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Nature Reviews Disease Primers, 2020
Epidermolysis bullosa (EB) is an inherited, heterogeneous group of rare genetic dermatoses characterized by mucocutaneous fragility and blister formation, inducible by often minimal trauma. A broad phenotypic spectrum has been described, with potentially severe extracutaneous manifestations, morbidity and mortality.
Bardhan, Ajoy +12 more
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Epidermolysis bullosa (EB) is an inherited, heterogeneous group of rare genetic dermatoses characterized by mucocutaneous fragility and blister formation, inducible by often minimal trauma. A broad phenotypic spectrum has been described, with potentially severe extracutaneous manifestations, morbidity and mortality.
Bardhan, Ajoy +12 more
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Oral Surgery, Oral Medicine, Oral Pathology, 1989
During the last 10 years, there has been considerable progress in the knowledge of epidermolysis bullosa, which has led to recognition of at least 18 different varieties. This review article attempts to classify these varieties and to emphasize the orodental findings in patients with epidermolysis bullosa.
H O, Sedano, R J, Gorlin
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During the last 10 years, there has been considerable progress in the knowledge of epidermolysis bullosa, which has led to recognition of at least 18 different varieties. This review article attempts to classify these varieties and to emphasize the orodental findings in patients with epidermolysis bullosa.
H O, Sedano, R J, Gorlin
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British Journal of Hospital Medicine, 2006
Over the last decade, defining the molecular pathology of the inherited blistering condition, epidermolysis bullosa, has led to more accurate diagnoses, better genetic counselling, the feasibility of DNA-based prenatal diagnosis, and the possibility of newer forms of treatment, including somatic gene therapy.
McGrath, J A, Mellerio, J E
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Over the last decade, defining the molecular pathology of the inherited blistering condition, epidermolysis bullosa, has led to more accurate diagnoses, better genetic counselling, the feasibility of DNA-based prenatal diagnosis, and the possibility of newer forms of treatment, including somatic gene therapy.
McGrath, J A, Mellerio, J E
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Annual Review of Medicine, 1993
Epidermolysis bullosa is a group of genetically determined diseases characterized by abnormal fragility of the skin and mucosa. In this chapter, we review current thinking about classification, pathogenesis, and molecular genetics, and we discuss management guidelines.
A N, Lin, D M, Carter
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Epidermolysis bullosa is a group of genetically determined diseases characterized by abnormal fragility of the skin and mucosa. In this chapter, we review current thinking about classification, pathogenesis, and molecular genetics, and we discuss management guidelines.
A N, Lin, D M, Carter
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International Journal of Dermatology, 1985
ABSTRACT: Nineteen patients with a ariety of types of epidermolysis bullosa were initially assessed in an open study of a treatment regime for the genetic type of epidermolysis bullosa. Nine of the ten patients admitted to the hospital for treatment showed definite objective improvement with decreased number of blisters and increased rate of healing ...
R M, Haber, C A, Ramsay, L B, Boxall
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ABSTRACT: Nineteen patients with a ariety of types of epidermolysis bullosa were initially assessed in an open study of a treatment regime for the genetic type of epidermolysis bullosa. Nine of the ten patients admitted to the hospital for treatment showed definite objective improvement with decreased number of blisters and increased rate of healing ...
R M, Haber, C A, Ramsay, L B, Boxall
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Epidermolysis Bullosa Pruriginosa
Dermatology, 2009Epidermolysis bullosa (EB) pruriginosa is a rare clinical subset of dystrophic EB, characterized by marked itching and presence of prurigo-like or lichenoid features. In order to further delineate the phenotype and understand the pathogenesis of this disorder, the clinical, histological and ultrastructural findings of a 19-year-old patient presenting a
S, Cambiaghi +4 more
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Der Hautarzt, 2009
Epidermolysis bullosa (EB) represents a group of diseases characterized by skin fragility usually developing blisters after minimal trauma. The clinical picture ranges from mild subtypes with minor skin reactions to severe forms with lethal outcome within the first months of life.
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Epidermolysis bullosa (EB) represents a group of diseases characterized by skin fragility usually developing blisters after minimal trauma. The clinical picture ranges from mild subtypes with minor skin reactions to severe forms with lethal outcome within the first months of life.
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Inherited epidermolysis bullosa
Arkhiv patologii, 2018To summarize an update on epidermolysis bullosa as a polymorphic group of inherited diseases with a failure of epidermal-dermal integrity. Emphasis is placed on the role of transmission electron microscopy in diagnosis and search directions for new types of the abnormality and its molecular markers.
S G, Lykova +5 more
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Ugeskrift for laeger, 2017
Epidermolysis bullosa (EB) is a rare genodermatosis. A new classification system is presented, distinguishing the subtypes of EB, and this system is based on the phenotype, mode of inheritance, ultrastructure, immunofluorescence findings, and specific mutation(s) present. EB is inherited in an autosomal dominant or -recessive fashion.
Firing, Camilla, Bygum, Anette
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Epidermolysis bullosa (EB) is a rare genodermatosis. A new classification system is presented, distinguishing the subtypes of EB, and this system is based on the phenotype, mode of inheritance, ultrastructure, immunofluorescence findings, and specific mutation(s) present. EB is inherited in an autosomal dominant or -recessive fashion.
Firing, Camilla, Bygum, Anette
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Epidermolysis Bullosa of the Esophagus
Archives of Pediatrics & Adolescent Medicine, 1969EPIDERMOLYSIS bullosa is a rare chronic hereditary disease of the skin and mucous membranes characterized by development of bullae following slight trauma. Newcomber et al 1 described two main forms of the disease: simplex and dystrophic, the latter occurring in both dominant and recessive forms.
E, Dupree, F, Hodges, J L, Simon
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