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WCD
MCID: NDL003
MIFTS: 51

Nodular Nonsuppurative Panniculitis (WCD)

Categories: Muscle diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes
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Aliases & Classifications for Nodular Nonsuppurative Panniculitis

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MalaCards integrated aliases for Nodular Nonsuppurative Panniculitis:

Name: Nodular Nonsuppurative Panniculitis 12 53 15
Weber-Christian Disease 12 53 59
Relapsing Febrile Nodular Nonsuppurative Panniculitis 12 59
Panniculitis, Nodular Nonsuppurative 44 73
Nodular Non-Suppurative Febrile Panniculitis 12
Relapsing Febrile Nodular Panniculitis 59
Nodular Non-Suppurative Panniculitis 59
Panniculitis Nodular Nonsuppurative 53
Pfeiffer-Weber-Christian Syndrome 59
Idiopathic Lobular Panniculitis 59
Idiopathic Nodular Panniculitis 59
Weber-Christian Panniculitis 59
Weber - Christian Disease 12
Weber Christian Disease 53
Weberchristian Disease 76
Wcd 59

Characteristics:

Orphanet epidemiological data:

59
nodular non-suppurative panniculitis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Skin diseases Muscle diseases
See all MalaCards categories (disease lists)
ICD10: 33 34
Orphanet: 59  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:1525
ICD10 33 M35.6
MeSH 44 D010201
SNOMED-CT 68 33760009
Orphanet 59 ORPHA33577
MESH via Orphanet 45 D010201
UMLS via Orphanet 74 C0030328
ICD10 via Orphanet 34 M35.6
UMLS 73 C0030328
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Summaries for Nodular Nonsuppurative Panniculitis

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NIH Rare Diseases : 53 Nodular nonsuppurative panniculitis describes a rare group of skin disorders characterized by tender, painful bumps below the surface of the skin (subcutaneous nodules) that usually lead to inflammation of the subcutaneous layer of fat (panniculitis). These nodules tend to be 1-2 centimeters in length and most often affect the legs and feet. In most people, this condition is associated with fever, a general feeling of ill health (malaise), muscle pain, and/or abdominal pain. These symptoms may subside after a few days or weeks and may recur weeks, months, or years later. The exact cause of nodular nonsuppurative panniculitis is unknown.

MalaCards based summary : Nodular Nonsuppurative Panniculitis, also known as weber-christian disease, is related to localized lipodystrophy and panniculitis. An important gene associated with Nodular Nonsuppurative Panniculitis is SERPINA1 (Serpin Family A Member 1), and among its related pathways/superpathways are Innate Immune System and Complement and coagulation cascades. Affiliated tissues include skin, eye and lung, and related phenotypes are nausea and vomiting and splenomegaly

Wikipedia : 76 Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue (the... more...

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Related Diseases for Nodular Nonsuppurative Panniculitis

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Diseases related to Nodular Nonsuppurative Panniculitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 72)
# Related Disease Score Top Affiliating Genes
1 localized lipodystrophy 11.5
2 panniculitis 10.8
3 mental retardation, skeletal dysplasia, and abducens palsy 10.4
4 glomerulonephritis 10.4
5 phacolytic glaucoma 10.2 CRYAA SERPINA1
6 immune-complex glomerulonephritis 10.2
7 alpha-1-antitrypsin deficiency 10.2
8 exophthalmos 10.2
9 hepatic lipase deficiency 10.2 LIPC PNLIP
10 familial lipoprotein lipase deficiency 10.2 LIPC PNLIP
11 biliary cirrhosis, primary, 1 10.1
12 rheumatic fever-related antigen 10.1
13 parkes weber syndrome 10.1
14 diabetes mellitus 10.1
15 primary biliary cirrhosis 10.1
16 uveitis 10.1
17 rheumatic fever 10.1
18 thyroiditis 10.1
19 amyloidosis 10.1
20 amyloidosis aa 10.1
21 primary biliary cholangitis 10.1
22 weber syndrome 10.1
23 sella turcica neoplasm 10.1 F8 POMC
24 acute poststreptococcal glomerulonephritis 10.1 CFP SERPINF2
25 rubeosis iridis 10.1 CRYAA SERPINF2 SNRPE
26 tuberculum sellae meningioma 10.1 F8 POMC
27 mastitis 10.1
28 nephrotic syndrome 10.1
29 granulomatous mastitis 10.1
30 hidradenitis suppurativa 10.1
31 hidradenitis 10.1
32 hemorrhagic disease 10.1 F8 SERPINA1 SERPINF2
33 cardiac tamponade 10.0 F8 TNFRSF1A
34 congenital granular cell tumor 10.0 LYZ SERPINA1
35 adult xanthogranuloma 10.0 LYZ SERPINA1
36 uveoparotid fever 10.0 LYZ POMC
37 diffuse glomerulonephritis 10.0 CFP LYZ
38 rheumatoid arthritis 10.0
39 reticulum cell sarcoma 10.0
40 focal segmental glomerulosclerosis 1 10.0
41 myelodysplastic syndrome 10.0
42 arthritis 10.0
43 focal segmental glomerulosclerosis 10.0
44 hemolytic anemia 10.0
45 liver disease 10.0
46 lymphoma 10.0
47 portal hypertension 10.0
48 filariasis 10.0
49 sarcoma 10.0
50 amenorrhea 10.0

Graphical network of the top 20 diseases related to Nodular Nonsuppurative Panniculitis:



Diseases related to Nodular Nonsuppurative Panniculitis
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Symptoms & Phenotypes for Nodular Nonsuppurative Panniculitis

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Human phenotypes related to Nodular Nonsuppurative Panniculitis:

59 32 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nausea and vomiting 59 32 hallmark (90%) Very frequent (99-80%) HP:0002017
2 splenomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0001744
3 hepatomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0002240
4 fever 59 32 hallmark (90%) Very frequent (99-80%) HP:0001945
5 subcutaneous nodule 59 32 hallmark (90%) Very frequent (99-80%) HP:0001482
6 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
7 abdominal pain 59 32 hallmark (90%) Very frequent (99-80%) HP:0002027
8 edema 59 32 hallmark (90%) Very frequent (99-80%) HP:0000969
9 weight loss 59 32 hallmark (90%) Very frequent (99-80%) HP:0001824
10 autoimmunity 59 32 occasional (7.5%) Occasional (29-5%) HP:0002960
11 myalgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0003326
12 aplasia/hypoplasia of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0008065
13 erythema 59 32 hallmark (90%) Very frequent (99-80%) HP:0010783
14 inflammatory abnormality of the eye 59 32 occasional (7.5%) Occasional (29-5%) HP:0100533
15 panniculitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0012490

GenomeRNAi Phenotypes related to Nodular Nonsuppurative Panniculitis according to GeneCards Suite gene sharing:

26 (show all 12)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00106-A-0 10.15 SNRPE TNFRSF1A
2 Decreased viability GR00221-A-1 10.15 IRS1
3 Decreased viability GR00221-A-2 10.15 IRS1
4 Decreased viability GR00221-A-3 10.15 RASA1
5 Decreased viability GR00221-A-4 10.15 RASA1
6 Decreased viability GR00240-S-1 10.15 PPIA
7 Decreased viability GR00342-S-2 10.15 IRS1
8 Decreased viability GR00342-S-3 10.15 IRS1
9 Decreased viability GR00381-A-1 10.15 RASA1 SERPINF2
10 Decreased viability GR00381-A-2 10.15 SERPINF2
11 Decreased viability GR00402-S-2 10.15 CFP CRYAA ELANE F8 IRS1 KLK4
12 no effect GR00402-S-1 9.6 CFP CRYAA ELANE F8 IRS1 KLK4

MGI Mouse Phenotypes related to Nodular Nonsuppurative Panniculitis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.1 CFP ELANE F8 IRS1 KLK4 LIPC
2 cardiovascular system MP:0005385 10.02 CFP IRS1 LYZ POMC PPIA RASA1
3 immune system MP:0005387 9.93 CFP ELANE F8 IRS1 LYZ POMC
4 endocrine/exocrine gland MP:0005379 9.92 IRS1 LIPC LYZ POMC PPIA SLC2A4
5 adipose tissue MP:0005375 9.88 IRS1 LYZ PNLIP POMC SLC2A4 TNFRSF1A
6 liver/biliary system MP:0005370 9.56 IRS1 LYZ PNLIP POMC PPIA SLC2A4
7 mortality/aging MP:0010768 9.4 CFP ELANE F8 IRS1 KLK4 LYZ
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Drugs & Therapeutics for Nodular Nonsuppurative Panniculitis

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Search Clinical Trials , NIH Clinical Center for Nodular Nonsuppurative Panniculitis

Cochrane evidence based reviews: panniculitis, nodular nonsuppurative

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Genetic Tests for Nodular Nonsuppurative Panniculitis

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Anatomical Context for Nodular Nonsuppurative Panniculitis

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MalaCards organs/tissues related to Nodular Nonsuppurative Panniculitis:

41
Skin, Eye, Lung, Liver, Thyroid, Pituitary, Pancreas
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Publications for Nodular Nonsuppurative Panniculitis

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Articles related to Nodular Nonsuppurative Panniculitis:

(show top 50) (show all 166)
# Title Authors Year
1
Weber-Christian disease presenting with lung nodules dramatically improved with corticosteroid therapy: one case report and literature review. ( 26224252 )
2018
2
Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease. ( 28352826 )
2016
3
Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature. ( 25325705 )
2016
4
Pfeifer-Weber-Christian Disease during Pregnancy Successfully Treated with Corticosteroids. ( 27688468 )
2016
5
Nephrotic syndrome in a patient with Pfeifer-Weber-Christian disease. ( 25543272 )
2015
6
Diagnosis of extensive myocardial, pericardial, and coronary calcification in Weber-Christian disease. ( 25925218 )
2015
7
Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. ( 25954116 )
2015
8
Fat-Removal Orbital Decompressing for Severe Bilateral Proptosis due to Weber-Christian Disease. ( 25978784 )
2015
9
Skin lesions depicting a systemic disease. Weber-Christian disease. ( 21325702 )
2011
10
A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. ( 20105325 )
2010
11
Chorioretinitis associated with weber-christian disease. ( 25391077 )
2009
12
Weber and Christian of Weber-Christian disease. ( 18401882 )
2008
13
Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. ( 16550303 )
2007
14
Childhood Weber-Christian disease: clinical investigation and virus detection. ( 17888054 )
2007
15
A case of Weber-Christian disease with collapsing glomerulopathy. ( 16931223 )
2006
16
Panniculitis and liver disease (hepatic Weber Christian disease). ( 15629524 )
2005
17
Successful treatment of steroid-resistant Weber-Christian disease with biliary ductopenia using cyclosporin A. ( 15757971 )
2005
18
Staphylococcus cohnii as a cause of multiple brain abscesses in Weber-Christian disease. ( 16054652 )
2005
19
Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. ( 12745859 )
2003
20
Weber-Christian disease presenting with ocular manifestations. ( 14576996 )
2003
21
Weber Christian disease. ( 14621048 )
2003
22
Successful treatment of idiopathic febrile panniculitis (Weber-Christian disease) with thalidomide in a patient having failed multiple other medical therapies. ( 17041381 )
2002
23
Weber-Christian disease with severe polyarthritis and polyosteitis. ( 12022332 )
2002
24
The men behind the eponym: Frederick Parkes Weber and Henry a. Christian: Weber-Christian disease. ( 12454605 )
2002
25
Hepatic Weber-Christian disease. ( 11296691 )
2001
26
Osteoarthropathy associated with Weber-Christian disease. ( 11550989 )
2001
27
Weber Christian disease in a type 2 diabetic patient. ( 11584948 )
2001
28
Weber-Christian disease presenting with proptosis: a case report. ( 10803707 )
2000
29
Systemic Weber-Christian disease complicated by partial transverse myelopathy. ( 11070575 )
2000
30
Systemic Weber-Christian disease. ( 11179936 )
2000
31
Steroid-resistant Weber-Christian Disease. ( 10435355 )
1999
32
Central serous retinopathy associated with Weber-Christian disease. ( 10435428 )
1999
33
Weber-Christian disease associated with lupus anticoagulant and anticardiolipin antibodies. ( 10501442 )
1999
34
Treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. ( 9738797 )
1998
35
Proptosis as the manifesting sign of Weber-Christian disease. ( 9222254 )
1997
36
Nodular nonsuppurative panniculitis in association with primary biliary cirrhosis and Hashimoto's thyroiditis. ( 8686385 )
1996
37
Weber-Christian disease with calcinosis: a case report. ( 8973879 )
1996
38
Recognizing Weber-Christian disease. ( 8987390 )
1996
39
Subarachnoid hemorrhage of unknown origin associated with Weber-Christian disease--case report. ( 7477690 )
1995
40
Weber-Christian disease with benign cytophagic histiocytes in the skin lesion. ( 7754752 )
1995
41
Glucocorticoid-unresponsive fever in a patient with Weber-Christian disease. ( 8031696 )
1994
42
Mammary calcifications in Weber-Christian disease. ( 8168588 )
1994
43
Acalculous cholecystitis (panniculitis) associated with Weber-Christian disease. ( 8439847 )
1993
44
Cultivation of Borrelia burgdorferi from the blood and a subcutaneous lesion of a patient with relapsing febrile nodular nonsuppurative panniculitis. ( 1538171 )
1992
45
Myalgia as the major symptom in systemic panniculitis (Weber-Christian disease). ( 1490498 )
1992
46
Cardiac manifestations of Weber-Christian disease: report and review of the literature. ( 1865427 )
1991
47
Weber-Christian disease. An unusual occurrence in a 12-year-old child. ( 2361793 )
1990
48
Weber-Christian disease producing splenic vein occlusion and bleeding gastric varices: successful treatment with sclerotherapy. ( 2385562 )
1990
49
Systemic Weber-Christian disease with reversible bilateral ureteric obstruction. ( 2608584 )
1989
50
Dyslipoproteinemia and an inhibitor of lipolytic enzymes in Weber-Christian disease. ( 2739146 )
1989
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Variations for Nodular Nonsuppurative Panniculitis

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Expression for Nodular Nonsuppurative Panniculitis

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Search GEO for disease gene expression data for Nodular Nonsuppurative Panniculitis.
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Pathways for Nodular Nonsuppurative Panniculitis

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Pathways related to Nodular Nonsuppurative Panniculitis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Innate Immune System 66
Show member pathways
 13.7 CFP ELANE IRS1 LYZ POMC PPIA
2
Complement and coagulation cascades 1 37
11.69 F8 SERPINA1 SERPINF2
3
Insulin resistance 37
Show member pathways
 11.47 IRS1 SLC2A4 TNFRSF1A
4
Adipocytokine signaling pathway 37
10.76 IRS1 POMC SLC2A4 TNFRSF1A
5
Transcription factor regulation in adipogenesis 1
10.66 IRS1 SLC2A4
6
Insulin signalling in human adipocytes (normal condition) 1
Show member pathways
 10.36 IRS1 SLC2A4
7
triacylglycerol degradation 1
10 LIPC PNLIP
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GO Terms for Nodular Nonsuppurative Panniculitis

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Cellular components related to Nodular Nonsuppurative Panniculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.73 CFP ELANE F8 KLK4 LIPC LYZ
2 endoplasmic reticulum lumen GO:0005788 9.67 CFP F8 LIPC SERPINA1
3 collagen-containing extracellular matrix GO:0062023 9.62 CFP ELANE SERPINA1 SERPINF2
4 secretory granule GO:0030141 9.58 ELANE KLK4 POMC
5 platelet alpha granule lumen GO:0031093 9.43 F8 SERPINA1 SERPINF2
6 extracellular space GO:0005615 9.36 CFP ELANE F8 LIPC LYZ PNLIP
7 specific granule lumen GO:0035580 9.33 CFP ELANE LYZ

Biological processes related to Nodular Nonsuppurative Panniculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 platelet degranulation GO:0002576 9.5 F8 SERPINA1 SERPINF2
2 defense response to bacterium GO:0042742 9.46 CFP ELANE LYZ TNFRSF1A
3 glucose homeostasis GO:0042593 9.43 IRS1 POMC SLC2A4
4 neutrophil degranulation GO:0043312 9.35 CFP ELANE LYZ PPIA SERPINA1
5 acute-phase response GO:0006953 8.8 F8 SERPINA1 SERPINF2

Molecular functions related to Nodular Nonsuppurative Panniculitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 triglyceride lipase activity GO:0004806 9.16 LIPC PNLIP
2 lipase activity GO:0016298 8.96 LIPC PNLIP
3 protease binding GO:0002020 8.8 ELANE SERPINA1 SERPINF2
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Sources for Nodular Nonsuppurative Panniculitis

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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
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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