Lipoid proteinosis of the larynx.
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.
Oz
Ferhan
F
Department of Otolaryngology, Head and Neck Surgery, Istanbul University Cerrahpasa Medical Faculty, Turkey.
Kalekoğlu
Nuran
N
Karakullukçu
Baris
B
Oztürk
Ozcan
O
Oz
Büge
B
D000328:Adult; D001706:Biopsy / Q000379:methods; D002648:Child; D005260:Female; D006685:Hoarseness / Q000209:etiology / Q000473:pathology; D006801:Humans; D007818:Laryngeal Diseases / Q000150:complications / Q000473:pathology*; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000150:complications / Q000473:pathology*; D035583:Rare Diseases