MCID: KMR001
MIFTS: 48

Kimura Disease

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

Aliases & Classifications for Kimura Disease

MalaCards integrated aliases for Kimura Disease:

Name: Kimura Disease 12 76 53 59 55 15 73
Angiolymphoid Hyperplasia with Eosinophilia 53
Eosinophilic Hyperplastic Lymphogranuloma 53
Eosinophilic Lymphofollicular Granuloma 53
Eosinophilic Granuloma of Soft Tissue 53
Eosinophilic Lymphofolliculosis 53
Eosinophilic Lymphogranuloma 59
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:



External Ids:

Disease Ontology 12 DOID:7365
MeSH 44 D000796
NCIt 50 C26867
Orphanet 59 ORPHA482
UMLS via Orphanet 74 C0033838
MESH via Orphanet 45 D000796
ICD10 via Orphanet 34 I89.8
SNOMED-CT via HPO 69 10890000
UMLS 73 C0033838

Summaries for Kimura Disease

NIH Rare Diseases : 53 Kimura disease is a rare, benign, chronic disorder that causes inflammation of tissue (nodules) under the skin of the head or neck.  These nodules tend to recur despite treatment. The cause of this condition is unknown, but may be due to an immune response.

MalaCards based summary : Kimura Disease, also known as angiolymphoid hyperplasia with eosinophilia, is related to loeffler endocarditis and cow milk allergy. 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 bone, and related phenotypes are eosinophilia and follicular hyperplasia

Wikipedia : 76 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 126)
# Related Disease Score Top Affiliating Genes
1 loeffler endocarditis 10.6 IL5 RNASE3
2 cow milk allergy 10.6 IL5 RNASE3
3 paragonimiasis 10.6 IL5 RNASE3
4 egg allergy 10.6 IL4 IL5
5 scleritis 10.6 IL4 IL5
6 trichinosis 10.6 IL4 IL5
7 apple allergy 10.5 IL4 IL5
8 cellulitis 10.5 IL5 RNASE3
9 sinusitis 10.5 IL5 RNASE3
10 non-suppurative otitis media 10.5 IL4 IL5
11 pemphigoid gestationis 10.5 CCL11 IL5
12 pollen allergy 10.5 IL5 RNASE3
13 cercarial dermatitis 10.5 IL4 IL5
14 chronic meningitis 10.5 CCL11 IL5
15 intestinal schistosomiasis 10.5 IL4 IL5
16 chronic tic disorder 10.5 IL4 IL5
17 toxic oil syndrome 10.5 IL4 IL5
18 primary amebic meningoencephalitis 10.5 IL4 IL5
19 strongyloidiasis 10.5 CCL11 IL5
20 wells syndrome 10.5 IL5 RNASE3
21 churg-strauss syndrome 10.5 IL5 RNASE3
22 allergic bronchopulmonary aspergillosis 10.5 IL4 IL5
23 enterobiasis 10.5 IL4 IL5
24 respiratory syncytial virus infectious disease 10.4 CCL5 IL5
25 papillary conjunctivitis 10.4 CCL11 IL4
26 maxillary sinusitis 10.4 CCL5 RNASE3
27 night blindness, congenital stationary, type 1a 10.4 CCL11 IL5
28 paranasal sinus disease 10.4 IL4 IL5
29 blepharitis 10.4 CCL11 IL4
30 radiculopathy 10.4 CCL5 IL5
31 ocular cicatricial pemphigoid 10.4 IL4 RNASE3
32 paracoccidioidomycosis 10.3 IL4 IL5
33 vernal conjunctivitis 10.3 IL4 RNASE3
34 vulvovaginitis 10.3 CCL11 CCL5
35 yellow fever 10.3 CCL5 IL5
36 genital herpes 10.3 CCL5 IL4
37 dermatitis herpetiformis 10.2 CCL11 IL4
38 cough variant asthma 10.2 IL4 IL5 RNASE3
39 inflammatory bowel disease 4 10.2 IL4 IL5 RNASE3
40 intrinsic asthma 10.2 IL4 IL5 RNASE3
41 atopic keratoconjunctivitis 10.2 IL4 IL5 RNASE3
42 cutaneous leishmaniasis 10.2 IL4 IL5
43 latex allergy 10.2 IL4 IL5 RNASE3
44 urinary schistosomiasis 10.2 IL4 IL5 RNASE3
45 milk allergy 10.2 IL4 IL5 RNASE3
46 parasitic helminthiasis infectious disease 10.2 IL4 IL5 RNASE3
47 food allergy 10.2 IL4 IL5 RNASE3
48 nephrotic syndrome 10.2
49 schistosomiasis 10.2 IL4 IL5 RNASE3
50 eosinophilic gastroenteritis 10.1 CCL11 IL5 RNASE3

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:

59 32 (show all 6)
# 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 ige level 59 32 hallmark (90%) Very frequent (99-80%) HP:0003212
4 lymphadenopathy 59 Frequent (79-30%)
5 abnormality of the salivary glands 59 Occasional (29-5%)
6 abnormal salivary gland morphology 32 occasional (7.5%) HP:0010286

MGI Mouse Phenotypes related to Kimura Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 hematopoietic system MP:0005397 9.7 IL4 IL5 TSLP CCL11 CCL5 CCR3
2 immune system MP:0005387 9.5 IL33 IL4 IL5 TSLP CCL11 CCL5
3 respiratory system MP:0005388 9.1 IL4 IL5 TSLP CCL11 CCR3 IL33

Drugs & Therapeutics for Kimura Disease

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Epithelioid Hemangioma of Bone And Soft Tissue Completed NCT03169595

Search NIH Clinical Center for Kimura Disease

Genetic Tests for Kimura Disease

Anatomical Context for Kimura Disease

MalaCards organs/tissues related to Kimura Disease:

41
Lymph Node, Skin, Bone, Salivary Gland, Colon, Brain, Kidney

Publications for Kimura Disease

Articles related to Kimura Disease:

(show top 50) (show all 106)
# Title Authors Year
1
Orbital Mass With Features of Both Kimura Disease and Immunoglobulin G4-Related Disease. ( 29923967 )
2018
2
Superficial Parotidectomy Plane for Debulking Surgery in Kimura Disease. ( 28106687 )
2017
3
Basophils are increased and express increased levels of interleukin-4 in the parotid lesions of Kimura disease. ( 29094020 )
2017
4
Treatment of Kimura disease with mycophenolate mofetil monotherapy. ( 28932783 )
2017
5
Kimura Disease: A Rare Case Report From Himalayan Region. ( 29238694 )
2017
6
Kimura disease. ( 29469792 )
2017
7
Kimura disease of buccal region in a pediatric patient with nephrotic syndrome: A case report. ( 29310412 )
2017
8
Superficial Parotidectomy Plane for Debulking Surgery in Kimura Disease. ( 28468187 )
2017
9
Detailed Imaging Findings in a Rare Case of Kimura Disease, with Special Mention on Diffusion Weighted Imaging. ( 29657623 )
2017
10
Expression of the interleukin-21 and phosphorylated extracellular signal regulated kinase 1/2 in Kimura disease. ( 28108473 )
2017
11
A Rare Case of Kimura Disease with Bilateral Parotid Involvement. ( 28946727 )
2017
12
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. ( 29279552 )
2017
13
An Unusual Clinical Presentation of Kimura Disease Manifesting with a Typical Cephalocervical Lesion and an Atypical Subcutaneous Hip Mass Lesion. ( 27086824 )
2016
14
Kimura disease in a Maori man with nephrotic syndrome. ( 27981775 )
2016
15
Eosinophilia and multiple lymphadenopathy: Kimura disease, a rare, but benign condition. ( 27581232 )
2016
16
Kimura disease as mimicker of lymphoma - A case report. ( 27728556 )
2016
17
Human Polyomavirus-6 Infecting Lymph Nodes of a Patient With an Angiolymphoid Hyperplasia With Eosinophilia or Kimura Disease. ( 26962076 )
2016
18
Kimura Disease of the Breast - A Previously Undescribed Entity. ( 27058987 )
2016
19
Kimura Disease Presenting as Forehead Soft Mass. ( 27100647 )
2016
20
A case of refractory Kimura disease with a buccal bulky mass successfullyA treated with low-dose cyclosporine A: Report andA reviewA ofA the literature. ( 26666475 )
2016
21
Concurrent Kimura disease and lupus nephritis: A case report. ( 27741124 )
2016
22
Comparison of Local Recurrence Rate of Three Treatment Modalities for Kimura Disease. ( 26674917 )
2016
23
Kimura disease. No age or ethnicity limit. ( 26905356 )
2016
24
Paradoxical brain embolism associated with Kimura disease mimics watershed infarction. ( 25447210 )
2015
25
Pulmonary embolism as the initial clinical presentation of Kimura disease: case report and literature review. ( 25699606 )
2015
26
Kimura disease associated with severe visual dysfunction due to remarkable periorbital involvement. ( 26031497 )
2015
27
Kimura disease: A rare case of bilateral infra-auricular masses. ( 26831431 )
2015
28
Unusual Cause of Swelling in the Upper Limb: Kimura Disease. ( 26421119 )
2015
29
Retrospective Multicentric Study of 25 Kimura Disease Patients: Emphasis on Therapeutics and Shared Features with Cutaneous IgG4-Related Disease. ( 26452023 )
2015
30
A mass under parotid gland: a patient with kimura disease. ( 25668110 )
2015
31
Recurrent Kimura Disease of the Inner Canthus With No Lymphadenopathy. ( 26020715 )
2015
32
Kimura Disease Presenting As an Eyelid Mass in a Young Asian Male. ( 26080258 )
2015
33
Kimura Disease Mimicking an Aneurysm of the Radial Artery. ( 26362092 )
2015
34
Kimura disease accompanied with nephrotic syndrome in a 45-year-old male. ( 25928198 )
2015
35
Kimura disease associated with IgA nephropathy. ( 24656163 )
2014
36
Kimura disease manifesting as synchronous bilateral parotid swelling in a young middle-eastern patient. ( 25544928 )
2014
37
Kimura Disease Simulating Hodgkin's Lymphoma on (18)F FDG PET-CT: Report of a Case. ( 26396638 )
2014
38
Preliminary study of diffusion-weighted imaging and magnetic resonance spectroscopy imaging in kimura disease. ( 25377973 )
2014
39
Kimura disease complicated with bowel infarction and multiple arterial thromboses in the extremities. ( 24356478 )
2014
40
Kimura Disease of the Epiglottis: A Case Report and Review of Literature. ( 23907346 )
2013
41
Kimura Disease: rare cause of a slowly progressive orbital mass. ( 24119195 )
2013
42
Kimura disease involving a caruncle. ( 23543681 )
2013
43
The false-positive finding of left pulmonary Kimura disease on 18F-FDG PET/CT. ( 23510880 )
2013
44
Intraparotid Kimura disease. ( 23021002 )
2013
45
Kimura disease: diagnostic challenges and clinical management. ( 21763034 )
2012
46
Treatment of Kimura disease with photodynamic therapy: a case study. ( 22369733 )
2012
47
Kimura disease: CT and MR imaging findings. ( 22173767 )
2012
48
Epitrochlear mass in a patient on maintenance hemodialysis--Kimura disease. ( 22280213 )
2012
49
Elevated numbers of cells producing interleukin-5 and interleukin-10 in a boy with Kimura disease. ( 22627370 )
2012
50
Microfilaria in lymph node mimicking Kimura disease. ( 23508372 )
2011

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 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.21 CCL11 CCL5 CCR3 IL33 IL4 IL5
2
Show member pathways
13.05 CCL11 CCL5 CCR3 IL4 IL5
3
Show member pathways
13 CCL11 CCL5 IL33 IL4 IL5
4
Show member pathways
12.94 CCL11 CCL5 CCR3 IL4 IL5
5
Show member pathways
12.57 CCL11 IL4 IL5 RNASE3
6
Show member pathways
12.36 IL4 IL5 TSLP
7
Show member pathways
12.08 CCL11 CCL5 CCR3
8
Show member pathways
12.06 CCL11 IL4 IL5
9
Show member pathways
11.72 CCL11 CCL5 IL4 IL5
10
Show member pathways
11.7 CCL11 CCL5 CCR3
11 11.67 CCR3 IL4 IL5
12 11.46 IL33 IL4 IL5 TSLP
13 11.34 CCL11 IL4 IL5
14 11.29 CCL5 IL4
15 11.26 CCL11 TSLP
16 11.12 CCL11 IL33 IL4 IL5
17 11.09 IL4 IL5
18 11.04 IL4 IL5
19 10.95 IL4 IL5
20 10.82 CCL11 CCL5 CCR3 IL4 IL5
21 10.76 IL33 IL4 IL5 TSLP

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 region GO:0005576 9.5 CCL11 CCL5 IL33 IL4 IL5 RNASE3
2 extracellular space GO:0005615 9.17 CCL11 CCL5 IL33 IL4 IL5 RNASE3

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

(show all 19)
# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.76 CCL11 CCL5 IL4 IL5
2 inflammatory response GO:0006954 9.71 CCL11 CCL5 CCR3 IL5
3 chemotaxis GO:0006935 9.65 CCL11 CCL5 CCR3
4 cytokine-mediated signaling pathway GO:0019221 9.62 CCL11 CCL5 IL4 IL5
5 neutrophil chemotaxis GO:0030593 9.58 CCL11 CCL5
6 positive regulation of tyrosine phosphorylation of STAT protein GO:0042531 9.58 CCL5 TSLP
7 positive regulation of T cell proliferation GO:0042102 9.57 CCL5 IL4
8 positive regulation of interleukin-6 production GO:0032755 9.56 IL33 TSLP
9 monocyte chemotaxis GO:0002548 9.55 CCL11 CCL5
10 positive regulation of B cell proliferation GO:0030890 9.54 IL4 IL5
11 lymphocyte chemotaxis GO:0048247 9.52 CCL11 CCL5
12 positive regulation of JAK-STAT cascade GO:0046427 9.51 CCL5 IL5
13 eosinophil chemotaxis GO:0048245 9.49 CCL11 CCL5
14 positive regulation of immunoglobulin secretion GO:0051024 9.43 IL33 IL5
15 positive regulation of interleukin-5 production GO:0032754 9.37 IL33 TSLP
16 chemokine-mediated signaling pathway GO:0070098 9.33 CCL11 CCL5 CCR3
17 type 2 immune response GO:0042092 9.26 IL33 IL4
18 positive regulation of interleukin-13 production GO:0032736 9.13 IL33 IL4 TSLP
19 regulation of signaling receptor activity GO:0010469 9.1 CCL11 CCL5 IL33 IL4 IL5 TSLP

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 CCL11 CCL5
2 cytokine activity GO:0005125 9.1 CCL11 CCL5 IL33 IL4 IL5 TSLP
3 CCR chemokine receptor binding GO:0048020 8.96 CCL11 CCL5

Sources for Kimura Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
Content
Loading form....