Junctional Epidermolysis Bullosa Associated Laryngeal Stenosis: A Case Report and Review of Literature.

Firyal Balushi, Ziyad Al Harrasi, Maathir Al Farsi, Marwa Al Qurani
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引用次数: 0

Abstract

Introduction: Introduction: Junctional Epidermolysis Bullosa (JEB) is a rare subtype of the Epidermolysis Bullosa which itself is a rare genetic disorder. While mucosal involvement of pharynx and oesophagus has been reported, laryngeal involvement is rare.

Case report: A 7-month-old male child who was known to have Junctional Epidermolysis Bullosa presented to the emergency department with respiratory distress associated with a stridor which was eventfully found to have multiple level laryngeal stenosis.

Conclusions: Longitudinal cohort studies are required to determine the long-term outcome and the anticipated behavior of epidermolysis bullosa in patients with laryngeal manifestation to avoid unnecessary surgical interventions.

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交界性表皮松解症伴有喉狭窄:病例报告和文献综述。
导言简介:简介:交界性表皮松解症(JEB)是表皮松解症的一种罕见亚型,而表皮松解症本身是一种罕见的遗传性疾病。虽然咽部和食道粘膜受累已有报道,但喉部受累却很少见:病例报告:一名已知患有交界性表皮松解症的 7 个月大男婴因呼吸困难并伴有喘鸣而到急诊科就诊,最终发现他患有多层次喉狭窄:需要进行纵向队列研究,以确定有喉部表现的表皮松解症患者的长期预后和预期行为,从而避免不必要的手术干预。
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来源期刊
Iranian Journal of Otorhinolaryngology
Iranian Journal of Otorhinolaryngology Medicine-Otorhinolaryngology
CiteScore
1.30
自引率
0.00%
发文量
72
审稿时长
12 weeks
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