Supraglottic laryngeal manifestation of epidermolysis bullosa in a pediatric population: A literature review with four case reports

Abstract

Epidermolysis bullosa (EB) is a group of inherited disorders characterized by increased vulnerability to mechanical stress at the dermo-epidermal junction and basilar layer of the epidermis. Laryngeal EB involvement is a major and life-threatening manifestation of the disease. Due to the nature of EB, granulation tissue and stenosis can occur, usually at the glottic, subglottic, or supraglottic levels, as seen in our patients.We described cases in which four patients with EB presented with supraglottic stenosis and were managed at tertiary hospital in Saudi Arabia. The clinical and therapeutic characteristics of all patients are discussed.

Keywords

• Epidermolysis bullosa
• Supraglottic stenosis
• Laryngeal manifestation