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Lipoid proteinosis of the larynx

Published online by Cambridge University Press:  08 March 2006

Ferhan Öz
Affiliation:
Department of Pathology, Istanbul University Cerrahpaşa Medical Faculty, Istanbul, Turkey.
Nuran Kalekoglu
Affiliation:
Department of Pathology, Istanbul University Cerrahpaşa Medical Faculty, Istanbul, Turkey.
Baris Karakullukçu
Affiliation:
Department of Pathology, Istanbul University Cerrahpaşa Medical Faculty, Istanbul, Turkey.
Özcan Öztürk
Affiliation:
Department of Pathology, Istanbul University Cerrahpaşa Medical Faculty, Istanbul, Turkey.
Büge Öz
Affiliation:
Departments of Otolaryngology, Head and Neck Surgery and Pathology, Istanbul, Turkey.

Abstract

Lipoid proteinosis is a rare disease that presents with hyaline deposits in many tissues. It involves predominantly the skin and upper aerodigestive tract, presenting with small yellowish papules and hoarseness. It may involve the central nervous system and cause intracerebral calcifications. Laryngeal lesions may resemble singer’s nodule or chronic laryngitis. The pathogenesis of the disease is not clear although several studies suggest a defective collagen production and/or lysosomal storage disease. In this article two cases with skin and larynx involvement are reported.

Type
Research Article
Copyright
© Royal Society of Medicine Press Limited 2002

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