MeSH Browser

MeSH Heading: Lipoid Proteinosis of Urbach and Wiethe
Tree Number(s): C08.618.490.500; C16.320.850.595
Unique ID: D008065
RDF Unique Identifier: http://id.nlm.nih.gov/mesh/D008065
Scope Note: An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN.
Entry Version: LIPOID PROTEINOSIS URBACH AND WIETHE
Entry Term(s): Hyalinosis Cutis et Mucosae; Lipoidproteinosis; Lipoproteinosis; Urbach-Wiethe Disease; Urbach-Wiethe Lipoid Proteinosis; Urbach-Wiethe Syndrome
See Also: Hyaline Fibromatosis Syndrome
Public MeSH Note: 2007; see LIPOIDPROTEINOSIS 1991-2006, see LIPOIDOSIS 1975-1990
History Note: 2007 (1975)
Date Established: 1991/01/01
Date of Entry: 1999/01/01
Revision Date: 2023/07/31

Allowable Qualifiers: blood (BL)

cerebrospinal fluid (CF)

chemically induced (CI)

classification (CL)

complications (CO)

diagnosis (DI)

diagnostic imaging (DG)

diet therapy (DH)

drug therapy (DT)

economics (EC)

embryology (EM)

enzymology (EN)

epidemiology (EP)

ethnology (EH)

etiology (ET)

genetics (GE)

history (HI)

immunology (IM)

metabolism (ME)

microbiology (MI)

mortality (MO)

nursing (NU)

parasitology (PS)

pathology (PA)

physiopathology (PP)

prevention & control (PC)

psychology (PX)

radiotherapy (RT)

rehabilitation (RH)

surgery (SU)

therapy (TH)

urine (UR)

veterinary (VE)

virology (VI)

Concept UI: M0012568
Scope Note: An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN.
Terms: Lipoid Proteinosis of Urbach and Wiethe Preferred Term
Term UI T647772
Date08/03/2005
LexicalTag EPO
ThesaurusID; Hyalinosis Cutis et Mucosae
Term UI T767818
Date03/24/2010
LexicalTag NON
ThesaurusID; Lipoidproteinosis
Term UI T024040
Date01/01/1999
LexicalTag NON
ThesaurusID NLM (1975); Urbach-Wiethe Disease
Term UI T647775
Date08/03/2005
LexicalTag EPO
ThesaurusID NLM (2007); Urbach-Wiethe Lipoid Proteinosis
Term UI T647773
Date08/03/2005
LexicalTag EPO
ThesaurusID NLM (2007); Urbach-Wiethe Syndrome
Term UI T647774
Date08/03/2005
LexicalTag EPO
ThesaurusID NLM (2007); Lipoproteinosis
Term UI T024041
Date07/31/1996
LexicalTag NON
ThesaurusID

Lipoid Proteinosis of Urbach and Wiethe MeSH Descriptor Data 2025