Laryngeal lipoid proteinosis (urbach-wiethe's disease): a case report
نویسندگان
چکیده
منابع مشابه
[Lipoid proteinosis (Urbach-Wiethe's Disease)].
A 25-year-old female patient was admitted to our outpatient clinic with postpartum hoarseness. Punch biopsy specimens obtained from the larynx and sublingual region revealed multi-folded squamous epithelium with a hyperkeratosis pattern and amorphous hyaline material aggregation. This aggregation was also remarkable around the vessels. Histochemical examination showed periodic acid-schiff-posit...
متن کاملLipoid proteinosis (Urbach-Wiethe disease).
The aim of this study has been to assess the clinical presentation and biochemical profile of lipoid proteinosis within a defined pedigree. Glycoprotein analysis was compared to normal values in an attempt to define a biochemical phenotype. Six affected family members were identified with variable degrees of disease expression. The most likely mode of inheritance is autosomal recessive due to c...
متن کاملlipoid proteinosis: a case report urbach & wiethe disease
lipoid proteinosis (lp) is a rare autosomal recessive disease characterized by the deposition of an amorphous hyaline material in the skin, mucosa and viscera. the classic manifestation is onset in infancy with a hoarse cry due to laryngeal infiltration. skin and mucous changes develope, and the disease follows a slowly progressive course. in this case report, a 49 year-old man presented with a...
متن کاملLipoid proteinosis (Urbach-Wiethe syndrome).
A Jewish-Iranian family suffered from lipoid proteinosis. The 8 affected siblings were from consanguineous matings and presented a wide range of phenotypic expressions. Minimal manifestations in 2 heterozygote carriers and the possibility of autosomal recessive inheritance are discussed.
متن کاملLipoid Proteinosis in a Young Female: A Case Report
Lipoid proteinosis or Urbach-Wiethe disease is a rare autosomal recessive disorder. It is characterized by progressive deposition of hyaline substance in the mucous membranes, skin and internal organs. It is characterized by papular and nodular lesions on the face, elbows, knees and hands. The tongue is firm and hoarseness may be present at birth. We aim to describe one case of this disease in ...
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ژورنال
عنوان ژورنال: Turkish Journal of Pathology
سال: 2009
ISSN: 1018-5615,1309-5730
DOI: 10.5146/tjpath.2009.00960