Skip Navigation LinksHome > December 2013 - Volume 21 - Issue 6 > Management of manifestations of epidermolysis bullosa
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Current Opinion in Otolaryngology & Head & Neck Surgery:
doi: 10.1097/MOO.0000000000000004
PEDIATRIC OTOLARYNGOLOGY: Edited by Lisa Buckmiller

Management of manifestations of epidermolysis bullosa

Elluru, Ravindhra G.a; Contreras, Jose M.b; Albert, David M.c

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Abstract

Purpose of review

Children with epidermolysis bullosa can present with disease(s) of the ears, nose, and throat, often related directly to the pathophysiology of their epidermolysis bullosa. Otolaryngologic diseases in children with epidermolysis bullosa have to be managed having a proper understanding of the diagnosis and pathophysiology of epidermolysis bullosa. The purpose of this review is to describe the current nomenclature and diagnostic algorithms for epidermolysis bullosa, and methods for the management of cutaneous and mucosal lesions.

Recent findings

Characterization of the gene defects leading to epidermolysis bullosa has allowed the utilization of immunofluorescent techniques as the primary method for epidermolysis bullosa diagnosis. Recognizing the difficulty in managing patients with epidermolysis bullosa, several multidisciplinary groups have developed guidelines using meta-analysis of the published literature, or expert panels. Though there are currently no effective treatment modalities for epidermolysis bullosa, techniques for gene and protein replacement show promising results for future use.

Summary

Currently, the management of cutaneous and mucosal disease in epidermolysis bullosa is based on the principles of prevention and wound care. Understanding the cause of epidermolysis bullosa types and subtypes, characteristics of skin and mucosal involvement, and prognosis will guide in the development of individualized treatment plans.

© 2013 Wolters Kluwer Health | Lippincott Williams & Wilkins

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