MCID: KMR001
MIFTS: 40

Kimura Disease

Categories: Bone diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Kimura Disease

MalaCards integrated aliases for Kimura Disease:

Name: Kimura Disease 12 74 20 58 54 15 17 71
Eosinophilic Lymphogranuloma 20 58
Eosinophilic Hyperplastic Lymphogranuloma 20
Eosinophilic Lymphofollicular Granuloma 20
Eosinophilic Granuloma of Soft Tissue 20
Eosinophilic Lymphofolliculosis 20
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:

Orphanet: 58  
Rare systemic and rhumatological diseases


External Ids:

Disease Ontology 12 DOID:7365
MeSH 44 D000796
NCIt 50 C26867
SNOMED-CT 67 399894006
MESH via Orphanet 45 D000796
ICD10 via Orphanet 33 I89.8
UMLS via Orphanet 72 C0033838
Orphanet 58 ORPHA482
SNOMED-CT via HPO 68 10890000
UMLS 71 C0033838

Summaries for Kimura Disease

GARD : 20 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 igg4-related disease and histiocytoid hemangioma. An important gene associated with Kimura Disease is IL5 (Interleukin 5), and among its related pathways/superpathways are Cytokine Signaling in Immune system and Pathways in cancer. Affiliated tissues include salivary gland, lymph node and colon, 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...

Related Diseases for Kimura Disease

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

(show top 50) (show all 328)
# Related Disease Score Top Affiliating Genes
1 igg4-related disease 30.3 PLA2R1 IL4
2 histiocytoid hemangioma 30.1 THSD7A IL5
3 acute asthma 30.1 RNASE3 IL5 IL4
4 disseminated eosinophilic collagen disease 30.0 RNASE3 IL5 IFNA1
5 cytokine deficiency 29.9 IL5 IL13
6 hypereosinophilic syndrome 29.8 RNASE3 IL5 IL4 IL13 IGHE IGES
7 mycosis fungoides 29.8 IL5 IFNA1 CD1A
8 otitis media 29.7 RNASE3 IL5 IL4 IGHE
9 autoimmune disease 29.7 IL5 IL4 IL13 IFNA1
10 exophthalmos 29.7 CD1E CD1A
11 mikulicz disease 29.7 IL5 IL4 IL13
12 lung disease 29.6 RNASE3 IL5 IL4 IL13
13 mastocytosis 29.5 IL4 IL13 IFNA1
14 pneumonia 29.4 IL5 IL4 IL13 IFNA1
15 folliculitis 29.4 IL5 IL4 CD1E CD1A
16 behcet syndrome 29.3 IL5 IL4 IL13 IFNA1
17 chronic urticaria 29.3 RNASE3 IL4 IL13 IGHE
18 dermatitis 29.1 RNASE3 IL5 IL4 IL13 IGHE IGES
19 rhinitis 29.1 RNASE3 IL5 IL4 IL13 IGHE
20 urticaria 29.1 RNASE3 IL5 IL4 IL13 IGHE
21 dermatitis, atopic 28.8 RNASE3 IL5 IL4 IL13 IGHE IGES
22 allergic rhinitis 28.8 RNASE3 IL5 IL4 IL13 IGHE IGES
23 leprosy 3 28.7 IL5 IL4 IL13 CD1E CD1A
24 nephrotic syndrome 10.5
25 loeffler endocarditis 10.3 RNASE3 IL5
26 cow milk allergy 10.3 IL5 IL4
27 patulous eustachian tube 10.3 RNASE3 IL5
28 pemphigoid gestationis 10.3 RNASE3 IL5
29 proteinuria, chronic benign 10.3
30 glomerulonephritis 10.3
31 wells syndrome 10.3 RNASE3 IL5
32 giant papillary conjunctivitis 10.2 RNASE3 IL4
33 chronic eosinophilic pneumonia 10.2 RNASE3 IL5
34 ocular cicatricial pemphigoid 10.2 RNASE3 IL4
35 scleritis 10.2 IL5 IL4
36 anisakiasis 10.2 RNASE3 IGHE
37 status asthmaticus 10.2 RNASE3 IL5
38 vasculitis 10.2
39 opisthorchiasis 10.2 IL4 IGES
40 erythema multiforme 10.2 IL5 IL4
41 endomyocardial fibrosis 10.2 RNASE3 IL5 IL4
42 eosinophilic gastroenteritis 10.2 RNASE3 IL5 IL4
43 autoimmune disease of blood 10.2 IL5 IL4
44 neuromyelitis optica 10.1 IL5 IL4 IFNA1
45 cellulitis 10.1 RNASE3 IL5 IGES
46 sezary's disease 10.1 IL5 IL4 IFNA1
47 toxic oil syndrome 10.1 IL5 IL4 IGHE
48 chronic mucocutaneous candidiasis 10.1 IL5 IL4 IFNA1
49 membranous nephropathy 10.1
50 lymphadenitis 10.1

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



Diseases related to Kimura Disease

Symptoms & Phenotypes for Kimura Disease

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%)

Drugs & Therapeutics for Kimura Disease

Search Clinical Trials , NIH Clinical Center for Kimura Disease

Genetic Tests for Kimura Disease

Anatomical Context for Kimura Disease

MalaCards organs/tissues related to Kimura Disease:

40
Salivary Gland, Lymph Node, Colon, T Cells

Publications for Kimura Disease

Articles related to Kimura Disease:

(show top 50) (show all 218)
# Title Authors PMID Year
1
Kimura disease: review of the literature. 20 61
18808562 2008
2
Comparison of the efficacy of different treatment modalities for Kimura's disease. 20
27614905 2017
3
Thrombotic storm in Kimura disease. 54 61
19468829 2010
4
Kimura's disease with eosinophilic panniculitis--treated with cyclosporine: a case report. 61 54
20236545 2010
5
The diagnosis of a giant cardiac malignant lymphoma in the right ventricle: a case report. 61
33529498 2021
6
Kimura disease in larynx: big lesion with soft symptoms. 61
33462061 2021
7
Recurrent Kimura Disease: Original Observations in 2 Patients With an Unusual Protracted Course of the Inflammatory Process. 61
31651639 2020
8
Reactive Eosinophil Proliferations in Tissue and the Lymphocytic Variant of Hypereosinophilic Syndrome. 61
33367482 2020
9
Warthin-Finkeldey giant cells-A useful clue for the cytodiagnosis of Kimura disease: A report of three cases. 61
33175471 2020
10
Kimura Disease: A Case Series and Systematic Review of Clinico-radiological Features. 61
33250297 2020
11
Kimura disease of the breast: Case report and literature review of current management. 61
32815218 2020
12
Kimura Disease Associated with Minimal Change Disease. 61
33006549 2020
13
Kimura disease with a history of Budd-Chiari syndrome: a case report. 61
32868502 2020
14
A case of IgG4-related disease presented with Kimura disease-like skin eruption, rheumatoid arthritis-like abnormality and interstitial pneumonia. 61
32189363 2020
15
Steroid-dependent Kimura Disease in a Child Treated with Cetirizine and Montelukast. 61
32844779 2020
16
Human Polyomavirus 6 with the Asian-Japanese Genotype in Cases of Kimura Disease and Angiolymphoid Hyperplasia with Eosinophilia. 61
31981580 2020
17
Kimura Disease Presenting with Buccal Mass: A Case Report and Literature Review. 61
32712881 2020
18
An Unusual Presentation of Recurrent Childhood Kimura Disease: Ileocecal Involvement. 61
32590418 2020
19
Groin abnormalities: ultrasonographic and clinical findings. 61
31786905 2020
20
A Rare Case of Kimura Disease. 61
32180608 2020
21
Kimura Disease with Parotid Swelling and Cervical Lymphadenopathy: A Case Report and Literature Review. 61
32733768 2020
22
Remission of Kimura Disease With Carotid Hypervascularization After Cyclosporine Treatment. 61
32363093 2020
23
Dyspnea and Cough: A Rare Report of the Kimura Disease. 61
32133969 2020
24
Diagnostic dilemma of kimura disease of eyelids. 61
32008028 2020
25
Buccal region's Kimura disease in a pediatric patient: A case report and review of the literatture. 61
32698293 2020
26
Radiomics Based on CECT in Differentiating Kimura Disease From Lymph Node Metastases in Head and Neck: A Non-Invasive and Reliable Method. 61
32850321 2020
27
Tissue-infiltrating immune cells contribute to understanding the pathogenesis of Kimura disease: A case report. 61
31852109 2019
28
Kimura disease in a 2-year-old boy with generalized lymphadenopathy. 61
31833620 2019
29
The clinicopathological characteristics of Kimura disease in Chinese patients. 61
31440918 2019
30
Kimura disease: a case report of a rare illness presenting as a common complaint. 61
30808157 2019
31
Mepolizumab as an effective treatment for Kimura's disease associated with ulcerative colitis: A case report. 61
31681687 2019
32
Kimura disease with nephrotic syndrome in a child- A rare association. 61
31361234 2019
33
Kimura disease: case report and brief review of literature. 61
31086850 2019
34
Angiolymphoid hyperplasia with eosinophilia and Kimura disease overlap, with evidence of diffuse visceral involvement. 61
30362278 2019
35
Kimura disease and immunoglobulin G4-related disease: a possible overlap in a patient with severe systemic symptoms. 61
30543201 2019
36
An unexpected asymptomatic epiglottal site of Kimura disease. 61
30712651 2019
37
Concurrent Kidney Glomerular and Interstitial Lesions Associated with Kimura's Disease. 61
31387106 2019
38
A rare case of inguinal kimura disease. 61
30350814 2018
39
Computerized tomography features and clinicopathological analysis of Kimura disease in head and neck. 61
30186444 2018
40
Orbital Mass With Features of Both Kimura Disease and Immunoglobulin G4-Related Disease. 61
29923967 2018
41
Imaging of Benign Neoplastic and Nonneoplastic Salivary Gland Tumors. 61
29622111 2018
42
Kimura Disease: A differential diagnosis in a nephrotic child. 61
30302182 2018
43
Kimura Disease: A Rare Case Report From Himalayan Region. 61
29238694 2017
44
Kimura disease of buccal region in a pediatric patient with nephrotic syndrome: A case report. 61
29310412 2017
45
Successful therapy with mycophenolic acid in a membranous glomerulonephritis due to Kimura diseaseā€©. 61
28853699 2017
46
Basophils are increased and express increased levels of interleukin-4 in the parotid lesions of Kimura disease. 61
29094020 2017
47
Kimura disease. 61
29469792 2017
48
Treatment of Kimura disease with mycophenolate mofetil monotherapy. 61
28932783 2017
49
A Rare Case of Kimura Disease with Bilateral Parotid Involvement. 61
28946727 2017
50
Expression of the interleukin-21 and phosphorylated extracellular signal regulated kinase 1/2 in Kimura disease. 61
28108473 2017

Variations for Kimura Disease

Expression for Kimura Disease

Search GEO for disease gene expression data for Kimura Disease.

Pathways for Kimura Disease

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

(show all 19)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.09 IL5 IL4 IL13 IGHE IFNA1
2 12.58 IL5 IL4 IL13 IFNA1
3
Show member pathways
12.54 RNASE3 IL5 IL4 IL13 IFNA1
4
Show member pathways
12.36 IL5 IL4 IL13 IGHE
5
Show member pathways
12.18 IL5 IL4 IL13 IFNA1
6
Show member pathways
12.09 IL5 IL4 IL13
7
Show member pathways
12.05 IL5 IL4 IL13 IFNA1
8 11.78 IL4 IL13 IGHE
9 11.6 IL5 IL4 IL13
10 11.57 IL5 IL4 IL13
11 11.43 IL5 IL4 IL13
12 11.41 IL5 IL4 IGHE
13 11.25 IL5 IL4 CD1E CD1A
14 11.21 IL5 IL4 IL13
15 11.09 IL5 IL4
16 11 IL5 IL4 IL13
17 10.9 IL5 IL4 IL13
18 10.75 IL5 IL4 IL13
19 9.88 IL5 IL4 IL13

GO Terms for Kimura Disease

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

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.5 RNASE3 IL5 IL4 IL13 IFNA1 CD1E
2 external side of plasma membrane GO:0009897 9.46 IL13 IGHE CD1E CD1A
3 extracellular region GO:0005576 9.23 THSD7A RNASE3 PLA2R1 IL5 IL4 IL13

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

(show all 15)
# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.78 IGHE IFNA1 CD1E CD1A
2 positive regulation of interleukin-10 production GO:0032733 9.55 IL4 IL13
3 negative regulation of endothelial cell apoptotic process GO:2000352 9.54 IL4 IL13
4 microglial cell activation GO:0001774 9.52 IL4 IL13
5 innate immune response in mucosa GO:0002227 9.51 RNASE3 IL4
6 positive regulation of T cell mediated cytotoxicity GO:0001916 9.49 CD1E CD1A
7 positive regulation of immunoglobulin production GO:0002639 9.46 IL5 IL13
8 macrophage activation GO:0042116 9.43 IL4 IL13
9 positive regulation of mast cell degranulation GO:0043306 9.4 IL4 IL13
10 antigen processing and presentation, exogenous lipid antigen via MHC class Ib GO:0048007 9.37 CD1E CD1A
11 cytokine-mediated signaling pathway GO:0019221 9.35 IFNA1 IGHE IL13 IL4 IL5
12 positive regulation of B cell proliferation GO:0030890 9.33 IL5 IL4 IL13
13 antigen processing and presentation, endogenous lipid antigen via MHC class Ib GO:0048006 9.32 CD1E CD1A
14 negative regulation of complement-dependent cytotoxicity GO:1903660 9.26 IL4 IL13
15 immune response GO:0006955 9.1 CD1A CD1E IGHE IL13 IL4 IL5

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

# Name GO ID Score Top Affiliating Genes
1 cytokine activity GO:0005125 9.56 IL5 IL4 IL13 IFNA1
2 lipopeptide binding GO:0071723 9.32 CD1E CD1A
3 exogenous lipid antigen binding GO:0030884 9.16 CD1E CD1A
4 endogenous lipid antigen binding GO:0030883 8.96 CD1E CD1A
5 cytokine receptor binding GO:0005126 8.8 IL4 IL13 IFNA1

Sources for Kimura Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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