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Lipoid Proteinosis of Urbach and Wiethe

Known as: lipoidosis cutis et mucosae, Urbach-Wiethe Disease, URBACH-WIETHE SYNDROME 
An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is… 
National Institutes of Health

Related topics

Related topics

19 relations
Aggressive behaviorAutosomal recessive inheritanceCDISC SEND Non-Neoplastic Finding Type TerminologyClinical Data Interchange Standards Consortium Terminology

Papers overview

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2015
2015
Quantitative Proteogenomics and the Reconstruction of the Metabolic Pathway in Lactobacillus mucosae LM1
Lactobacillus mucosae is a natural resident of the gastrointestinal tract of humans and animals and a potential probiotic… 
2010
2010
Otolaryngological manifestations and genetic characteristics of lipoid proteinosis.
OBJECTIVES We investigated the otolaryngological manifestations and genetic characteristics of lipoid proteinosis (LP). METHODS… 
2005
2005
A novel mutation of the extracellular matrix protein 1 gene (ECM1) in a patient with lipoid proteinosis (Urbach–Wiethe disease) from Sicily
Background  Lipoid proteinosis (LP), also known as Urbach–Wiethe disease, is a rare autosomal recessive disorder characterized by… 
Highly Cited
1993
Highly Cited
1993
Accumulation of surfactant protein D in human pulmonary alveolar proteinosis.
Surfactant protein D (SP-D) is a collagenous calcium-dependent carbohydrate-binding protein that is structurally related to the… 
1987
1987
Advanced Dermatologic Therapy
Thoughts on Our Way to the Clinic Abscess Acanthosis Nigricans Acne Fulminans Acne Keloidalis Acne Vulgaris Acrodermatitis… 
Highly Cited
1982
Highly Cited
1982
Experimental silicosis. II. Long-term effects of intratracheally instilled quartz on collagen metabolism and morphologic characteristics of rat lungs.
Rats received intratracheal instillations of 50 mg of silica (quartz, 0.5 mu particles). One, 2, 4, 5, 6, 9, and 12 months later… 
Highly Cited
1978
Highly Cited
1978
Alveolar macrophages in rabbits exposed to nickel dust. Ultrastructural changes and effect on phagocytosis.
1963
1963
Lipoid Proteinosis (of Urbach-Wiethe)
Introduction Lipoid proteinosis is a rare disease of skin and mucous membranes. Characteristic lesions of the eyelid occur, but… 
Review
1961
Review
1961
Case of Lipoid Proteinosis
Lipoid proteinosis (Urbach-Wiethe disease; hyalinosis cutis et mucosae) is a rare and striking disorder in which there is a… 
1939
1939
XANTHOMATOSIS: IV. LIPOID PROTEINOSIS (PHOSPHATIDE LIPOIDOSIS)
There are various types of xanthoma which may result in lesions involving the mucous membranes of the mouth, throat, larynx and… 
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