Junctional epidermolysis bullosa of the larynx. Report of a case and literature review.
Junctional epidermolysis bullosa of the larynx. Report of a case and literature review.
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喉交界性大疱性表皮松解症。
DOI：
10.1177/000348949210101010
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发表时间：
1992
期刊：
影响因子：
--
通讯作者：
Carter,DM
中科院分区：
文献类型：
--
作者：
Berson,S;Lin,AN;Ward,RF;Carter,DM
大疱性表皮松解症(EB)是一组罕见的遗传性疾病，轻微创伤会在皮肤和粘膜(包括食道)形成水泡。发病率随疾病类型的不同而不同，从偶尔出现的轻微皮肤水泡到婴儿死亡。据报道，有9名患者喉部受累，表现为声音嘶哑和呼吸困难，其中5名患者患有交界性EB。我们报告第六例伴有喉部受累的交界性EB病例，并为耳鼻喉科医生和麻醉科医生护理这些脆弱的患者提供指南。
Epidermolysis bullosa (EB) is a group of rare inherited disorders in which minor trauma causes blister formation in the skin and mucosa, including the esophagus. Morbidity varies with the type of disease and ranges from occasional trivial skin blisters to death in infancy. Laryngeal involvement presenting as hoarseness and respiratory distress has been reported in nine patients, five of whom had junctional EB. We present the sixth case of junctional EB with laryngeal involvement, and offer guidelines for otolaryngologists and anesthesiologists caring for these fragile patients.