Title:
Vocal fold hyalinosis in Urbach-Wiethe disease, a rare cause of hoarseness
Authors:
J. Honings , M. M. van Rossum and F. J. A. van den Hoogen 1
Institutions:
Departments of Oto-Rhino-Laryngology and Head & Neck Surgery and Dermatolgy, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands
Keywords:
Lipoid proteinosis; laryngeal; hyalinosis; laser; hoarseness; Urbach-Wiethe
Pages:
151 - 155
Abstract:
Vocal fold hyalinosis in Urbach-Wiethe disease, a rare cause of hoarseness. 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 near­aphonic  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 patients 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.
Issue:
Vol. 11, 2015, 2nd trimester


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Vocal fold hyalinosis in Urbach-Wiethe disease, a rare cause of hoarseness