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Acute Laryngeal Obstruction in Junctional Epidermolysis Bullosa
Author(s) -
Davies Hugh,
Atherton David J.
Publication year - 1987
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1987.tb00760.x
Subject(s) - medicine , junctional epidermolysis bullosa (veterinary medicine) , epidermolysis bullosa , granulation tissue , airway obstruction , larynx , pathology , dermatology , anatomy , airway , surgery , wound healing , biochemistry , chemistry , mutation , gene
A boy with junctional epidermolysis bullosa died from acute laryngeal obstruction at the age of 29 months, having been hoarse since early infancy. Post mortem studies showed gross narrowing of the laryngeal airway by cystic dilatations of the ducts of the seromucinous glands, and replacement of the laryngeal epithelium by oedematous granulation tissue. Laryngeal involvement appears to occur not infrequently in junctional epidermolysis bullosa, and contributes to its considerable mortality.