Hereditary epidermolysis bullosa.
J Dtsch Dermatol Ges
; 13(11): 1125-33, 2015 Nov.
Article
em En
| MEDLINE
| ID: mdl-26513070
ABSTRACT
The term epidermolysis bullosa (EB) includes a group of rare genodermatoses characterized by mutational impairment of the structural and functional integrity of intraepidermal adhesion and dermoepidermal anchorage. Clinically, these disorders are marked by increased skin fragility as well as characteristic mechanically inducible blisters on the skin and mucous membranes. Extracutaneous manifestations and their complications in other epithelialized organs render EB a multi-system disease associated with significant morbidity and mortality. Cornerstones of a dynamically changing healthcare structure include precise and early diagnosis; coordinated, multidisciplinary, individually adjusted patient care at specialized centers; optimized symptomatic therapies; and access to research-based, potentially curative therapeutic strategies.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Pele
/
Marcadores Genéticos
/
Epidermólise Bolhosa
Tipo de estudo:
Diagnostic_studies
/
Screening_studies
Limite:
Humans
Idioma:
En
Revista:
J Dtsch Dermatol Ges
Assunto da revista:
DERMATOLOGIA
Ano de publicação:
2015
Tipo de documento:
Article
País de afiliação:
Áustria