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MCID: KMR001
MIFTS: 43

Kimura Disease

Categories: Bone diseases, Immune diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes
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Aliases & Classifications for Kimura Disease

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MalaCards integrated aliases for Kimura Disease:

Name: Kimura Disease 12 74 52 58 54 15 17 71
Eosinophilic Lymphogranuloma 52 58
Eosinophilic Hyperplastic Lymphogranuloma 52
Eosinophilic Lymphofollicular Granuloma 52
Eosinophilic Granuloma of Soft Tissue 52
Eosinophilic Lymphofolliculosis 52
Kimura's Disease 12

Characteristics:

Orphanet epidemiological data:

58
kimura disease
Prevalence: <1/1000000 (Worldwide); Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Bone diseases Skin diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 33
Orphanet: 58  
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:7365
MeSH 43 D000796
NCIt 49 C26867
SNOMED-CT 67 399894006
MESH via Orphanet 44 D000796
ICD10 via Orphanet 33 I89.8
UMLS via Orphanet 72 C0033838
Orphanet 58 ORPHA482
SNOMED-CT via HPO 68 10890000
UMLS 71 C0033838
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Summaries for Kimura Disease

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NIH Rare Diseases : 52 Kimura disease is a rare, noncancerous, chronic condition that causes the tissue under the skin of the head or neck to become swollen. Masses generally appear in a person's mid-20s and the disease mainly affects Asian men. Kimura disease is diagnosed by a surgical biopsy and the cause of the disease is unknown. Treatment includes surgery, medication, or a combination of the two. However, the masses tend to develop again after treatment. Because this condition rarely causes health problems, people with Kimura disease have a good outcome.

MalaCards based summary : Kimura Disease, also known as eosinophilic lymphogranuloma, is related to disseminated eosinophilic collagen disease and cytokine deficiency. An important gene associated with Kimura Disease is IL5 (Interleukin 5), and among its related pathways/superpathways are Innate Immune System and PEDF Induced Signaling. Affiliated tissues include lymph node, skin and salivary gland, and related phenotypes are eosinophilia and follicular hyperplasia

Wikipedia : 74 Kimura's disease is a benign rare chronic inflammatory disorder. Its primary symptoms are subdermal... more...

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Related Diseases for Kimura Disease

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Diseases related to Kimura Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 280)
# Related Disease Score Top Affiliating Genes
1 disseminated eosinophilic collagen disease 30.4 RNASE3 IL5
2 cytokine deficiency 30.3 IL5 IL13
3 mikulicz disease 30.1 IL5 IL4 IL13
4 igg4-related disease 29.9 IL4 IL33
5 autoimmune disease 29.8 IL5 IL4 IL13 CCL5
6 mycosis fungoides 29.8 IL5 IL4 CCR3
7 ulcerative colitis 29.8 IL4 IL13 CCR3
8 colitis 29.6 IL4 IL13 CCR3
9 folliculitis 29.5 IL5 IL4 CCL5 CCL11
10 urticaria 29.3 RNASE3 IL5 IL4 IL13 IGHE
11 dermatitis 29.1 TSLP IL5 IL4 IL13 IGHE CCL11
12 hypereosinophilic syndrome 28.8 RNASE3 IL5 IL4 IL13 IGHE IGES
13 lung disease 28.8 RNASE3 IL5 IL4 IL13 CCR3 CCL5
14 behcet syndrome 28.5 IL5 IL4 IL33 IL13 CCR3
15 otitis media 28.5 RNASE3 IL5 IL4 IL13 IGHE CCL5
16 allergic rhinitis 27.5 RNASE3 IL5 IL4 IL13 IGHE IGES
17 rhinitis 26.8 TSLP RNASE3 IL5 IL4 IL33 IL13
18 allergic hypersensitivity disease 26.2 TSLP RNASE3 IL5 IL4 IL33 IL13
19 asthma 25.8 TSLP RNASE3 IL5 IL4 IL33 IL13
20 dermatitis, atopic 25.3 TSLP RNASE3 IL5 IL4 IL33 IL13
21 nephrotic syndrome 10.6
22 loeffler endocarditis 10.4 RNASE3 IL5
23 vasomotor rhinitis 10.3 RNASE3 IL5
24 nickel allergic contact dermatitis 10.3 IL5 IL4
25 papillary conjunctivitis 10.3 IL4 CCL11
26 glomerulonephritis 10.3
27 aztreonam allergy 10.3 IL5 IL13
28 anisakiasis 10.3 RNASE3 IGHE
29 wells syndrome 10.3 RNASE3 IL5
30 angioimmunoblastic lymphadenopathy with dysproteinemia 10.3 IL5 IL13
31 cefaclor allergy 10.3 IL5 IL13
32 idiopathic nephrotic syndrome 10.3 IL4 IGHE
33 cefuroxime allergy 10.3 IL5 IL13
34 ceftriaxone allergy 10.3 IL5 IL13
35 ocular cicatricial pemphigoid 10.3 RNASE3 IL4
36 adhesive otitis media 10.3 RNASE3 CCL11
37 vasculitis 10.2
38 lymphadenitis 10.2
39 opisthorchiasis 10.2 IL4 IGES
40 drug allergy 10.2 IL5 IGHE
41 maxillary sinusitis 10.2 RNASE3 CCL5
42 endomyocardial fibrosis 10.2 RNASE3 IL5 IL4
43 scleritis 10.2 IL5 IL4
44 dacryoadenitis 10.2 IL4 IL13
45 cow milk allergy 10.2 RNASE3 IL5 IL4
46 membranous nephropathy 10.2
47 proliferative glomerulonephritis 10.2
48 pemphigoid gestationis 10.2 RNASE3 IL5 CCL11
49 cellulitis 10.2 RNASE3 IL5 IL4
50 toxic oil syndrome 10.2 IL5 IL4 IGHE

Graphical network of the top 20 diseases related to Kimura Disease:



Diseases related to Kimura Disease
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Symptoms & Phenotypes for Kimura Disease

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Human phenotypes related to Kimura Disease:

58 31 (show all 7)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 eosinophilia 58 31 hallmark (90%) Very frequent (99-80%) HP:0001880
2 follicular hyperplasia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002729
3 increased circulating ige level 31 hallmark (90%) HP:0003212
4 abnormal salivary gland morphology 31 occasional (7.5%) HP:0010286
5 lymphadenopathy 58 Frequent (79-30%)
6 increased circulating total ige level 58 Very frequent (99-80%)
7 abnormality of the salivary glands 58 Occasional (29-5%)

MGI Mouse Phenotypes related to Kimura Disease:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.81 CCL11 CCL5 CCR3 CRLF2 IL13 IL33
2 digestive/alimentary MP:0005381 9.73 CRLF2 IL13 IL33 IL4 IL5 TSLP
3 immune system MP:0005387 9.61 CCL11 CCL5 CCR3 CRLF2 IL13 IL33
4 respiratory system MP:0005388 9.23 CCL11 CCR3 CRLF2 IL13 IL33 IL4
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Drugs & Therapeutics for Kimura Disease

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Search Clinical Trials , NIH Clinical Center for Kimura Disease

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Genetic Tests for Kimura Disease

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Anatomical Context for Kimura Disease

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MalaCards organs/tissues related to Kimura Disease:

40
Lymph Node, Skin, Salivary Gland, Bone, Kidney, Colon, Brain
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Publications for Kimura Disease

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Articles related to Kimura Disease:

(show top 50) (show all 205)
# Title Authors PMID Year
1
Kimura disease: review of the literature. 61 52
18808562 2008
2
Comparison of the efficacy of different treatment modalities for Kimura's disease. 52
27614905 2017
3
Thrombotic storm in Kimura disease. 61 54
19468829 2010
4
Kimura's disease with eosinophilic panniculitis--treated with cyclosporine: a case report. 61 54
20236545 2010
5
Groin abnormalities: ultrasonographic and clinical findings. 61
31786905 2020
6
A case of IgG4-related disease presented with Kimura disease-like skin eruption, rheumatoid arthritis-like abnormality and interstitial pneumonia. 61
32189363 2020
7
A Rare Case of Kimura Disease. 61
32180608 2020
8
Dyspnea and Cough: A Rare Report of the Kimura Disease. 61
32133969 2020
9
Remission of Kimura Disease With Carotid Hypervascularization After Cyclosporine Treatment. 61
32363093 2020
10
Diagnostic dilemma of kimura disease of eyelids. 61
32008028 2020
11
Human Polyomavirus 6 with the Asian-Japanese Genotype in Cases of Kimura Disease and Angiolymphoid Hyperplasia with Eosinophilia. 61
31981580 2020
12
Kimura disease in a 2-year-old boy with generalized lymphadenopathy. 61
31833620 2019
13
Tissue-infiltrating immune cells contribute to understanding the pathogenesis of Kimura disease: A case report. 61
31852109 2019
14
The clinicopathological characteristics of Kimura disease in Chinese patients. 61
31440918 2019
15
Kimura disease: a case report of a rare illness presenting as a common complaint. 61
30808157 2019
16
Recurrent Kimura Disease: Original Observations in 2 Patients With an Unusual Protracted Course of the Inflammatory Process. 61
31651639 2019
17
Mepolizumab as an effective treatment for Kimura's disease associated with ulcerative colitis: A case report. 61
31681687 2019
18
Kimura disease with nephrotic syndrome in a child- A rare association. 61
31361234 2019
19
Kimura disease: case report and brief review of literature. 61
31086850 2019
20
An unexpected asymptomatic epiglottal site of Kimura disease. 61
30712651 2019
21
Kimura disease and immunoglobulin G4-related disease: a possible overlap in a patient with severe systemic symptoms. 61
30543201 2019
22
Angiolymphoid hyperplasia with eosinophilia and Kimura disease overlap, with evidence of diffuse visceral involvement. 61
30362278 2019
23
Concurrent Kidney Glomerular and Interstitial Lesions Associated with Kimura's Disease. 61
31387106 2019
24
A rare case of inguinal kimura disease. 61
30350814 2018
25
Computerized tomography features and clinicopathological analysis of Kimura disease in head and neck. 61
30186444 2018
26
Orbital Mass With Features of Both Kimura Disease and Immunoglobulin G4-Related Disease. 61
29923967 2018
27
Imaging of Benign Neoplastic and Nonneoplastic Salivary Gland Tumors. 61
29622111 2018
28
Kimura Disease: A differential diagnosis in a nephrotic child. 61
30302182 2018
29
Kimura Disease: A Rare Case Report From Himalayan Region. 61
29238694 2017
30
Kimura disease of buccal region in a pediatric patient with nephrotic syndrome: A case report. 61
29310412 2017
31
Successful therapy with mycophenolic acid in a membranous glomerulonephritis due to Kimura disease
. 61
28853699 2017
32
Kimura disease. 61
29469792 2017
33
Basophils are increased and express increased levels of interleukin-4 in the parotid lesions of Kimura disease. 61
29094020 2017
34
A Rare Case of Kimura Disease with Bilateral Parotid Involvement. 61
28946727 2017
35
Treatment of Kimura disease with mycophenolate mofetil monotherapy. 61
28932783 2017
36
Expression of the interleukin-21 and phosphorylated extracellular signal regulated kinase 1/2 in Kimura disease. 61
28108473 2017
37
Superficial Parotidectomy Plane for Debulking Surgery in Kimura Disease. 61
28468187 2017
38
Recurrent Kimura Disease of the Inner Canthus With No Lymphadenopathy. 61
26020715 2017
39
[An indolent and fluctuating subcutaneous mass of the skull in a 5-year-old patient: Diagnostic approach and difficulties]. 61
28233718 2017
40
Acute Limb Ischemia and Coronary Artery Disease in a Case of Kimura's Disease. 61
28382271 2017
41
Nephrotic syndrome during the tapering of oral steroids after pathological diagnosis of Kimura disease from a lacrimal gland mass: case report and review of 10 Japanese patients. 61
29279552 2017
42
Detailed Imaging Findings in a Rare Case of Kimura Disease, with Special Mention on Diffusion Weighted Imaging. 61
29657623 2017
43
Kimura disease in a Maori man with nephrotic syndrome. 61
27981775 2016
44
Concurrent Kimura disease and lupus nephritis: A case report. 61
27741124 2016
45
Eosinophilia and multiple lymphadenopathy: Kimura disease, a rare, but benign condition. 61
27581232 2016
46
Kimura Disease of the Breast - A Previously Undescribed Entity. 61
27058987 2016
47
Human Polyomavirus-6 Infecting Lymph Nodes of a Patient With an Angiolymphoid Hyperplasia With Eosinophilia or Kimura Disease. 61
26962076 2016
48
Kimura Disease Presenting as Forehead Soft Mass. 61
27100647 2016
49
A case of refractory Kimura disease with a buccal bulky mass successfully treated with low-dose cyclosporine A: report and review of the literature. 61
26666475 2016
50
Uncommon clinical presentation of KimuraÂ’s disease as bilateral retroauricular masses in a young Malian male: successful surgical approach. 61
27373132 2016
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Variations for Kimura Disease

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Expression for Kimura Disease

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Search GEO for disease gene expression data for Kimura Disease.
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Pathways for Kimura Disease

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Pathways related to Kimura Disease according to GeneCards Suite gene sharing:

(show all 26)
# Super pathways Score Top Affiliating Genes
1
Innate Immune System 65
Show member pathways
 13.81 RNASE3 IL5 IL4 IL33 IL13 IGHE
2
PEDF Induced Signaling 63
Show member pathways
 13.4 TSLP IL5 IL4 IL33 IL13 CRLF2
3
Akt Signaling 63
Show member pathways
 13.22 IL5 IL4 IL13 CCR3 CCL5 CCL11
4
Cytokine Signaling in Immune system 65
Show member pathways
 13.16 IL5 IL4 IL33 IL13 IGHE CCL5
5
PAK Pathway 63
Show member pathways
 13.12 IL5 IL4 IL13 CCR3 CCL5 CCL11
6
Allograft rejection 1 36
Show member pathways
 12.69 RNASE3 IL5 IL4 IL13 CCL11
7
Immune response Fc epsilon RI pathway 22
Show member pathways
 12.42 IL5 IL4 IL13 IGHE
8
JAK-STAT signaling pathway 36
Show member pathways
 12.27 TSLP IL5 IL4 IL13 CRLF2
9
IL-17 Family Signaling Pathways 64
Show member pathways
 12.19 IL5 IL4 IL13 CCL11
10
Immune response CCR3 signaling in eosinophils 22
Show member pathways
 12.15 CCR3 CCL5 CCL11
11
Chemokine Superfamily: Human/Mouse Ligand-Receptor Interactions 64
Show member pathways
 12.13 CCR3 CCL5 CCL11
12
Hematopoietic Stem Cells and Lineage-specific Markers 64
11.85 IL5 IL4 CCR3
13
Interleukin-4 and 13 signaling 65
11.79 IL4 IL13 IGHE CCL11
14
Glucocorticoid receptor regulatory network 1
11.67 IL5 IL4 IL13
15
Innate Lymphoid Cells Differentiation 64
11.54 TSLP IL5 IL4 IL33 IL13 CRLF2
16
IL4-mediated signaling events 1
11.42 IL5 IL4 IGHE CCL11
17
Interleukin-10 signaling 65
11.4 IL4 IL13 CCL5
18
Thymic Stromal LymphoPoietin (TSLP) Signaling Pathway 1
11.37 TSLP CRLF2 CCL11
19
Lung fibrosis 1
11.23 IL5 IL4 IL13 CCR3 CCL5 CCL11
20
Development and heterogeneity of the ILC family 1
11.16 TSLP IL5 IL4 IL33 IL13
21
Inflammatory Response Pathway 1
11.14 IL5 IL4
22
Cytokines and Inflammatory Response 1
11.13 IL5 IL4 IL13
23
OX40 Pathway 63
11.05 IL5 IL4 IL13
24
G-protein signaling_Rap2B regulation pathway 22
10.87 IL5 IL4 IL13 CCL5 CCL11
25
Th2 Differentiation 64
10.76 IL5 IL4 IL33 IL13 CRLF2 CCL11
26
IL-9 Signaling and its Primary Biological Effects in Different Immune Cell Types 64
10.62 IL5 IL4 IL13
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GO Terms for Kimura Disease

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Cellular components related to Kimura Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.56 TSLP RNASE3 IL5 IL4 IL33 IL13
2 external side of plasma membrane GO:0009897 9.46 IL13 IGHE CRLF2 CCR3
3 extracellular region GO:0005576 9.32 TSLP RNASE3 IL5 IL4 IL33 IL13

Biological processes related to Kimura Disease according to GeneCards Suite gene sharing:

(show all 26)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of cell proliferation GO:0008284 9.92 TSLP IL5 IL4 CRLF2
2 chemotaxis GO:0006935 9.75 CCR3 CCL5 CCL11
3 inflammatory response GO:0006954 9.72 IL5 IL13 CCR3 CCL5 CCL11
4 chemokine-mediated signaling pathway GO:0070098 9.67 CCR3 CCL5 CCL11
5 lymphocyte chemotaxis GO:0048247 9.63 CCL5 CCL11
6 positive regulation of JAK-STAT cascade GO:0046427 9.62 IL5 CCL5
7 interleukin-7-mediated signaling pathway GO:0038111 9.61 TSLP CRLF2
8 microglial cell activation GO:0001774 9.61 IL4 IL13
9 positive regulation of interleukin-10 production GO:0032733 9.6 TSLP IL4
10 innate immune response in mucosa GO:0002227 9.59 RNASE3 IL4
11 eosinophil chemotaxis GO:0048245 9.58 CCL5 CCL11
12 positive regulation of macrophage activation GO:0043032 9.57 IL33 IL13
13 macrophage activation GO:0042116 9.56 IL4 IL13
14 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.56 TSLP IL4 IL13 CCL5
15 positive regulation of immunoglobulin secretion GO:0051024 9.55 IL5 IL33
16 positive regulation of interleukin-10 secretion GO:2001181 9.54 IL4 IL13
17 positive regulation of B cell proliferation GO:0030890 9.54 IL5 IL4 IL13
18 positive regulation of mast cell degranulation GO:0043306 9.52 IL4 IL13
19 positive regulation of interleukin-5 production GO:0032754 9.51 TSLP IL33
20 immune response GO:0006955 9.5 IL5 IL4 IL13 IGHE CCR3 CCL5
21 positive regulation of interleukin-5 secretion GO:2000664 9.48 TSLP CRLF2
22 positive regulation of STAT cascade GO:1904894 9.46 TSLP CRLF2
23 positive regulation of mast cell activation GO:0033005 9.43 TSLP CRLF2
24 negative regulation of complement-dependent cytotoxicity GO:1903660 9.4 IL4 IL13
25 positive regulation of interleukin-13 production GO:0032736 9.33 TSLP IL4 IL33
26 cytokine-mediated signaling pathway GO:0019221 9.17 IL5 IL4 IL13 IGHE CRLF2 CCL5

Molecular functions related to Kimura Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.17 TSLP IL5 IL4 IL33 IL13 CCL5
2 CCR chemokine receptor binding GO:0048020 9.16 CCL5 CCL11
3 cytokine receptor binding GO:0005126 8.96 IL4 IL13
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Sources for Kimura Disease

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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