; Markus Mezger; Ingrid Hausser; Rupert Handgretinger; Leena Bruckner-Tuderman; Alexander Nyström
Journal:  Mol Ther       Date:  2015-04-10       Impact factor: 11.454

2.  Safety and early efficacy outcomes for lentiviral fibroblast gene therapy in recessive dystrophic epidermolysis bullosa.

Authors:  Su M Lwin; Farhatullah Syed; Wei-Li Di; Tendai Kadiyirire; Lu Liu; Alyson Guy; Anastasia Petrova; Alya Abdul-Wahab; Fiona Reid; Rachel Phillips; Maria Elstad; Christos Georgiadis; Sophia Aristodemou; Patricia A Lovell; James R McMillan; John Mee; Snaigune Miskinyte; Matthias Titeux; Linda Ozoemena; Rashida Pramanik; Sonia Serrano; Racheal Rowles; Clarisse Maurin; Elizabeth Orrin; Magdalena Martinez-Queipo; Ellie Rashidghamat; Christos Tziotzios; Alexandros Onoufriadis; Mei Chen; Lucas Chan; Farzin Farzaneh; Marcela Del Rio; Jakub Tolar; Johann W Bauer; Fernando Larcher; Michael N Antoniou; Alain Hovnanian; Adrian J Thrasher; Jemima E Mellerio; Waseem Qasim; John A McGrath
Journal:  JCI Insight       Date:  2019-06-06

3.  Gentamicin induces LAMB3 nonsense mutation readthrough and restores functional laminin 332 in junctional epidermolysis bullosa.

Authors:  Vadim Lincoln; Jon Cogan; Yingping Hou; Michaela Hirsch; Michelle Hao; Vitali Alexeev; Michele De Luca; Laura De Rosa; Johann W Bauer; David T Woodley; Mei Chen
Journal:  Proc Natl Acad Sci U S A       Date:  2018-06-26       Impact factor: 11.205

4.  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

5.  A phase II randomized vehicle-controlled trial of intradermal allogeneic fibroblasts for recessive dystrophic epidermolysis bullosa.

Authors:  Supriya S Venugopal; Wenfei Yan; John W Frew; Heather I Cohn; Lesley M Rhodes; Kim Tran; Wei Melbourne; Janis A Nelson; Marian Sturm; Janice Fogarty; M Peter Marinkovich; Satomi Igawa; Akemi Ishida-Yamamoto; Dedee F Murrell
Journal:  J Am Acad Dermatol       Date:  2013-09-24       Impact factor: 11.527

6.  Safety and Wound Outcomes Following Genetically Corrected Autologous Epidermal Grafts in Patients With Recessive Dystrophic Epidermolysis Bullosa.

Authors:  Zurab Siprashvili; Ngon T Nguyen; Emily S Gorell; Kylie Loutit; Phuong Khuu; Louise K Furukawa; H Peter Lorenz; Thomas H Leung; Douglas R Keene; Kerri E Rieger; Paul Khavari; Alfred T Lane; Jean Y Tang; M Peter Marinkovich
Journal:  JAMA       Date:  2016-11-01       Impact factor: 56.272

7.  Cell- and protein-based therapy approaches for epidermolysis bullosa.

Authors:  Alexander Nyström; Leena Bruckner-Tuderman; Johannes S Kern
Journal:  Methods Mol Biol       Date:  2013

8.  Genotype, Clinical Course, and Therapeutic Decision Making in 76 Infants with Severe Generalized Junctional Epidermolysis Bullosa.

Authors:  Johanna Hammersen; Cristina Has; Nora Naumann-Bartsch; Daniel Stachel; Dimitra Kiritsi; Stephan Söder; Mathilde Tardieu; Markus Metzler; Leena Bruckner-Tuderman; Holm Schneider
Journal:  J Invest Dermatol       Date:  2016-06-29       Impact factor: 8.551

9.  Losartan ameliorates dystrophic epidermolysis bullosa and uncovers new disease mechanisms.

Authors:  Alexander Nyström; Kerstin Thriene; Venugopal Mittapalli; Johannes S Kern; Dimitra Kiritsi; Jörn Dengjel; Leena Bruckner-Tuderman
Journal:  EMBO Mol Med       Date:  2015-09       Impact factor: 12.137

10.  Diacerein inhibits the pro-atherogenic & pro-inflammatory effects of IL-1 on human keratinocytes & endothelial cells.

Authors:  Girish C Mohan; Huayi Zhang; Lei Bao; Benjamin Many; Lawrence S Chan
Journal:  PLoS One       Date:  2017-03-21       Impact factor: 3.240

View more
  2 in total

1.  Clinical trial of ABCB5+ mesenchymal stem cells for recessive dystrophic epidermolysis bullosa.

Authors:  Dimitra Kiritsi; Kathrin Dieter; Elke Niebergall-Roth; Silvia Fluhr; Cristina Daniele; Jasmina Esterlechner; Samar Sadeghi; Seda Ballikaya; Leoni Erdinger; Franziska Schauer; Stella Gewert; Martin Laimer; Johann W Bauer; Alain Hovnanian; Giovanna Zambruno; May El Hachem; Emmanuelle Bourrat; Maria Papanikolaou; Gabriela Petrof; Sophie Kitzmüller; Christen L Ebens; Markus H Frank; Natasha Y Frank; Christoph Ganss; Anna E Martinez; John A McGrath; Jakub Tolar; Mark A Kluth
Journal:  JCI Insight       Date:  2021-11-22

2.  Translational perspectives to treat Epidermolysis bullosa-Where do we stand?

Authors:  Christine Prodinger; Johann W Bauer; Martin Laimer
Journal:  Exp Dermatol       Date:  2020-11       Impact factor: 4.511

  2 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.