title: Vocal fold hyalinosis in Urbach-Wiethe disease, a rare cause of hoarseness.
abstract: BACKGROUND
  Lipoid proteinosis is an autosomal recessive disorder characterized by hyalin deposits in the skin and mucosa of the upper aerodigestive tract; currently, no treatment exists. Nearly all patients experience hoarseness and speech difficulties, due to hyalin deposition in the vocal folds and diminished mobility in infiltrated lips, tongue, and palate.
  
  
  METHODS
  We describe a patient with extensive hyalin plaques on the vocal folds, which resulted in near-aphonic hoarseness. Hyalin deposits in the vocal folds and skin were treated with laser resection.
  
  
  RESULTS
  Both the vocal folds and skin improved in appearance, with smoother surface epithelium. However, the patient's speech remained impaired, due to extensive hyalin plaques in the mouth, tongue, and lips. The voice improved only temporarily.
  
  
  CONCLUSIONS
  Laser resection of hyalin plaques in the vocal folds and skin is a feasible treatment for lipoid proteinosis. However, speech may remain severely limited, due to impaired tongue and lip movement.
authors.forename: J
authors.initials: J
authors.lastname: Honings
authors.forename: M M
authors.initials: MM
authors.lastname: van Rossum
authors.forename: F J A
authors.initials: FJ
authors.lastname: van den Hoogen
mesh_terms: D000328:Adult; D006685:Hoarseness / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000150:complications* / Q000473:pathology / Q000601:surgery; D008297:Male; D064706:Vocal Cord Dysfunction / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D014827:Vocal Cords / Q000473:pathology* / Q000601:surgery