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Descriptor English: Lipoid Proteinosis of Urbach and Wiethe
Descriptor Spanish: Proteinosis Lipoidea de Urbach y Wiethe
Descriptor Portuguese: Proteinose Lipoide de Urbach e Wiethe
Descriptor French: Protéinose lipoïde
Entry term(s): Hyalinosis Cutis et Mucosae
Lipoid Proteinosis, Urbach-Wiethe
Lipoidproteinosis
Lipoproteinosis
Urbach Wiethe Disease
Urbach Wiethe Lipoid Proteinosis
Urbach-Wiethe Disease
Urbach-Wiethe Lipoid Proteinosis
Urbach-Wiethe Syndrome
Tree number(s): C08.618.490.500
C16.320.850.595
RDF Unique Identifier: https://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.
Allowable Qualifiers: BL blood
CF cerebrospinal fluid
CI chemically induced
CL classification
CO complications
DG diagnostic imaging
DH diet therapy
DI diagnosis
DT drug therapy
EC economics
EH ethnology
EM embryology
EN enzymology
EP epidemiology
ET etiology
GE genetics
HI history
IM immunology
ME metabolism
MI microbiology
MO mortality
NU nursing
PA pathology
PC prevention & control
PP physiopathology
PS parasitology
PX psychology
RH rehabilitation
RT radiotherapy
SU surgery
TH therapy
UR urine
VE veterinary
VI virology
Public MeSH Note: 2007; see LIPOIDPROTEINOSIS 1991-2006, see LIPOIDOSIS 1975-1990
History Note: 2007 (1975)
Entry Version: LIPOID PROTEINOSIS URBACH AND WIETHE
Related: Hyalinosis, Systemic MeSH
DeCS ID: 8240
Unique ID: D008065
Documents indexed in the Virtual Health Library (VHL): Click here to access the VHL documents
Date Established: 1991/01/01
Date of Entry: 1999/01/01
Revision Date: 2013/07/09
Lipoid Proteinosis of Urbach and Wiethe - Preferred
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.
Preferred term Lipoid Proteinosis of Urbach and Wiethe
Entry term(s) Hyalinosis Cutis et Mucosae
Lipoid Proteinosis, Urbach-Wiethe
Lipoidproteinosis
Lipoproteinosis
Urbach Wiethe Disease
Urbach Wiethe Lipoid Proteinosis
Urbach-Wiethe Disease
Urbach-Wiethe Lipoid Proteinosis
Urbach-Wiethe Syndrome



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