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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 75 53 59 55 15 17 72
Eosinophilic Lymphogranuloma 53 59
Eosinophilic Hyperplastic Lymphogranuloma 53
Eosinophilic Lymphofollicular Granuloma 53
Eosinophilic Granuloma of Soft Tissue 53
Eosinophilic Lymphofolliculosis 53
Kimura's Disease 12

Characteristics:

Orphanet epidemiological data:

59
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: 34
Orphanet: 59  
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:7365
MeSH 44 D000796
NCIt 50 C26867
SNOMED-CT 68 69484003
MESH via Orphanet 45 D000796
ICD10 via Orphanet 34 I89.8
UMLS via Orphanet 73 C0033838
Orphanet 59 ORPHA482
UMLS 72 C0033838
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Summaries for Kimura Disease

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NIH Rare Diseases : 53 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 hypereosinophilic syndrome and lung disease. An important gene associated with Kimura Disease is IL5 (Interleukin 5), and among its related pathways/superpathways are PEDF Induced Signaling and Akt Signaling. Affiliated tissues include lymph node, skin and salivary gland, and related phenotypes are eosinophilia and follicular hyperplasia

Wikipedia : 75 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 230)
# Related Disease Score Top Affiliating Genes
1 hypereosinophilic syndrome 30.5 RNASE3 IL5 IL4 CCL11
2 lung disease 29.7 RNASE3 IL5 CCL5
3 neuritis 29.7 IL4 CCL5
4 otitis media 29.7 RNASE3 IL4 CCL11
5 dermatitis 29.5 TSLP IL5 IL4 CCL11
6 allergic rhinitis 29.1 RNASE3 IL5 IL4 CCL11
7 folliculitis 28.9 IL5 IL4 CCL5 CCL11
8 rhinitis 28.4 RNASE3 IL5 IL4 CCL5 CCL11
9 dermatitis, atopic 26.5 TSLP RNASE3 IL5 IL4 CCR3 CCL5
10 allergic hypersensitivity disease 26.4 TSLP RNASE3 IL5 IL4 CCR3 CCL5
11 asthma 26.4 TSLP RNASE3 IL5 IL4 CCR3 CCL5
12 nephrotic syndrome 10.6
13 loeffler endocarditis 10.4 RNASE3 IL5
14 paragonimiasis 10.4 RNASE3 IL5
15 cow milk allergy 10.4 RNASE3 IL5
16 nickel allergic contact dermatitis 10.4 IL5 IL4
17 allergic contact dermatitis 10.4 IL5 IL4
18 contact dermatitis 10.4 IL5 IL4
19 eosinophilic granuloma 10.4
20 egg allergy 10.4 IL5 IL4
21 scleritis 10.4 IL5 IL4
22 sulfamethoxazole allergy 10.3 IL5 IL4
23 trichinosis 10.3 IL5 IL4
24 apple allergy 10.3 IL5 IL4
25 pollen allergy 10.3 RNASE3 IL5
26 gaucher disease, type ii 10.3 IL5 IL4
27 non-suppurative otitis media 10.3 IL5 IL4
28 pemphigoid gestationis 10.3 IL5 CCL11
29 filarial elephantiasis 10.3 IL5 IL4
30 glomerulonephritis 10.3
31 chronic tic disorder 10.3 IL5 IL4
32 intestinal schistosomiasis 10.3 IL5 IL4
33 toxic oil syndrome 10.3 IL5 IL4
34 wells syndrome 10.3 RNASE3 IL5
35 chronic meningitis 10.3 IL5 CCL11
36 enterobiasis 10.3 IL5 IL4
37 churg-strauss syndrome 10.3 RNASE3 IL5
38 strongyloidiasis 10.3 IL5 CCL11
39 papillary conjunctivitis 10.3 IL4 CCL11
40 allergic bronchopulmonary aspergillosis 10.3 IL5 IL4
41 aspergillosis 10.3 IL5 IL4
42 ascaris lumbricoides infection 10.3 TSLP IL5
43 cellulitis 10.2 RNASE3 IL5
44 radiculopathy 10.2 IL5 CCL11
45 vasculitis 10.2
46 lymphadenitis 10.2
47 respiratory syncytial virus infectious disease 10.2 IL5 CCL5
48 paranasal sinus disease 10.2 IL5 IL4
49 vernal conjunctivitis 10.2 RNASE3 IL4
50 night blindness, congenital stationary, type 1a 10.2 IL5 CCL11

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:

59 32 (show all 7)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 eosinophilia 59 32 hallmark (90%) Very frequent (99-80%) HP:0001880
2 follicular hyperplasia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002729
3 increased circulating total ige level 32 hallmark (90%) HP:0003212
4 abnormal salivary gland morphology 32 occasional (7.5%) HP:0010286
5 lymphadenopathy 59 Frequent (79-30%)
6 increased ige level 59 Very frequent (99-80%)
7 abnormality of the salivary glands 59 Occasional (29-5%)

MGI Mouse Phenotypes related to Kimura Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.63 CCL11 CCL5 CCR3 IL4 IL5 TSLP
2 immune system MP:0005387 9.43 CCL11 CCL5 CCR3 IL4 IL5 TSLP
3 respiratory system MP:0005388 9.02 CCL11 CCR3 IL4 IL5 TSLP
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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:

41
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 193)
# Title Authors PMID Year
1
Kimura disease: review of the literature. 38 6
18808562 2008
2
Comparison of the efficacy of different treatment modalities for Kimura's disease. 6
27614905 2017
3
Thrombotic storm in Kimura disease. 9 38
19468829 2010
4
Kimura's disease with eosinophilic panniculitis--treated with cyclosporine: a case report. 9 38
20236545 2010
5
Concurrent Kidney Glomerular and Interstitial Lesions Associated with Kimura's Disease. 38
31387106 2019
6
Kimura disease with nephrotic syndrome in a child- A rare association. 38
31361234 2019
7
Kimura disease: case report and brief review of literature. 38
31086850 2019
8
Angiolymphoid hyperplasia with eosinophilia and Kimura disease overlap, with evidence of diffuse visceral involvement. 38
30362278 2019
9
An unexpected asymptomatic epiglottal site of Kimura disease. 38
30712651 2019
10
Kimura disease: a case report of a rare illness presenting as a common complaint. 38
30808157 2019
11
Kimura disease and immunoglobulin G4-related disease: a possible overlap in a patient with severe systemic symptoms. 38
30543201 2019
12
A rare case of inguinal kimura disease. 38
30350814 2018
13
Computerized tomography features and clinicopathological analysis of Kimura disease in head and neck. 38
30186444 2018
14
Orbital Mass With Features of Both Kimura Disease and Immunoglobulin G4-Related Disease. 38
29923967 2018
15
Imaging of Benign Neoplastic and Nonneoplastic Salivary Gland Tumors. 38
29622111 2018
16
Kimura Disease: A differential diagnosis in a nephrotic child. 38
30302182 2018
17
Kimura Disease: A Rare Case Report From Himalayan Region. 38
29238694 2017
18
Kimura disease of buccal region in a pediatric patient with nephrotic syndrome: A case report. 38
29310412 2017
19
Successful therapy with mycophenolic acid in a membranous glomerulonephritis due to Kimura disease
. 38
28853699 2017
20
Kimura disease. 38
29469792 2017
21
Basophils are increased and express increased levels of interleukin-4 in the parotid lesions of Kimura disease. 38
29094020 2017
22
Treatment of Kimura disease with mycophenolate mofetil monotherapy. 38
28932783 2017
23
A Rare Case of Kimura Disease with Bilateral Parotid Involvement. 38
28946727 2017
24
Expression of the interleukin-21 and phosphorylated extracellular signal regulated kinase 1/2 in Kimura disease. 38
28108473 2017
25
Recurrent Kimura Disease of the Inner Canthus With No Lymphadenopathy. 38
26020715 2017
26
Superficial Parotidectomy Plane for Debulking Surgery in Kimura Disease. 38
28468187 2017
27
Acute Limb Ischemia and Coronary Artery Disease in a Case of Kimura's Disease. 38
28382271 2017
28
[An indolent and fluctuating subcutaneous mass of the skull in a 5-year-old patient: Diagnostic approach and difficulties]. 38
28233718 2017
29
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. 38
29279552 2017
30
Detailed Imaging Findings in a Rare Case of Kimura Disease, with Special Mention on Diffusion Weighted Imaging. 38
29657623 2017
31
Kimura disease in a Maori man with nephrotic syndrome. 38
27981775 2016
32
Concurrent Kimura disease and lupus nephritis: A case report. 38
27741124 2016
33
Eosinophilia and multiple lymphadenopathy: Kimura disease, a rare, but benign condition. 38
27581232 2016
34
Kimura Disease of the Breast - A Previously Undescribed Entity. 38
27058987 2016
35
Human Polyomavirus-6 Infecting Lymph Nodes of a Patient With an Angiolymphoid Hyperplasia With Eosinophilia or Kimura Disease. 38
26962076 2016
36
Kimura Disease Presenting as Forehead Soft Mass. 38
27100647 2016
37
A case of refractory Kimura disease with a buccal bulky mass successfully treated with low-dose cyclosporine A: report and review of the literature. 38
26666475 2016
38
Uncommon clinical presentation of KimuraÂ’s disease as bilateral retroauricular masses in a young Malian male: successful surgical approach. 38
27373132 2016
39
Synchronous Kimura lesions at two different sites-a diagnostic dilemma! 38
27190775 2016
40
Kimura disease: No age or ethnicity limit. 38
26905356 2016
41
Epithelioid Hemangioma (Angiolymphoid Hyperplasia With Eosinophilia) of the Heart With Peripheral Eosinophilia and Nephrotic Syndrome. 38
26338719 2016
42
Comparison of Local Recurrence Rate of Three Treatment Modalities for Kimura Disease. 38
26674917 2016
43
An Unusual Clinical Presentation of Kimura Disease Manifesting with a Typical Cephalocervical Lesion and an Atypical Subcutaneous Hip Mass Lesion. 38
27086824 2016
44
Kimura disease: A rare case of bilateral infra-auricular masses. 38
26831431 2015
45
Kimura Disease Mimicking an Aneurysm of the Radial Artery. 38
26362092 2015
46
Unusual Cause of Swelling in the Upper Limb: Kimura Disease. 38
26421119 2015
47
Kimura disease associated with severe visual dysfunction due to remarkable periorbital involvement. 38
26031497 2015
48
Angiolymphoid Hyperplasia with Eosinophilia of the Orbit and Adnexa. 38
27171790 2015
49
Extranodal hematopoietic neoplasms and mimics in the head and neck: an update. 38
26118762 2015
50
Pulmonary embolism as the initial clinical presentation of Kimura disease: case report and literature review. 38
25699606 2015
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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
PEDF Induced Signaling 64
Show member pathways
 13.27 TSLP IL5 IL4 CCR3 CCL5 CCL11
2
Akt Signaling 64
Show member pathways
 13.08 IL5 IL4 CCR3 CCL5 CCL11
3
Cytokine Signaling in Immune system 66
Show member pathways
 13.03 IL5 IL4 CCL5 CCL11
4
PAK Pathway 64
Show member pathways
 12.97 IL5 IL4 CCR3 CCL5 CCL11
5
Allograft rejection 37 1
Show member pathways
 12.59 RNASE3 IL5 IL4 CCL11
6
JAK-STAT signaling pathway (KEGG) 37
Show member pathways
 12.14 TSLP IL5 IL4
7
Immune response CCR3 signaling in eosinophils 22
Show member pathways
 12.09 CCR3 CCL5 CCL11
8
IL-17 Family Signaling Pathways 65
Show member pathways
 12.07 IL5 IL4 CCL11
9
Chemokine Superfamily Pathway: Human/Mouse Ligand-Receptor Interactions 65
Show member pathways
 11.99 CCR3 CCL5 CCL11
10
Immune response IFN gamma signaling pathway 22
Show member pathways
 11.75 IL5 IL4 CCL5 CCL11
11
Hematopoietic Stem Cell Differentiation Pathways and Lineage-specific Markers 65
11.74 IL5 IL4 CCR3
12
Glucocorticoid receptor regulatory network 1
11.54 IL5 IL4
13
Calcineurin-regulated NFAT-dependent transcription in lymphocytes 1
Show member pathways
 11.5 IL5 IL4
14
Innate Lymphoid Cell Differentiation Pathways 65
11.48 TSLP IL5 IL4
15
Microglia Activation During Neuroinflammation: Microglia Polarization 65
11.4 IL4 CCL5 CCL11
16
Interleukin-10 signaling 66
11.3 IL4 CCL5
17
IL4-mediated signaling events 1
11.3 IL5 IL4 CCL11
18
Thymic Stromal LymphoPoietin (TSLP) Signaling Pathway 1
11.28 TSLP CCL11
19
Inflammatory Response Pathway 1
11.11 IL5 IL4
20
Th2 Differentiation Pathway 65
11.1 IL5 IL4 CCL11
21
Cytokines and Inflammatory Response 1
11.05 IL5 IL4
22
OX40 Pathway 64
10.99 IL5 IL4
23
Lung fibrosis 1
10.82 IL5 IL4 CCR3 CCL5 CCL11
24
G-protein signaling_Rap2B regulation pathway 22
10.73 IL5 IL4 CCL5 CCL11
25
G-protein signaling_RhoB regulation pathway 22
10.69 IL5 IL4 CCL5 CCL11
26
Development and heterogeneity of the ILC family 1
10.64 TSLP IL5 IL4
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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 region GO:0005576 9.43 TSLP RNASE3 IL5 IL4 CCL5 CCL11
2 extracellular space GO:0005615 9.1 TSLP RNASE3 IL5 IL4 CCL5 CCL11

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 inflammatory response GO:0006954 9.62 IL5 CCR3 CCL5 CCL11
2 chemotaxis GO:0006935 9.58 CCR3 CCL5 CCL11
3 cellular response to interleukin-1 GO:0071347 9.55 CCL5 CCL11
4 positive regulation of endothelial cell proliferation GO:0001938 9.54 CCR3 CCL11
5 positive regulation of T cell proliferation GO:0042102 9.52 IL4 CCL5
6 monocyte chemotaxis GO:0002548 9.51 CCL5 CCL11
7 positive regulation of B cell proliferation GO:0030890 9.49 IL5 IL4
8 lymphocyte chemotaxis GO:0048247 9.48 CCL5 CCL11
9 positive regulation of JAK-STAT cascade GO:0046427 9.46 IL5 CCL5
10 eosinophil chemotaxis GO:0048245 9.43 CCL5 CCL11
11 chemokine-mediated signaling pathway GO:0070098 9.43 CCR3 CCL5 CCL11
12 positive regulation of interleukin-13 production GO:0032736 9.37 TSLP IL4
13 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.33 TSLP IL4 CCL5
14 cytokine-mediated signaling pathway GO:0019221 9.26 IL5 IL4 CCL5 CCL11
15 immune response GO:0006955 9.02 IL5 IL4 CCR3 CCL5 CCL11

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

# Name GO ID Score Top Affiliating Genes
1 chemokine activity GO:0008009 9.16 CCL5 CCL11
2 cytokine activity GO:0005125 9.02 TSLP IL5 IL4 CCL5 CCL11
3 CCR chemokine receptor binding GO:0048020 8.96 CCL5 CCL11
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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 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 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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