title: [Mucosal manifestations of epidermolysis bullosa : Clinical presentation and management].
abstract: Mucosal lesions occur with different prevalence and severity in all subtypes of hereditary epidermolysis bullosa (EB), a group of rare genodermatoses. They are associated with increased morbidity and mortality, especially in severe junctional and dystrophic subtypes. Despite progress in clinical approaches to curative therapy, the management of these patients is still primarily symptom-oriented. Current recommendations mainly rely on expert opinion and experience from health care professionals of specialized centers, since the rarity of this disease largely limits the availability and feasibility of randomized controlled trials. Accurate preventive and supportive care measures, however, can significantly lessen symptoms, avoid/ameliorate complications, and enhance the quality of life of these patients.
authors: Universitätsklinik für Dermatologie, Universitätsklinikum Salzburg, Müllner Hauptstr. 48, 5020, Salzburg, Österreich. ch.prodinger@salk.at.
authors: C
authors: C
authors: Prodinger
authors: Universitätsklinik für Dermatologie, Universitätsklinikum Salzburg, Müllner Hauptstr. 48, 5020, Salzburg, Österreich.
authors: A
authors: A
authors: Diem
authors: Universitätsklinik für Dermatologie, Universitätsklinikum Salzburg, Müllner Hauptstr. 48, 5020, Salzburg, Österreich.
authors: J W
authors: JW
authors: Bauer
authors: Universitätsklinik für Dermatologie, Universitätsklinikum Salzburg, Müllner Hauptstr. 48, 5020, Salzburg, Österreich.
authors: M
authors: M
authors: Laimer
mesh_terms: D003937:Diagnosis, Differential; D004820:Epidermolysis Bullosa / Q000175:diagnosis* / Q000188:drug therapy*; D019317:Evidence-Based Medicine; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009092:Mucous Membrane / Q000187:drug effects / Q000473:pathology*; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D007155:Immunologic Factors; D007166:Immunosuppressive Agents
keywords: Genodermatoses; Multisystemic disease; Prevention; Quality of life; Wound care