MCID: SCL025
MIFTS: 30

Scleromyxedema malady

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Scleromyxedema

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Sources:
30ICD10 via Orphanet, 39MESH via Orphanet, 47NIH Rare Diseases, 53Orphanet, 67UMLS, 68UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Scleromyxedema:

Name: Scleromyxedema 47 53 67
Generalized Lichenoid Papular Eruption 47 53
Arndt-Gottron Disease 47 53
 
Generalized Papular and Sclerodermoid Lichen Myxedematosus 53
Generalized Papular and Sclerodermoid 47

Characteristics:

Orphanet epidemiological data:

53
scleromyxedema:
Age of onset: Adult

Classifications:

Orphanet: 53 
Rare skin diseases


External Ids:

Orphanet53 ORPHA167635
ICD10 via Orphanet30 L98.5
MESH via Orphanet39 D053718
UMLS via Orphanet68 C0263390

Summaries for Scleromyxedema

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Wikipedia:70 Papular mucinosis (also known as \"Scleromyxedema,\" \"Generalized lichen myxedematosus,\" and... more...

MalaCards based summary: Scleromyxedema, also known as generalized lichenoid papular eruption, is related to scleromyxedema without monoclonal gammopathy and papular mucinosis. An important gene associated with Scleromyxedema is ACTC1 (Actin, Alpha, Cardiac Muscle 1), and among its related pathways are Cardiac Progenitor Differentiation and Integrins in angiogenesis. Affiliated tissues include skin and lymph node, and related mouse phenotype cardiovascular system.

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:

35
Skin, Lymph node

Animal Models for Scleromyxedema or affiliated genes

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MGI Mouse Phenotypes related to Scleromyxedema:

40
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053858.0ACTC1, F13A1, FGF2, PIK3C2A

Publications for Scleromyxedema

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Articles related to Scleromyxedema:

(show top 50)    (show all 173)
idTitleAuthorsYear
1
Successful treatment of scleromyxedema with melphalan and dexamethasone followed by thalidomide maintenance therapy. (27118492)
2016
2
Neurological Involvement in the Course of Scleromyxedema: A Case Report. (27282303)
2016
3
Scleromyxedema Clinically Resembling to Scleroderma. (26951784)
2016
4
Scleromyxedema, a therapeutic dilemma. (25814746)
2015
5
Dermato-neuro syndrome in a case of scleromyxedema. (27708955)
2015
6
Scleromyxedema. (25366027)
2014
7
Scleromyxedema with histology resembling granuloma annulare. (25780962)
2014
8
Scleromyxedema. (25215418)
2014
9
Dermato-Neuro Syndrome in a Patient Treated With Autologous Stem Cell Transplant for Scleromyxedema. (25065780)
2014
10
A case of atypical scleromyxedema without gammopathy treated with cyclosporine. (24823421)
2014
11
Atypical scleromyxedema with prominent nodular lesions associated with immune thrombocytopenia: an unusual presentation. (25219747)
2014
12
Specific lymph node involvement in scleromyxedema: a new diagnostic entity for hypermetabolic lymphadenopathy. (23681113)
2013
13
Combination of intravenous immunoglobulins and lenalidomide in the treatment of scleromyxedema. (23866873)
2013
14
Granulomatous Scleromyxedema: Case Report and Literature Review. (24247576)
2013
15
Scleromyxedema with monoclonal gammopathy and neurological involvement: recovery from coma after plasmapheresis? (21671910)
2012
16
The combination of bortezomib and dexamethasone is an efficient therapy for relapsed/refractory scleromyxedema: a rare disease with new clinical insights. (22404151)
2012
17
Intravenous gammaglobulin and thalidomide may be an effective therapeutic combination in refractory scleromyxedema: case report and discussion of the literature. (18221985)
2008
18
Scleromyxedema diagnosis following unexplained encephalopathy. (18086595)
2008
19
Scleroderma-like acute renal crisis in a patient with scleromyxedema. (17389621)
2007
20
Scleromyxedema: successful treatment with thalidomide in two patients. (17191049)
2007
21
Can scleromyxedema be differentiated from nephrogenic fibrosing dermatopathy by the distribution of the infiltrate? (17083697)
2006
22
Successful treatment of scleromyxedema with dexamethasone cyclophosphamide pulse therapy. (16394362)
2005
23
High-dose dexamethasone in scleromyxedema: report of 2 additional cases. (16198811)
2005
24
Successful treatment of scleromyxedema with autologous peripheral blood stem cell transplantation. (16230566)
2005
25
Histopathologic comparison of nephrogenic fibrosing dermopathy and scleromyxedema. (16008692)
2005
26
A complete and durable clinical response to high-dose dexamethasone in a patient with scleromyxedema. (15280830)
2004
27
Scleromyxedema with subclinical myositis. (17642556)
2004
28
Successful treatment of intractable scleromyxedema with cyclosporin A. (14657639)
2003
29
Heparan sulphate proteoglycan in scleromyxedema promotes FGF-2 activity. (12498227)
2002
30
Scleromyxedema-like fibromucinosis in a patient undergoing hemodialysis. (12100695)
2002
31
The neurologic complications of scleromyxedema. (11552085)
2001
32
Acute rhabdomyolysis during the treatment of scleromyxedema with interferon alfa. (12243709)
2001
33
Scleromyxedema. (7601944)
1995
34
Ophthalmic findings in scleromyxedema. (8115146)
1994
35
Scleromyxedema associated with arthritis and myopathy: a case report. (8275591)
1993
36
Extracorporeal photochemotherapy: a potentially useful treatment for scleromyxedema. (1791229)
1991
37
Cutaneous focal mucinosis associated with reticular erythematous mucinosis and scleromyxedema. (2033150)
1991
38
Myeloma associated scleromyxedema with extensive involvement of small bowel. (1838147)
1991
39
Scleromyxedema: case for therapeutic suggestion. (2340934)
1990
40
Successful treatment of scleromyxedema with plasmapheresis and immunosuppression. (2347969)
1990
41
Stimulation of fibroblasts by paraprotein in scleromyxedema. (2808811)
1989
42
Scleromyxedema serum increases proliferation but not the glycosaminoglycan synthesis of dermal fibroblasts. (2778769)
1989
43
Iatrogenic myelomonocytic leukemia following melphalan treatment of scleromyxedema. (3470169)
1987
44
Scleromyxedema with systemic involvement mimics rheumatic diseases. (2943292)
1986
45
Treatment of scleromyxedema with etretinate. (6715602)
1984
46
Pathology quiz case 3. Lichen myxedematosus (scleromyxedema). (7259228)
1981
47
Treatment of scleromyxedema with melphalan. (434843)
1979
48
Scleromyxedema myopathy. Histochemical and electron microscopic observations. (645644)
1978
49
Scleromyxedema. (5761799)
1969
50
Scleromyxedema. A variant of Lichen myxedematosus. (4221381)
1966

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 pathwaysScoreTop Affiliating Genes
19.1ACTC1, FGF2
29.1FGF2, 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-3-phosphate biosynthetic processGO:00360929.6FGF2, PIK3C2A
2phosphatidylinositol biosynthetic processGO:00066619.5FGF2, PIK3C2A
3phosphatidylinositol phosphorylationGO:00468549.5FGF2, PIK3C2A
4phosphatidylinositol-mediated signalingGO:00480159.5FGF2, PIK3C2A
5positive regulation of gene expressionGO:00106289.1ACTC1, FGF2

Molecular functions related to Scleromyxedema according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
11-phosphatidylinositol-3-kinase activityGO:00163039.1FGF2, PIK3C2A

Sources for Scleromyxedema

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
26GTR
27HGMD
28HMDB
29ICD10
30ICD10 via Orphanet
31ICD9CM
32IUPHAR
33KEGG
36MedGen
38MeSH
39MESH via Orphanet
40MGI
43NCI
44NCIt
45NDF-RT
48NINDS
49Novoseek
51OMIM
52OMIM via Orphanet
56PubMed
57QIAGEN
62SNOMED-CT via Orphanet
66Tumor Gene Family of Databases
67UMLS
68UMLS via Orphanet