Document Detail

Lipoid proteinosis (Urbach-Wiethe disease): a case report from India.
MedLine Citation:
PMID:  18800328     Owner:  NLM     Status:  MEDLINE    
Lipoid proteinosis (Urbach-Wiethe disease) is a rare autosomal-recessive anomaly that primarily affects the skin and the mucosa of the upper aerodigestive tract in children. It is caused by hyaline deposits in tissues. Hoarseness secondary to laryngeal involvement is frequently the first presenting feature. It is important to consider this disease in the differential diagnosis of hoarseness because it might lead to life-threatening airway compromise. We report a case of lipoid proteinosis in a 12-year-old girl who presented with hoarseness and skin lesions.
Kadambari Batra; Anil Safaya; Kiran Aggarwal
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Ear, nose, & throat journal     Volume:  87     ISSN:  1942-7522     ISO Abbreviation:  Ear Nose Throat J     Publication Date:  2008 Sep 
Date Detail:
Created Date:  2008-09-18     Completed Date:  2008-12-30     Revised Date:  2009-05-20    
Medline Journal Info:
Nlm Unique ID:  7701817     Medline TA:  Ear Nose Throat J     Country:  United States    
Other Details:
Languages:  eng     Pagination:  531-2     Citation Subset:  IM    
Department of Ear Nose and Throat-Head and Neck Surgery, Vardhman Mahaveer Medical College and Safdarjung Hospital, New Delhi, India.
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MeSH Terms
Biopsy, Needle
Fiber Optic Technology
Follow-Up Studies
Hoarseness / diagnosis,  etiology
Laryngeal Diseases / diagnosis*,  surgery*
Laryngoscopy / methods
Lipoid Proteinosis of Urbach and Wiethe / diagnosis*,  surgery*
Mouth Mucosa / pathology
Risk Assessment
Severity of Illness Index
Treatment Outcome

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