title: Diagnosis and management of laryngeal obstruction in childhood pachyonychia congenita.
abstract: Pachyonychia congenita is a genetic syndrome of epithelial dysplasia. In infants and young children, laryngeal involvement can present a life threatening complication: obstruction of the patient's airway due to leukokeratosis can lead to severe respiratory distress. This report concentrates on the diagnosis and successful microsurgical management of laryngeal obstruction in the first female case, a 19-month-old girl with pachyonychia congenita.
authors.affiliation: Department of Pediatrics, University of Ulm, Donau, FRG.
authors.forename: S A
authors.initials: SA
authors.lastname: Wudy
authors.forename: H
authors.initials: H
authors.lastname: Lenders
authors.forename: W
authors.initials: W
authors.lastname: Pirsig
authors.forename: W
authors.initials: W
authors.lastname: Mohr
authors.forename: W M
authors.initials: WM
authors.lastname: Teller
mesh_terms: D000402:Airway Obstruction / Q000150:complications / Q000503:physiopathology; D004848:Epithelium / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D007642:Keratosis / Q000150:complications / Q000503:physiopathology; D007818:Laryngeal Diseases / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D007828:Laryngoscopy; D007830:Larynx / Q000503:physiopathology* / Q000601:surgery / Q000648:ultrastructure; D009260:Nail Diseases / Q000150:complications / Q000503:physiopathology; D014832:Voice Disorders / Q000150:complications