Results 41 to 50 of about 21,752 (228)

Pain and quality of life evaluation in patients with localized epidermolysis bullosa simplex

Orphanet Journal of Rare Diseases, 2017
Background A localized form of epidermolysis bullosa simplex (EBS-l) is considered one of the mildest forms of epidermolysis bullosa (EB), with blisters limited to the palms and soles. However, these lesions can be very painful.
Jennifer Brun, Christine Chiaverini, Caroline Devos, Stéphanie Leclerc-Mercier, Juliette Mazereeuw, Emmanuelle Bourrat, Annabel Maruani, Stéphanie Mallet, Claire Abasq, Alice Phan, Pierre Vabres, Ludovic Martin, Christine Bodemer, Sylvie Lagrange, Jean-Philippe Lacour, the Research Group of the French Society of Pediatric Dermatology   +15 more
doaj   +1 more source

Whole-genome expression Profiling in skin reveals sYK as a Key regulator of inflammation in experimental epidermolysis Bullosa acquisita [PDF]

, 2018
Because of the morbidity and limited therapeutic options of autoimmune diseases, there is a high, and thus far, unmet medical need for development of novel treatments.
Aga, Behnen, Bieber, Bieber, Buijsrogge, Carvalho, Chen, Chiriac, Corr, Dorward, Efron, Futosi, Geahlen, Genovese, Hellberg, Hirose, Hirose, Huang, Iwata, Jakus, Kalies, Karsten, Kasperkiewicz, Kasperkiewicz, Kasprick, Kasprick, Kemmer, Kleinau, Koga, Kovács, Kulkarni, Langfelder, Langfelder, Lin, Ludwig, Ludwig, Ludwig, Mihai, Mocsai, Müller, Németh, Németh, Patterson, Pihur, Pliyev, Recke, Recke, Renckens, Sadeghi, Sadeghi, Schmidt, Sezin, Shimanovich, Sitaru, van der Steen, Xue, Yamamoto, Zhao   +57 more
core   +3 more sources

Clinical management for epidermolysis bullosa dystrophica [PDF]

, 2008
Epidermolysis bullosa (EB) consists of a group of genetic hereditary disorders in which patients frequently present fragile skin and mucosa that form blisters following minor trauma.
CANDIDO, Liliani A., MACHADO, Maria Aparecida A. M., OLIVEIRA, Thais M., SAKAI, Vivien T., SILVA, Salete M. B.   +4 more
core   +3 more sources

Epidermolysis bullosa acquisita

Clinics in Dermatology, 2012
Epidermolysis bullosa acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis.
Rishu, Gupta, David T, Woodley, Mei, Chen   +2 more
openaire   +3 more sources

First Report of Homozygous COL7A1 c.5756delG Mutation Causing Recessive Dystrophic Epidermolysis Bullosa in a Non‐Consanguineous Japanese Family

JEADV Clinical Practice, EarlyView.
ABSTRACT Severe recessive dystrophic epidermolysis bullosa (RDEB) is usually caused by biallelic loss‐of‐function mutations in COL7A1. While the c.5756delG variant has been previously reported in heterozygous form, its clinical impact in homozygosity has not been described.
Nozomi Kohama, Sayaka Yamaguchi, Teruki Yanagi, Yumi Akamine, Kimiko Nonaka, Daisuke Utsumi, Kenzo Takahashi   +6 more
wiley   +1 more source

Rat model for dominant dystrophic epidermolysis bullosa: glycine substitution reduces collagen VII stability and shows gene-dosage effect. [PDF]

PLoS ONE, 2013
Dystrophic epidermolysis bullosa, a severely disabling hereditary skin fragility disorder, is caused by mutations in the gene coding for collagen VII, a specialized adhesion component of the dermal-epidermal junction zone.
Alexander Nyström, Jens Buttgereit, Michael Bader, Tatiana Shmidt, Cemil Ozcelik, Ingrid Hausser, Leena Bruckner-Tuderman, Johannes S Kern   +7 more
doaj   +1 more source

Response to Oral Dapsone in a Patient With Generalised Junctional Epidermolysis Bullosa

JEADV Clinical Practice, EarlyView.
ABSTRACT Dapsone is a potent antibacterial agent used as a first‐line treatment for leprosy and employed also as an anti‐inflammatory agent. Additionally, dapsone is used for a variety of other cutaneous conditions. Herein, we report a case of intermediate junctional epidermolysis bullosa that improved significantly with oral 4,4’‐diamino‐diphenyl ...
Shiho Mori, Manabu Fujimoto, Katsuto Tamai   +2 more
wiley   +1 more source

Full-thickness Skin Grafts for Hand Contractures in an Adult Patient with Junctional Epidermolysis Bullosa: A Case Report

Journal of Plastic and Reconstructive Surgery, 2023
Epidermolysis bullosa is a group of inherited skin fragility disorders with blister formation in the basement membrane zone. Chronic scarring after repeated blistering of the hands causes narrowing of the first web, flexion contractures of the digits ...
Sayaka Nakamura, Susumu Saito, Yoshihiro Ishida, Yo Kaku, Ken Natsuga, Naoki Morimoto   +5 more
doaj  

Reproductive alternatives for patients with dystrophic epidermolysis bullosa

Einstein (São Paulo)
Epidermolysis bullosa describes a group of skin conditions caused by mutations in genes encoding proteins related to dermal-epidermal adhesion. In the United States, 50 cases of epidermolysis bullosa per 1 million live births are estimated, 92% of which ...
Denise Maria Christofolini, José Ricardo Magliocco Ceroni, Giovanna Guimarães Soares, Gustavo Bertollini Lamy, Ana Carolina Nemeth Calvo, Tamara Alba dos Santos, Bianca Del Bel Sonoda, Bianca Bianco, Caio Parente Barbosa   +8 more
doaj   +1 more source

Pro-Inflammatory Chemokines and Cytokines Dominate the Blister Fluid Molecular Signature in Patients with Epidermolysis Bullosa and Affect Leukocyte and Stem Cell Migration. [PDF]

, 2017
Hereditary epidermolysis bullosa (EB) is associated with skin blistering and the development of chronic nonhealing wounds. Although clinical studies have shown that cell-based therapies improve wound healing, the recruitment of therapeutic cells to ...
Alexeev, Vitali, Fortuna, Giulio, Igoucheva, Olga, Mukhtarzada, Lila, Palisson, Francis, Salas-Alanis, Julio Cesar, South, Andrew P., Uitto, Jouni   +7 more
core   +1 more source