MCID: SCL025
MIFTS: 31

Scleromyxedema malady

Rare diseases, Skin diseases categories

Aliases & Classifications for Scleromyxedema

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Sources:
45NIH Rare Diseases, 51Orphanet, 65UMLS, 28ICD10 via Orphanet, 37MESH via Orphanet, 66UMLS via Orphanet
See all sources

Aliases & Descriptions for Scleromyxedema:

Name: Scleromyxedema 45 51 65
Generalized Lichenoid Papular Eruption 45 51
Arndt-Gottron Disease 45 51
 
Generalized Papular and Sclerodermoid Lichen Myxedematosus 51
Generalized Papular and Sclerodermoid 45


Classifications:

Orphanet: 51 
Rare skin diseases


External Ids:

Orphanet51 167635
ICD10 via Orphanet28 L98.5
MESH via Orphanet37 D053718
UMLS via Orphanet66 C0263390

Summaries for Scleromyxedema

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NIH Rare Diseases:45 Scleromyxedema is a rare, severe skin disorder. signs and symptoms include abnormal accumulation of mucin in the skin (mucinosis), causing papular and sclerodermoid bumps; increased production of fibroblasts (connective tissue cells) in the absence of a thyroid disorder; and monoclonal gammopathy (abnormal proteins in the blood). it often involves internal organs and may affect various body systems. the cause of scleromyxedema is not known. management may involve the use of intravenous immunoglobulin (ivig) and/or plasmapheresis, but no standard treatment exists. last updated: 4/18/2014

MalaCards based summary: Scleromyxedema, also known as generalized lichenoid papular eruption, is related to papular mucinosis and myopathy. An important gene associated with Scleromyxedema is ACTC1 (Actin, Alpha, Cardiac Muscle 1), and among its related pathways are Cardiac Progenitor Differentiation and VEGF Signaling. Affiliated tissues include skin, thyroid and lymph node, and related mouse phenotype homeostasis/metabolism.

Related Diseases for Scleromyxedema

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Graphical network of the top 20 diseases related to Scleromyxedema:



Diseases related to scleromyxedema

Symptoms for Scleromyxedema

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Drugs & Therapeutics for Scleromyxedema

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Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Scleromyxedema

Genetic Tests for Scleromyxedema

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Anatomical Context for Scleromyxedema

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MalaCards organs/tissues related to Scleromyxedema:

33
Skin, Thyroid, Lymph node

Animal Models for Scleromyxedema or affiliated genes

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MGI Mouse Phenotypes related to Scleromyxedema:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053768.0ACTC1, F13A1, FGF2, PIK3C2A

Publications for Scleromyxedema

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Articles related to Scleromyxedema:

(show top 50)    (show all 159)
idTitleAuthorsYear
1
Scleromyxedema (papular mucinosis) with dermato-neuro syndrome: A rare, potentially fatal complication. (26051450)
2015
2
Scleromyxedema, a therapeutic dilemma. (25814746)
2015
3
Scleromyxedema. (25366027)
2014
4
Periocular Scleromyxedema. (24807804)
2014
5
Scleromyxedema with histology resembling granuloma annulare. (25780962)
2014
6
Specific lymph node involvement in scleromyxedema: a new diagnostic entity for hypermetabolic lymphadenopathy. (23681113)
2013
7
Combination of intravenous immunoglobulins and lenalidomide in the treatment of scleromyxedema. (23866873)
2013
8
Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis? (21671910)
2012
9
Scleromyxedema: a cutaneous paraneoplastic syndrome associated with thymic carcinoma. (22162593)
2012
10
Scleromyxedema vs scleredema: a diagnostic challenge. (21771713)
2011
11
Cardiomyopathy in scleromyxedema: report of a fatal case. (20977988)
2010
12
Scleromyxedema with an interstitial granulomatous-like pattern: a rare histologic variant mimicking granuloma annulare. (19638067)
2010
13
Treatment of scleromyxedema and the dermatoneuro syndrome with intravenous immunoglobulin. (19249127)
2009
14
Scleromyxedema with myopathy was successfully treated by thalidomide. (18435730)
2009
15
Suspected cardiac toxicity to intravenous immunoglobulin used for treatment of scleromyxedema. (18583794)
2008
16
Unusual granulomatous variant of scleromyxedema. (18485525)
2008
17
Scleroderma-like acute renal crisis in a patient with scleromyxedema. (17389621)
2007
18
Steady remission of scleromyxedema 3 years after autologous stem cell transplantation: an in vivo and in vitro study. (16822907)
2006
19
Can scleromyxedema be differentiated from nephrogenic fibrosing dermatopathy by the distribution of the infiltrate? (17083697)
2006
20
Tumorous variant of scleromyxedema. Successful therapy with intravenous immunoglobulins. (15987294)
2005
21
Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy. (16394362)
2005
22
Thalidomide as a potential treatment for scleromyxedema. (15023770)
2004
23
Treatment of recalcitrant scleromyxedema with thalidomide in 3 patients. (15243538)
2004
24
Scleromyxedema in a patient with multiple sclerosis and monoclonal gammopathy on interferon beta-1a. (14760958)
2004
25
A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. (15280830)
2004
26
Successful treatment of intractable scleromyxedema with cyclosporin A. (14657639)
2003
27
Heparan sulphate proteoglycan in scleromyxedema promotes FGF-2 activity. (12498227)
2002
28
Scleromyxedema-like fibromucinosis in a patient undergoing hemodialysis. (12100695)
2002
29
The neurologic complications of scleromyxedema. (11552085)
2001
30
Scleromyxedema: possible association with seminoma. (10767692)
2000
31
Scleromyxedema. (10426928)
1999
32
Fever, convulsions and coma in scleromyxedema: a "dermato-neuro syndrome". (8649597)
1996
33
Scleromyxedema. (7601944)
1995
34
Extracorporeal photochemotherapy: a potentially useful treatment for scleromyxedema. (1791229)
1991
35
Scleromyxedema: case for therapeutic suggestion. (2340934)
1990
36
Stimulation of fibroblasts by paraprotein in scleromyxedema. (2808811)
1989
37
Scleromyxedema associated with non-Hodgkin lymphoma. (2592130)
1989
38
Scleromyxedema: therapeutic response to isotretinoin in three patients. (3170809)
1988
39
Plasmapheresis in a patient with scleromyxedema. (3107482)
1987
40
Scleromyxedema with systemic involvement mimics rheumatic diseases. (2943292)
1986
41
Scleromyxedema and inflammatory myopathy: a clinicopathologic study of three patients. (3465308)
1986
42
Treatment of scleromyxedema with etretinate. (6715602)
1984
43
Major surgery and scleromyxedema. (6230537)
1984
44
Scleromyxedema associated with IgG lambda multiple myeloma. (6416712)
1983
45
Scleromyxedema - a sclerodermoid disorder. (7288775)
1981
46
Pathology quiz case 3. Lichen myxedematosus (scleromyxedema). (7259228)
1981
47
Scleromyxedema --report of a case with hyperpolygammopathy and immunoglobulin and complement deposition in cutaneous lesions--. (6997351)
1980
48
Scleromyxedema myopathy. Histochemical and electron microscopic observations. (645644)
1978
49
Scleromyxedema. (813582)
1976
50
Scleromyxedema. (5761799)
1969

Variations for Scleromyxedema

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Expression for genes affiliated with Scleromyxedema

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Search GEO for disease gene expression data for Scleromyxedema.

Pathways for genes affiliated with Scleromyxedema

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Pathways related to Scleromyxedema according to GeneCards Suite gene sharing:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.1ACTC1, FGF2
2
Show member pathways
9.1FGF2, PIK3C2A
39.1FGF2, PIK3C2A

GO Terms for genes affiliated with Scleromyxedema

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Cellular components related to Scleromyxedema according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1blood microparticleGO:00725629.3ACTC1, F13A1

Biological processes related to Scleromyxedema according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1phosphatidylinositol biosynthetic processGO:00066619.5FGF2, PIK3C2A
2positive regulation of gene expressionGO:00106289.3ACTC1, FGF2
3platelet activationGO:00301689.3F13A1, PIK3C2A
4wound healingGO:00420609.2F13A1, FGF2

Sources for Scleromyxedema

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2CDC
14ExPASy
15FDA
16FMA
24GTR
25HGMD
26HMDB
27ICD10
28ICD10 via Orphanet
29ICD9CM
30IUPHAR
31KEGG
34MedGen
36MeSH
37MESH via Orphanet
38MGI
41NCI
42NCIt
43NDF-RT
46NINDS
47Novoseek
49OMIM
50OMIM via Orphanet
54PubMed
55QIAGEN
60SNOMED-CT via Orphanet
64Tumor Gene Family of Databases
65UMLS
66UMLS via Orphanet