Epidermolysis bullosa aquista (EBA) with obliteration of the larynx – a case report

The acquired type of Epidermolysis bullosa with antibodies against collagen VII is a rare subepithelial blistering disease with a prevalence of two affected persons per 10 million people. Current literature reveals only seven cases with laryngeal manifestation.

A 25-year old tracheotomised patient approached our clinic aiming for a laryngeal reconstruction and recovery of aphonia.

We begin by summarising the recent literature concerning the manifestation of EBA in ENT-patients. Distinct mucosal and dermal scars are a typical phenomenon in EBA patients. Stenosis of the larynx is also a common complication but to our knowledge a complete obliteration is not described until now. This report will then examine the previous treatment this patient received.

Finally, we explain the stepwise treatment delivered in this unique case. The first successful surgery enabled the patient to recover the function of his voice. However, due to previous treatment, a bilateral fixation of the cricoarytenoid joints according to a posterior glottis stenosis (PGS) IV disturbed the complete regain of mobility.

This report will highlight the risks of medical treatments such as bougienage when the patient has not recovered completely from the underlying disease.

This case report shows the importance of the etiological clarification of laryngeal pathologies in advance of further surgery. After securing the airway a histological and serological confirmation of the diagnosis is the best therapeutic approach to deal with systemic diseases with laryngeal association.

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Artikel online veröffentlicht:
10. Juni 2020