Literature DB >> 12113646

Epidermolysis bullosa: new and emerging trends.

Shan Pai1, M Peter Marinkovich.   

Abstract

Epidermolysis bullosa is a family of inherited blistering skin disorders characterized by blister formation in response to mechanical trauma. Major types of epidermolysis bullosa include epidermolysis bullosa simplex, hemidesmosomal epidermolysis bullosa, junctional epidermolysis bullosa, and dystrophic epidermolysis bullosa. Current treatment for epidermolysis bullosa consists of supportive care for skin and other organ systems and entails a combination of wound management, infection support for chronic wounds, surgical management as needed, nutritional support, and preventative screening for squamous cell carcinoma in recessive dystrophic epidermolysis bullosa. The regimen must be tailored specifically to the severity and extent of skin and systemic involvement in each case. Recent studies have identified specific protein and genetic abnormalities for most epidermolysis bullosa subtypes. These new advancements in the understanding of molecular pathophysiology have provided much of the basis for current efforts to develop effective gene and protein therapy for epidermolysis bullosa.

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Year:  2002        PMID: 12113646     DOI: 10.2165/00128071-200203060-00001

Source DB:  PubMed          Journal:  Am J Clin Dermatol        ISSN: 1175-0561            Impact factor:   7.403


  8 in total

1.  Nexus between epidermolysis bullosa and transcriptional regulation by thyroid hormone in epidermal keratinocytes.

Authors:  Marjana Tomic-Canic; Olivera Stojadinovic; Brian Lee; Rebecca Walsh; Miroslav Blumenberg
Journal:  Clin Transl Sci       Date:  2008-05       Impact factor: 4.689

2.  Remodeling of the dermal-epidermal junction in bilayered skin constructs after silencing the expression of the p.R2622Q and p.G2623C collagen VII mutants.

Authors:  Andrzej Steplewski; Anthony Kasinskas; Andrzej Fertala
Journal:  Connect Tissue Res       Date:  2012-04-10       Impact factor: 3.417

Review 3.  Squamous Cell Carcinoma in Patients with Inherited Epidermolysis Bullosa: Review of Current Literature.

Authors:  Domenico Bonamonte; Angela Filoni; Aurora De Marco; Lucia Lospalluti; Eleonora Nacchiero; Valentina Ronghi; Anna Colagrande; Giuseppe Giudice; Gerardo Cazzato
Journal:  Cells       Date:  2022-04-17       Impact factor: 7.666

4.  Growth outcomes and complications after radiologic gastrostomy in 120 children.

Authors:  Evan Cole Lewis; Bairbre Connolly; Michael Temple; Philip John; Peter G Chait; Jennifer Vaughan; Joao G Amaral
Journal:  Pediatr Radiol       Date:  2008-07-12

5.  The use of cultured allogenic keratinocyte grafting in a patient with epidermolysis bullosa simplex.

Authors:  Kee Cheol Shin; Bo Young Park; Han Koo Kim; Woo Seob Kim; Tae Hui Bae
Journal:  Ann Dermatol       Date:  2011-12-27       Impact factor: 1.444

6.  Treatment of symptomatic epidermolysis bullosa simplex with botulinum toxin in a pediatric patient.

Authors:  Heather M Holahan; Ronda S Farah; Nkanyezi N Ferguson; Amy S Paller; Allison A Legler
Journal:  JAAD Case Rep       Date:  2016-07-14

7.  Inherited epidermolysis bullosa: clinical and therapeutic aspects.

Authors:  Vanessa Lys Simas Yamakawa Boeira; Erica Sales Souza; Bruno de Oliveira Rocha; Pedro Dantas Oliveira; Maria de Fátima Santos Paim de Oliveira; Vitória Regina Pedreira de Almeida Rêgo; Ivonise Follador
Journal:  An Bras Dermatol       Date:  2013 Mar-Apr       Impact factor: 1.896

8.  Pain in Patients with Dystrophic Epidermolysis Bullosa: Association with Anxiety and Depression.

Authors:  Giulio Fortuna; Massimo Aria; Rodrigo Cepeda-Valdes; Maria Guadalupe Moreno Trevino; Julio Cesar Salas-Alanís
Journal:  Psychiatry Investig       Date:  2017-11-07       Impact factor: 2.505

  8 in total

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