Urbach-Wiethe disease (lipoid proteinosis)
Lipoid proteinosis is a rare autosomal recessive disorder of variable severity that may involve the skin, mucous membranes of the upper respiratory tract and internal organs that may display different clinical manifestations based on the site of involvement. A 34-year-old male patient was admitted t...
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Wolters Kluwer Medknow Publications
2012-01-01
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Series: | Indian Journal of Pathology and Microbiology |
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Online Access: | http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2012;volume=55;issue=3;spage=375;epage=376;aulast=Kucuk |
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doaj-739f6c3824ef4b23b1cad5788af335f52020-11-24T21:19:50ZengWolters Kluwer Medknow PublicationsIndian Journal of Pathology and Microbiology0377-49292012-01-0155337537610.4103/0377-4929.101749Urbach-Wiethe disease (lipoid proteinosis)Ulku KucukIsin G ErdoganUmit BayolNazife HaciogluIbrahim CukurovaCengiz BicakciLipoid proteinosis is a rare autosomal recessive disorder of variable severity that may involve the skin, mucous membranes of the upper respiratory tract and internal organs that may display different clinical manifestations based on the site of involvement. A 34-year-old male patient was admitted to our hospital with a complaint of hoarseness present since childhood but had worsened in recent years. A dermatological examination revealed light brown papillomatous skin lesions on the dorsum of both hands and elbows present for a long duration. Endoscopic examination of the larynx revealed widespread mucosal thickening and irregularity. Microscopic examination revealed acid Schiff (PAS) positive, Congo red negative eosinophilic hyaline material deposits in the dermis and submucosal regions. The final diagnosis was lipoid proteinosis with skin and larynx involvement. This typical case has been reported to draw attention to the fact that lipoid proteinosis should be included in the differential diagnosis of patients presenting with hoarseness in childhood.http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2012;volume=55;issue=3;spage=375;epage=376;aulast=KucukLarynxlipoid proteinosisUrbach-Wiethe disease |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ulku Kucuk Isin G Erdogan Umit Bayol Nazife Hacioglu Ibrahim Cukurova Cengiz Bicakci |
spellingShingle |
Ulku Kucuk Isin G Erdogan Umit Bayol Nazife Hacioglu Ibrahim Cukurova Cengiz Bicakci Urbach-Wiethe disease (lipoid proteinosis) Indian Journal of Pathology and Microbiology Larynx lipoid proteinosis Urbach-Wiethe disease |
author_facet |
Ulku Kucuk Isin G Erdogan Umit Bayol Nazife Hacioglu Ibrahim Cukurova Cengiz Bicakci |
author_sort |
Ulku Kucuk |
title |
Urbach-Wiethe disease (lipoid proteinosis) |
title_short |
Urbach-Wiethe disease (lipoid proteinosis) |
title_full |
Urbach-Wiethe disease (lipoid proteinosis) |
title_fullStr |
Urbach-Wiethe disease (lipoid proteinosis) |
title_full_unstemmed |
Urbach-Wiethe disease (lipoid proteinosis) |
title_sort |
urbach-wiethe disease (lipoid proteinosis) |
publisher |
Wolters Kluwer Medknow Publications |
series |
Indian Journal of Pathology and Microbiology |
issn |
0377-4929 |
publishDate |
2012-01-01 |
description |
Lipoid proteinosis is a rare autosomal recessive disorder of variable severity that may involve the skin, mucous membranes of the upper respiratory tract and internal organs that may display different clinical manifestations based on the site of involvement. A 34-year-old male patient was admitted to our hospital with a complaint of hoarseness present since childhood but had worsened in recent years. A dermatological examination revealed light brown papillomatous skin lesions on the dorsum of both hands and elbows present for a long duration. Endoscopic examination of the larynx revealed widespread mucosal thickening and irregularity. Microscopic examination revealed acid Schiff (PAS) positive, Congo red negative eosinophilic hyaline material deposits in the dermis and submucosal regions. The final diagnosis was lipoid proteinosis with skin and larynx involvement. This typical case has been reported to draw attention to the fact that lipoid proteinosis should be included in the differential diagnosis of patients presenting with hoarseness in childhood. |
topic |
Larynx lipoid proteinosis Urbach-Wiethe disease |
url |
http://www.ijpmonline.org/article.asp?issn=0377-4929;year=2012;volume=55;issue=3;spage=375;epage=376;aulast=Kucuk |
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