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J Eur Acad Dermatol Venereol ; 35(12): 2349-2360, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34545960

RESUMO

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.


Assuntos
Epidermólise Bolhosa , Vesícula , Consenso , Epidermólise Bolhosa/diagnóstico , Epidermólise Bolhosa/terapia , Humanos , Doenças Raras/diagnóstico , Doenças Raras/terapia , Pele
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