MCID: ERY017
MIFTS: 45

Erythema Elevatum Diutinum

Categories: Bone diseases, Cardiovascular diseases, Rare diseases, Skin diseases

Aliases & Classifications for Erythema Elevatum Diutinum

MalaCards integrated aliases for Erythema Elevatum Diutinum:

Name: Erythema Elevatum Diutinum 12 53 59 44 15 72 33

Characteristics:

Orphanet epidemiological data:

59
erythema elevatum diutinum
Age of onset: All ages; Age of death: normal life expectancy;

Classifications:



External Ids:

Disease Ontology 12 DOID:0060567
MeSH 44 C535509
SNOMED-CT 68 58872001
ICD10 33 L95.1
MESH via Orphanet 45 C535509
ICD10 via Orphanet 34 L95.1
UMLS via Orphanet 73 C0263398
Orphanet 59 ORPHA90000
UMLS 72 C0263398

Summaries for Erythema Elevatum Diutinum

NIH Rare Diseases : 53 Erythema elevatum diutinum is a rare type of chronic cutaneous vasculitis characterized by red, purple, brown or yellow papules, plaques (thick, red patches of skin), or nodules.These lesions are located mainly on the knees, elbows, hands, feet, face, genitals and buttocks. Although it is a chronic condition, most cases respond well to treatment with dapsone. The cause of this condition remains unknown.

MalaCards based summary : Erythema Elevatum Diutinum is related to vasculitis and granuloma annulare. An important gene associated with Erythema Elevatum Diutinum is PECAM1 (Platelet And Endothelial Cell Adhesion Molecule 1), and among its related pathways/superpathways are Innate Immune System and Class I MHC mediated antigen processing and presentation. Affiliated tissues include skin, neutrophil and bone, and related phenotypes are arthralgia and skin rash

Disease Ontology : 12 A vasculitis characterised by red, purple, brown or yellow papules (raised spot), plaques, or nodules, found on the backs of the hands, other extensor surfaces overlying joints, and on the buttocks.

Wikipedia : 75 Erythema elevatum diutinum is a form of... more...

Related Diseases for Erythema Elevatum Diutinum

Diseases related to Erythema Elevatum Diutinum via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 144)
# Related Disease Score Top Affiliating Genes
1 vasculitis 32.0 TNFRSF1B S100A9 CD40LG
2 granuloma annulare 31.7 TNFRSF1B F13A1
3 skin disease 10.7
4 pyoderma 10.7
5 pyoderma gangrenosum 10.7
6 keratitis, hereditary 10.7
7 myelodysplastic syndrome 10.7
8 alk+ histiocytosis 10.6 F13A1 CD1A
9 kaposi sarcoma 10.6
10 immune deficiency disease 10.6
11 dermatitis herpetiformis, familial 10.6
12 dermatitis herpetiformis 10.6
13 myeloma, multiple 10.6
14 myofibroma 10.5 F13A1 CD1A
15 fibroma 10.5
16 dermatitis 10.5
17 necrobiosis lipoidica 10.5
18 crohn's disease 10.5
19 lupus erythematosus 10.5
20 eosinophilic granuloma 10.5
21 acute necrotizing encephalitis 10.5 TBX21 FOXP3
22 autoimmune disease 10.4
23 systemic lupus erythematosus 10.4
24 celiac disease 1 10.4
25 neutrophilic dermatosis, acute febrile 10.4
26 human immunodeficiency virus type 1 10.4
27 cutaneous lupus erythematosus 10.4
28 exanthem 10.4
29 inflammatory bowel disease 10.4
30 bacterial infectious disease 10.4
31 sarcoma 10.4
32 scleritis 10.4
33 hypothyroidism 10.4
34 hepatitis b 10.4
35 relapsing polychondritis 10.4
36 spindle cell sarcoma 10.4
37 acquired immunodeficiency syndrome 10.4
38 connective tissue disease 10.4
39 hypereosinophilic syndrome 10.4
40 kaposiform hemangioendothelioma 10.4 PECAM1 CD34
41 multifocal motor neuropathy 10.4 CD40LG CD1E CD1A
42 cochlear disease 10.3 TNFRSF1B GATA3
43 ecthyma 10.3 TBX21 GATA3 CD4
44 acroosteolysis 10.3
45 spondyloarthropathy 1 10.3
46 breast cancer 10.3
47 diabetes mellitus, noninsulin-dependent 10.3
48 leukemia, chronic lymphocytic 10.3
49 pityriasis rubra pilaris 10.3
50 porphyria cutanea tarda 10.3

Graphical network of the top 20 diseases related to Erythema Elevatum Diutinum:



Diseases related to Erythema Elevatum Diutinum

Symptoms & Phenotypes for Erythema Elevatum Diutinum

Human phenotypes related to Erythema Elevatum Diutinum:

59 32 (show all 8)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
2 skin rash 59 32 hallmark (90%) Very frequent (99-80%) HP:0000988
3 vasculitis in the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0200029
4 abnormal blistering of the skin 59 32 frequent (33%) Frequent (79-30%) HP:0008066
5 skin vesicle 59 32 frequent (33%) Frequent (79-30%) HP:0200037
6 increased antibody level in blood 59 32 frequent (33%) Frequent (79-30%) HP:0010702
7 myalgia 59 32 occasional (7.5%) Occasional (29-5%) HP:0003326
8 skin nodule 59 32 occasional (7.5%) Occasional (29-5%) HP:0200036

GenomeRNAi Phenotypes related to Erythema Elevatum Diutinum according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.17 CD1A CD1D CD34 CXCL8 F13A1 S100A9

MGI Mouse Phenotypes related to Erythema Elevatum Diutinum:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.97 CD1D CD40LG F13A1 FOXP3 G6PD GATA3
2 hematopoietic system MP:0005397 9.97 CD1D CD34 CD4 CD40LG CD68 F13A1
3 cellular MP:0005384 9.96 CD34 CD4 CD40LG FOXP3 G6PD GATA3
4 homeostasis/metabolism MP:0005376 9.73 CD1D CD34 CD4 CD40LG F13A1 FOXP3
5 immune system MP:0005387 9.36 CD1D CD34 CD4 CD40LG CD68 FOXP3

Drugs & Therapeutics for Erythema Elevatum Diutinum

Search Clinical Trials , NIH Clinical Center for Erythema Elevatum Diutinum

Cochrane evidence based reviews: erythema elevatum diutinum

Genetic Tests for Erythema Elevatum Diutinum

Anatomical Context for Erythema Elevatum Diutinum

MalaCards organs/tissues related to Erythema Elevatum Diutinum:

41
Skin, Neutrophil, Bone, Liver, Testes, Breast, Thyroid

Publications for Erythema Elevatum Diutinum

Articles related to Erythema Elevatum Diutinum:

(show top 50) (show all 321)
# Title Authors PMID Year
1
Primary Surgical Treatment of Erythema Elevatum Diutinum. 38
30241974 2019
2
Erythema elevatum diutinum: a case report and review of literature. 38
30074624 2019
3
Erythema elevatum diutinum a rare and poorly understood cutaneous vasculitis: A single institution experience. 38
30362150 2019
4
[Erratum to: Monolocular erythema elevatum diutinum on the back of the hand]. 38
30270407 2018
5
[Monolocular erythema elevatum diutinum on the back of the hand]. 38
30264321 2018
6
Rare presentation of erythema elevatum diutinum. 38
30238047 2018
7
Erythema elevatum diutinum. 38
30066783 2018
8
Late-Stage Erythema Elevatum Diutinum Mimicking a Fibroblastic Tumor: A Potential Pitfall. 38
29293121 2018
9
Unusual presentation of erythema elevatum diutinum with underlying hepatitis B infection. 38
30063775 2018
10
Erythema elevatum diutinum-like vasculitis secondary to cocaine adulterated with levamisole. 38
29423942 2018
11
HIV-associated erythema elevatum diutinum: a case report and review of a clinically distinct variant. 38
30142732 2018
12
Erythema elevatum diutinum verbunden mit schwerer oropharyngealer Ulzeration und Pyoderma gangraenosum. 38
29750450 2018
13
Erythema elevatum diutinum associated with severe oropharyngeal ulceration and pyoderma gangrenosum. 38
29750453 2018
14
Neutrophilic dermatoses: a broad spectrum of disease. 38
29368857 2018
15
Pyoderma gangrenosum and erythema elevatum diutinum associated with a high-risk myelodysplastic syndrome: case report. 38
30057994 2018
16
Erythema Elevatum Diutinum Treated With Mycophenolate Mofetil. 38
29509562 2018
17
Erythema elevatum diutinum with pustule formation: An unusual finding. 38
29292528 2018
18
Late-stage nodular erythema elevatum diutinum mimicking sclerotic fibroma. 38
29068068 2018
19
Truncal Erythema Elevatum Diutinum With Elevated Cryoglobulin Level. 38
29901001 2017
20
Complete resolution of erythema elevatum diutinum using oral sulfasalazine. 38
29469798 2017
21
Erythema elevatum diutinum in Crohn's disease-associated Spondyloarthritis - a rare vasculitis, an unusual association. 38
28917218 2017
22
Vesiculobullous variant of erythema elevatum diutinum. 38
28639709 2017
23
Erythema elevatum diutinum presenting with palmoplantar keratoderma. 38
28512994 2017
24
Erythema elevatum diutinum in a healthy child. 38
28397292 2017
25
Chronic Localized Fibrosing Leukocytoclastic Vasculitis Associated With Lymphedema, Intralymphatic and Intravascular Lymphocytosis, and Chronic Myelogenous Leukemia: A Case Report of Unilateral Erythema Elevatum Diutinum. 38
27930381 2017
26
Erythema elevatum diutinum-associated with loss of the uvula. 38
28443312 2017
27
Erythema Elevatum Diutinum. 38
28183422 2017
28
Approach to cutaneous vasculitides with special emphasis on small vessel vasculitis: histopathology and direct immunofluorescence. 38
27787337 2017
29
A rare case of erythema elevatum diutinum presenting as diffuse neuropathy. 38
28050587 2017
30
Erythema elevatum diutinum involving palms and soles: a case report and literature review. 38
28469800 2017
31
Pediatric case of microscopic polyangiitis with skin manifestations resembling vesiculobullous type erythema elevatum diutinum with immunoglobulin A antineutrophil cytoplasmic antibody. 38
27097593 2016
32
Erythema elevatum diutinum in association with IgA monoclonal gammopathy: A rare case report. 38
27559509 2016
33
Hidradenitis suppurativa (HS): An unrecognized paradoxical effect of biologic agents (BA) used in chronic inflammatory diseases. 38
26965410 2016
34
Neutrophilic Dermatoses in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A French Multicenter Study of 17 Cases and Literature Review. 38
26986103 2016
35
Granuloma Faciale and Erythema Elevatum Diutinum in Relation to Immunoglobulin G4-Related Disease: An Appraisal of 32 Cases. 38
27124923 2016
36
Primary cutaneous lymphomas: the analysis of cases treated in the Department of Dermatology University Hospital in Krakow. 38
29676886 2016
37
Erythema elevatum diutinum in acquired immune deficiency syndrome: Can it be an immune reconstitution inflammatory syndrome? 38
27190420 2016
38
A case of mistaken identity: unilateral erythema elevatum diutinum associated with IgA paraproteinaemia. 38
25754401 2015
39
A Case of Erythema Elevatum Diutinum With Pancytopenia: Focus on Dapsone-Induced Hematologic Side Effects and Colchicine as a Safe Treatment Option. 38
26461818 2015
40
[Cutaneous symptoms of various vasculitides]. 38
26195032 2015
41
Erythema elevatum diutinum and hypothyroidism: coincidence or causal relationship? 38
26375227 2015
42
Early Onset Dapsone-induced Photosensitive Dermatitis: A Rare Side Effect of a Common Drug. 38
26999988 2015
43
Erythema elevatum diutinum coexisting with ankylosing spondylitis. 38
27708930 2015
44
Erythema elevatum diutinum: an atypical presentation. 38
25521326 2015
45
Case of erythema elevatum diutinum associated with IgA paraproteinemia successfully controlled with thalidomide and plasma exchange. 38
25257541 2015
46
Erythema elevatum diutinum: a review of presentation and treatment. 38
25288365 2014
47
Nodular Erythema Elevatum Diutinum Mimicking Kaposi's Sarcoma in a Human Immunodeficiency Virus Infected Patient. 38
25484391 2014
48
[Erythema elevatum diutinum on glans penis is a rare manifestation]. 38
25353682 2014
49
An unusual case of Sweet syndrome in a child: overlapping presentation with erythema elevatum diutinum. 38
24521734 2014
50
Neutrophilic dermatoses as systemic diseases. 38
24767185 2014

Variations for Erythema Elevatum Diutinum

Expression for Erythema Elevatum Diutinum

Search GEO for disease gene expression data for Erythema Elevatum Diutinum.

Pathways for Erythema Elevatum Diutinum

Pathways related to Erythema Elevatum Diutinum according to GeneCards Suite gene sharing:

(show all 20)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.84 TNFRSF1B S100A9 PECAM1 GATA3 F13A1 CXCL8
2
Show member pathways
13.29 CD40LG CD4 CD34 CD1D CD1C CD1B
3
Show member pathways
12.71 TNFRSF1B FOXP3 CXCL8 CD40LG CD4
4
Show member pathways
12.42 TBX21 GATA3 FOXP3 CD4
5 12.32 TNFRSF1B TBX21 PECAM1 GATA3 FOXP3 CD40LG
6 12.06 CD1E CD1D CD1C CD1B CD1A
7 11.98 PECAM1 CD40LG CD4 CD34
8
Show member pathways
11.88 TBX21 GATA3 CD4
9 11.86 CD68 CD4 CD34 CD1C
10 11.84 TNFRSF1B GATA3 F13A1 CXCL8
11 11.76 CD40LG CD34 CD1D CD1C CD1B CD1A
12 11.66 TBX21 GATA3 CD4 CD34
13 11.65 TBX21 GATA3 CXCL8
14
Show member pathways
11.65 TBX21 GATA3 FOXP3 CXCL8 CD40LG
15 11.62 GATA3 CD4 CD34
16 11.59 CD4 CD34 CD1D CD1C CD1A
17 11.4 PECAM1 CXCL8 CD40LG
18 11.36 GATA3 FOXP3 CD4
19 11.15 CD4 CD34 CD1E CD1D CD1C CD1B
20 11.08 CD40LG CD4 CD34 CD1D CD1C

GO Terms for Erythema Elevatum Diutinum

Cellular components related to Erythema Elevatum Diutinum according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 integral component of plasma membrane GO:0005887 9.95 CD40LG CD4 CD34 CD1E CD1D CD1C
2 external side of plasma membrane GO:0009897 9.73 PECAM1 CD40LG CD4 CD34
3 membrane raft GO:0045121 9.67 TNFRSF1B PECAM1 CD4 CD1A
4 lysosome GO:0005764 9.65 CD34 CD1E CD1D CD1C CD1B
5 endosome GO:0005768 9.63 CD68 CD1E CD1D CD1C CD1B CD1A
6 endosome membrane GO:0010008 9.35 CD68 CD1D CD1C CD1B CD1A
7 cell surface GO:0009986 9.02 CD40LG CD4 CD34 CD1D CD1B
8 plasma membrane GO:0005886 10.1 TNFRSF1B S100A9 PECAM1 CD68 CD40LG CD4

Biological processes related to Erythema Elevatum Diutinum according to GeneCards Suite gene sharing:

(show all 23)
# Name GO ID Score Top Affiliating Genes
1 immune system process GO:0002376 9.97 S100A9 GATA3 CD4 CD1E CD1D CD1C
2 inflammatory response GO:0006954 9.91 TNFRSF1B S100A9 CXCL8 CD40LG
3 immune response GO:0006955 9.91 TNFRSF1B CXCL8 CD40LG CD4 CD1E CD1D
4 adaptive immune response GO:0002250 9.89 CD4 CD1E CD1C CD1B CD1A
5 cytokine-mediated signaling pathway GO:0019221 9.88 TNFRSF1B GATA3 F13A1 CXCL8 CD4
6 regulation of immune response GO:0050776 9.87 TBX21 CD40LG CD34 CD1D CD1C CD1B
7 T cell receptor signaling pathway GO:0050852 9.77 GATA3 FOXP3 CD4
8 response to virus GO:0009615 9.76 TBX21 GATA3 FOXP3
9 negative regulation of inflammatory response GO:0050728 9.75 TNFRSF1B GATA3 FOXP3
10 positive regulation of T cell proliferation GO:0042102 9.73 CD40LG CD4 CD1D
11 T cell differentiation GO:0030217 9.71 TBX21 GATA3 CD4
12 cytokine production GO:0001816 9.63 S100A9 FOXP3 CD4
13 positive regulation of interleukin-10 production GO:0032733 9.61 CD40LG CD34
14 positive regulation of interleukin-4 production GO:0032753 9.61 GATA3 FOXP3 CD40LG
15 regulation of neuron apoptotic process GO:0043523 9.6 GATA3 G6PD
16 negative regulation of interleukin-2 production GO:0032703 9.58 TBX21 GATA3 FOXP3
17 negative regulation of T-helper 17 cell differentiation GO:2000320 9.57 TBX21 FOXP3
18 lymphocyte migration GO:0072676 9.55 TBX21 GATA3
19 positive regulation of T cell mediated cytotoxicity GO:0001916 9.55 CD1E CD1D CD1C CD1B CD1A
20 T cell selection GO:0045058 9.52 CD4 CD1D
21 glomerular endothelium development GO:0072011 9.51 PECAM1 CD34
22 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.35 CD1E CD1D CD1C CD1B CD1A
23 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 9.02 CD1E CD1D CD1C CD1B CD1A

Molecular functions related to Erythema Elevatum Diutinum according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lipopeptide binding GO:0071723 9.55 CD1E CD1D CD1C CD1B CD1A
2 exogenous lipid antigen binding GO:0030884 9.35 CD1E CD1D CD1C CD1B CD1A
3 endogenous lipid antigen binding GO:0030883 9.02 CD1E CD1D CD1C CD1B CD1A

Sources for Erythema Elevatum Diutinum

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11 DGIdb
17 EFO
18 ExPASy
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30 HGMD
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33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
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42 MedGen
44 MeSH
45 MESH via Orphanet
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57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
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73 UMLS via Orphanet
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