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Hereditary epidermolysis bullosa
Author(s) -
Laimer Martin,
Prodinger Christine,
Bauer Johann W.
Publication year - 2015
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/ddg.12774
Subject(s) - epidermolysis bullosa , medicine , dermatology , hemidesmosome , multidisciplinary team , disease , pathology , basement membrane , nursing
Summary 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.