E-ISSN: 2667-7474
Otolaryngologic findings of five lipoid proteinosis cases
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Case Report
VOLUME: 44 ISSUE: 1
P: 44-47
March 2006

Otolaryngologic findings of five lipoid proteinosis cases

Turk Arch Otorhinolaryngol 2006;44(1):44-47
Altan Yıldırım 1
Bülent Satar 2
Mustafa Gerek 2
1. Department of Otolaryngology & Head and Neck Surgery, Faculty of Medicine, Cumhuriyet University, Sivas
2. Department of Otolaryngology & Head and Neck Surgery, Gülhane Military Academy of Medicine, Ankara
No information available.
No information available
Accepted Date: 27.10.2005
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ABSTRACT

Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomally recessive inherited disease, which presents with various symptoms due to deposits of hyaline material in tissues. Lesions of larynx and skin are frequent. Otolaryngologic findings of 5 cases of lipoid proteinosis were analyzed, and literature was reviewed. We observed involvement of larynx in all cases with skin lesions in our series. The disease was determined in oral cavities of 3 cases, and tongue base of a case.

ÖZET

Lipoid proteinozis (Urbach-Wiethe hastalığı) ender görülen ve oto-zomal resesif geçiş gösteren herediter bir hastalıktır. Hiyalin maddesinin dokularda birikmesi sonucu çok çeşitli semptomlar oluşabilir. Larinks ve cilt lezyonları sık görülür. Beş lipoid proteinozis olgusunun kulak burun boğaz bulguları incelendi ve literatür gözden geçirildi. Bizim serimizde larinks tutulumu bütün olgularda izlendi. Üç olguda oral bölgede, bir olguda dil kökünde hastalık saptandı.

Keywords:
Lipoid proteinosis, Urbach-Wiethe disease, otolaryngology