Laminin 332-Dependent YAP Dysregulation Depletes Epidermal Stem Cells in Junctional Epidermolysis Bullosa [PDF]
Summary: Laminin 332-deficient junctional epidermolysis bullosa (JEB) is a severe genetic skin disease. JEB is marked by epidermal stem cell depletion, the origin of which is unknown.
Giorgio De Santis +2 more
exaly +4 more sources
Efficacy of intradermal allogeneic fibroblast injections in junctional epidermolysis bullosa
Objective — to assess the efficacy and safety of intradermal injections of allogeneic fibroblasts into non-healing wounds in a patient with junctional epidermolysis bullosa.
Alexey A. Kubanov +4 more
doaj +2 more sources
Junctional epidermolysis bullosa in a calf [PDF]
A case of epidermolysis bullosa in a calf descendent from a Gir bull and a Gir crossbreed cow is reported. The calf presented with exungulation of all hooves, widespread erosions and crusts on the skin, and ulcers in the oral cavity. Histologically, the skin showed subepidermal separation with clefts occasionally filled with eosinophilic clear fluid ...
Gildenor X, Medeiros +5 more
openaire +3 more sources
Junctional Epidermolysis Bullosa
Epidermolysis bullosa (EB) encompasses a heterogeneous group of genodermatoses, characterized by fragility and blistering of the skin, often associated with extracutaneous manifestations. The level of vesiculation within the skin defines 3 major subtypes of EB: EB simplex, junctional EB, and dystrophic EB.
Kao, Chuan-Hong +5 more
openaire +3 more sources
Junctional Epidermolysis Bullosa in a 30-day-old Infant: A Case Report [PDF]
Epidermolysis bullosa is a group of hereditary mechanobullous disorders which are associated with appearance of bullae secondary to physical stress like heat or mechanical trauma or sometimes without any trigger.
Pratima Bisen +4 more
doaj +2 more sources
Epidermolysis Bullosa—A Different Genetic Approach in Correlation with Genetic Heterogeneity
Epidermolysis bullosa is a heterogeneous group of rare genetic disorders characterized by mucocutaneous fragility and blister formation after minor friction or trauma. There are four major epidermolysis bullosa types based on the ultrastructural level of
Monica-Cristina Pânzaru +4 more
doaj +2 more sources
Dermatofibrosarcoma protuberans in a young patient with epidermolysis bullosa: a case report
Background Epidermolysis bullosa is a group of rare inherited skin diseases characterized by blister formation following mechanical skin trauma. Epidermolysis bullosa is associated with increased skin cancer rates, predominantly squamous cell carcinomas,
B. Bonaventura +4 more
doaj +2 more sources
Weekly Intraperitoneal Injection of Tamoxifen in an Inducible In Vivo Model of Junctional Epidermolysis Bullosa Generates Early and Advanced Disease Phenotypes [PDF]
Junctional epidermolysis bullosa caused by loss-of-function variants in genes encoding the skin basement membrane proteins laminin 332, type XVII collagen, or integrin α6β4 affects patients from birth with severe blistering, eventually leading to ...
Eleri Mai Jones +5 more
doaj +2 more sources
Junctional epidermolysis bullosa [PDF]
“Vlinderkinderen” worden ze genoemd, de patiëntjes met epidermolysis bullosa (EB) met een huid zo teer als de vleugels van een vlinder. Er worden zo’n 25 typen EB onderscheiden, waarvan de oorzaak in één van de 15 betrokken genen kan liggen. Junctionele epidermolysis bullosa (JEB) is een EB type met splijting door de junctionele laag tussen opperhuid ...
Yuen, Wing Yan
core +6 more sources
Independent COL17A1 Variants in Cats with Junctional Epidermolysis Bullosa. [PDF]
Epidermolysis bullosa (EB), characterized by defective adhesion of the epidermis to the dermis, is a heterogeneous disease with many subtypes in human patients and domestic animals. We investigated two unrelated cats with recurring erosions and ulcers on
Kiener S +10 more
europepmc +4 more sources

