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Providing a precise diagnosis is the first step in approaching a patient suspected with EB and will yield invaluable prognostic information. The diagnosis of EB is based on a combination of clinical features, family history and laboratory findings.
11 sty 2024 · The prognosis in epidermolysis bullosa is variable and dictated by the specific causative subtype and the complications arising from cutaneous and extracutaneous manifestations. In some subtypes, particularly those that are milder, epidermolysis bullosa has minimal impact on life expectancy.
2 sie 2024 · Targeted corrective therapies are the ultimate way of treating epidermolysis bullosa (EB). In vivo gene therapy is entering the stage of clinical application. Beremagene geperpavec (B-VEC) has been approved by the FDA and has become the first gene therapy available to patients with dystrophic EB.
Abstract. Inherited epidermolysis bullosa (EB) comprises rare disorders that manifest with fragility and blistering of the skin and mucous membranes, with variable clinical severity.
These recommendations have been developed from a literature review and consensus from experts of the European Network for Rare Skin Disorders (ERN-Skin) to aid decision-making and optimize clinical care by non-EB expert health professionals encountering emergency situations in babies, children and adults with EB.
26 wrz 2023 · Epidermolysis bullosa is a group of inherited disorders characterised by mechanical fragility of the skin and epithelial tissues. Presents as recurrent erosions, blisters, and scars. Risk of extracutaneous complications, resulting from recurrent blistering or scarring of tissues.
24 wrz 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.
