Ear, Nose and Throat Examination in Epidermolysis Bullosa and Procedural Approach

Ismail Cem Temel¹, Asli Bilgic² and Dedee Frances Murrell*^2,3

Received:October 4, 2018;   Published: October 10, 2018

DOI: 10.26717/BJSTR.2018.09.001866

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Abstract

Epidermolysis Bullosa is a spectrum of genetic diseases containing more than 25 subtypes of diseases. The most important characteristic of this disease group is the predisposition towards mechanical fragility which results in blistering of skin and mucous membranes. Mucosal involvement of the ear, nose, and throat areas can cause significant morbidity, especially in junctional and recessive dystrophic EB subtypes. ENT surgeons are frequently involved in the management of EB patients. Thus, in this article, we summarize the important points for ENT surgeons in the management, examination and procedural approach for EB patients.

Keywords : Epidermolysis Bullosa, Ear, Nose and Throat, Mucosal Involvement, Management, Surgical Procedures

Abbreviations : EB: Epidermolysis Bullosa; EBS: Epidermolysis Bullosa simplex; JEB: Junctional Epidermolysis Bullosa; DEB: Dystrophic Epidermolysis Bullosa; ENT: Ear, nose, and throat

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