Results 151 to 160 of about 13,589 (213)

Cutaneous Squamous Cell Carcinoma in Epidermolysis Bullosa: A Review of Pathogenesis, Diagnosis and Management. [PDF]

Cancers (Basel)
Chandrasekaran A, Moser JC.
europepmc   +1 more source

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Junctional Epidermolysis Bullosa of the Larynx

Pediatrics, 1986
Epidermolysis bullosa is a rare genetically determined, dermatologic disease in which minor trauma causes blister formation.1 A new variant of hereditary epidermolysis bullosa, generalized atrophic benign epidermolysis bullosa, junctional form, has been recently reported.2 Airway involvement has not been a notable feature of this disease. We report the
Sylvan E Stool, Susan B Mallory
exaly   +3 more sources

Upper Airway Complications of Junctional Epidermolysis Bullosa

Journal of Pediatrics, 2012
To assess the incidence of upper airway pathology in patients with junctional epidermolysis bullosa (JEB).We conducted a retrospective chart review of all patients with JEB who came to an interdisciplinary epidermolysis bullosa center at a tertiary care institution between 2004 and 2010.Twenty-five patients with JEB were identified, and 12 patients ...
Richard G Azizkhan, Anne W Lucky, Ravindhra G Elluru   +2 more
exaly   +3 more sources

Junctional Epidermolysis Bullosa of the Larynx

Annals of Otology, Rhinology and Laryngology, 1992
Epidermolysis bullosa (EB) is a group of rare inherited disorders in which minor trauma causes blister formation in the skin and mucosa, including the esophagus. Morbidity varies with the type of disease and ranges from occasional trivial skin blisters to death in infancy. Laryngeal involvement presenting as hoarseness and respiratory distress has been
Andrew N Lin, D Martin Carter, A N Lin
exaly   +3 more sources

Transgenic Epidermal Cultures for Junctional Epidermolysis Bullosa — 5-Year Outcomes

New England Journal of Medicine, 2021
Inherited junctional epidermolysis bullosa is a severe genetic skin disease that leads to epidermal loss caused by structural and mechanical fragility of the integuments. There is no established cure for junctional epidermolysis bullosa.
Michele De Luca, Tobias Hirsch
exaly   +2 more sources

Leading edge: emerging drug, cell, and gene therapies for junctional epidermolysis bullosa

Expert Opinion on Biological Therapy, 2020
Introduction Junctional epidermolysis bullosa (JEB) is a rare inherited genetic disorder with limited treatments beyond palliative care. A major hallmark of JEB is skin blistering caused by functional loss or complete absence of major structural proteins
Allison R Keith, Christen L Ebens, Jakub Tolar   +2 more
exaly   +2 more sources

Treatment With Ataluren for Wound Healing and Health Complications in a Patient With Junctional Epidermolysis Bullosa.

JAMA dermatology, 2023
This case report describes an 11-year-old boy with widespread chronic wounds, frequent corneal erosions, dental caries, a tracheostomy, and failure to thrive.
G. Orlowski, Shinya U. Amano, K. Flanagan, Kerri Rieger, M. Marinkovich, K. Wiss   +5 more
semanticscholar   +1 more source

Genotypic and phenotypic analysis of 34 cases of inherited junctional epidermolysis bullosa caused by COL17A1 mutations

British Journal of Dermatology, 2021
Inherited epidermolysis bullosa (EB) defines a heterogeneous group of genodermatoses characterized by skin and/or mucosa fragility resulting in blistering.
A. Hérissé, A. Charlesworth, N. Bellon, S. Leclerc‐Mercier, E. Bourrat, S. Hadj-Rabia, C. Bodemer, J. Lacour, C. Chiaverini   +8 more
semanticscholar   +1 more source

Junctional Epidermolysis Bullosa

American Journal of Diseases of Children, 1983
Junctional epidermolysis bullosa (EB) is a rare, heritable, blistering disease of the skin characterized by presence of bullae at birth, lack of scarring of the lesions, and early death. To date there has been no effective treatment for the disease. Phenytoin, which decreases collagenase activity in human skin explants and fibroblast cultures, has been
M F, Guill, B B, Wray, R B, Rogers, K B, Yancey, B S, Allen   +4 more
openaire   +2 more sources

Cicatricial junctional epidermolysis bullosa

Journal of the American Academy of Dermatology, 1985
Six types of junctional epidermolysis bullosa (JEB) have been described previously. Each of these types may develop atrophy after recurrent blistering but healing with scar formation does not occur. We describe three cases with a new type of JEB that we have designated as cicatricial junctional epidermolysis bullosa (CJEB).
R M, Haber, W, Hanna, C A, Ramsay, L B, Boxall   +3 more
openaire   +2 more sources