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Practical management of epidermolysis bullosa: consensus clinical position statement from the European Reference Network for Rare Skin Diseases
C. Has, M. El Hachem, H. Bučková, P. Fischer, M. Friedová, C. Greco, P. Nevoránková, C. Salavastru, JE. Mellerio, G. Zambruno, C. Bodemer
Jazyk angličtina Země Velká Británie
Typ dokumentu časopisecké články
PubMed
34545960
DOI
10.1111/jdv.17629
Knihovny.cz E-zdroje
- MeSH
- epidermolysis bullosa * diagnóza terapie MeSH
- konsensus MeSH
- kůže MeSH
- lidé MeSH
- puchýř MeSH
- vzácné nemoci diagnóza terapie MeSH
- Check Tag
- lidé MeSH
- Publikační typ
- časopisecké články MeSH
Inherited epidermolysis bullosa (EB) comprises rare disorders that manifest with fragility and blistering of the skin and mucous membranes, with variable clinical severity. Management of EB is challenging due to disease rarity and complexity, the wide range of extracutaneous manifestations and a profound impact on daily life for the patient and family members. Although reference centres providing multidisciplinary care for EB exist in each European country, it is common for healthcare professionals that are not specialized in this rare disorder to treat EB patients. Here, experts of the European Reference Network for Rare and Undiagnosed Skin Diseases (ERN-Skin, https://ern-skin.eu) propose practical recommendations for the diagnosis and management of the commonest clinical issues, skin blisters and wounds, oral manifestations, pain and itch.
Department of Dermatology Children's Hospital University Hospital Brno Brno Czech Republic
NevDent Dental Private Clinic Brno Czech Republic
Pain and Palliative Care Unit Hôpital Necker Enfants Malades Paris France
Service de Dermatologie Hôpital Necker Enfants Malades Paris France
St John's Institute of Dermatology Guy's and St Thomas' NHS Foundation Trust London UK
Citace poskytuje Crossref.org
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- $a Inherited epidermolysis bullosa (EB) comprises rare disorders that manifest with fragility and blistering of the skin and mucous membranes, with variable clinical severity. Management of EB is challenging due to disease rarity and complexity, the wide range of extracutaneous manifestations and a profound impact on daily life for the patient and family members. Although reference centres providing multidisciplinary care for EB exist in each European country, it is common for healthcare professionals that are not specialized in this rare disorder to treat EB patients. Here, experts of the European Reference Network for Rare and Undiagnosed Skin Diseases (ERN-Skin, https://ern-skin.eu) propose practical recommendations for the diagnosis and management of the commonest clinical issues, skin blisters and wounds, oral manifestations, pain and itch.
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