[学龄儿童和青少年的遗传性大疱性表皮松解症。临床表现及多学科管理]

[Hereditary epidermolysis bullosa in school children and adolescents. Clinical picture and interdisciplinary management].

作者信息

Ott H, Eich C, Schriek K, Ludwikowski B

机构信息

Fachbereich Pädiatrische Dermatologie und Allergologie, Kinder- und Jugendkrankenhaus AUF DER BULT, Janusz-Korczak-Allee 12, 30173, Hannover, Deutschland.

Epidermolysis bullosa-Zentrum Hannover, Kinder- und Jugendkrankenhaus AUF DER BULT, Hannover, Deutschland.

出版信息

Hautarzt. 2016 Apr;67(4):279-86. doi: 10.1007/s00105-016-3774-6.

DOI:10.1007/s00105-016-3774-6

PMID:26943360

Abstract

Hereditary epidermolysis bullosa (EB) represents a clinically heterogeneous group of congenital blistering disorders requiring multiprofessional care. EB is associated with a broad spectrum of potentially severe complications often reaching their full extent during school age and adolescence. This review aims at summarizing cutaneous manifestations of EB as well as extracutaneous complications of this complex disease and their interdisciplinary management.

摘要

遗传性大疱性表皮松解症（EB）是一组临床异质性的先天性水疱性疾病，需要多专业护理。EB与一系列潜在的严重并发症相关，这些并发症通常在学龄期和青春期充分显现。本综述旨在总结EB的皮肤表现以及这种复杂疾病的皮肤外并发症及其跨学科管理。