Lipoid proteinosis of larynx: Review of four cases

Acar A., Eryilmaz A., GÖÇER C. , Akmansu H., Korkmaz H.

International Journal of Pediatric Otorhinolaryngology, vol.68, no.12, pp.1557-1561, 2004 (Journal Indexed in SCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 68 Issue: 12
  • Publication Date: 2004
  • Doi Number: 10.1016/j.ijporl.2004.07.012
  • Title of Journal : International Journal of Pediatric Otorhinolaryngology
  • Page Numbers: pp.1557-1561
  • Keywords: lipoid proteinosis, larynx, skin, LESIONS


Lipoid proteinosis is a rare autosomal recessive disorder characterized by intercellular deposition of an amorphous hyaline material. It mainly involves skin and mucosal membranes of upper aerodigestive tract as well as central nervous system, lung, lymph nodes and striated muscles. Etiology and pathogenesis are unknown. Infantile hoarseness is a common presenting feature of the disease due to infiltration of larynx. In two-thirds of the cases, voice changes are present at birth or in early infancy as the first manifestation. We present four patients with lipoid proteinosis involving skin, oropharynx and larynx. © 2004 Elsevier Ireland Ltd. All rights reserved.