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MCID: NDL003
MIFTS: 48

Nodular Nonsuppurative Panniculitis

Categories: 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 54 15 17
Weber-Christian Disease 12 77 54 60
Relapsing Febrile Nodular Nonsuppurative Panniculitis 12 60
Panniculitis, Nodular Nonsuppurative 45 74
Nodular Non-Suppurative Febrile Panniculitis 12
Relapsing Febrile Nodular Panniculitis 60
Nodular Non-Suppurative Panniculitis 60
Panniculitis Nodular Nonsuppurative 54
Pfeiffer-Weber-Christian Syndrome 60
Idiopathic Lobular Panniculitis 60
Idiopathic Nodular Panniculitis 60
Weber-Christian Panniculitis 60
Weber - Christian Disease 12
Weber Christian Disease 54
Wcd 60

Characteristics:

Orphanet epidemiological data:

60
nodular non-suppurative panniculitis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

MalaCards categories:
Global: Rare diseases
Anatomical: Skin diseases
See all MalaCards categories (disease lists)
ICD10: 34 35
Orphanet: 60  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:1525
MeSH 45 D010201
SNOMED-CT 69 33760009
ICD10 34 M35.6
MESH via Orphanet 46 D010201
ICD10 via Orphanet 35 M35.6
UMLS via Orphanet 75 C0030328
Orphanet 60 ORPHA33577
UMLS 74 C0030328
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Summaries for Nodular Nonsuppurative Panniculitis

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NIH Rare Diseases : 54 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 Complement and coagulation cascades and Insulin resistance. Affiliated tissues include skin, thyroid and eye, and related phenotypes are nausea and vomiting and fever

Wikipedia : 77 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 84)
# Related Disease Score Top Affiliating Genes
1 localized lipodystrophy 11.6
2 panniculitis 10.8
3 mental retardation, skeletal dysplasia, and abducens palsy 10.4
4 glomerulonephritis 10.4
5 cutaneous solitary mastocytoma 10.3 POMC SERPINA1
6 hepatic lipase deficiency 10.3 LIPC PNLIP
7 familial lipoprotein lipase deficiency 10.3 LIPC PNLIP
8 acute poststreptococcal glomerulonephritis 10.2 CFP SERPINF2
9 membranous nephropathy 10.2
10 alopecia 10.2
11 hepatitis 10.2
12 ulcerative colitis 10.2
13 colitis 10.2
14 immune-complex glomerulonephritis 10.2
15 sella turcica neoplasm 10.2 F8 POMC
16 tuberculum sellae meningioma 10.2 F8 POMC
17 alpha-1-antitrypsin deficiency 10.2
18 exophthalmos 10.2
19 thyroid crisis 10.2 F8 POMC
20 cholesterol ester storage disease 10.2 PNLIP SERPINA1
21 biliary cirrhosis, primary, 1 10.2
22 hashimoto thyroiditis 10.2
23 rheumatic fever-related antigen 10.2
24 diabetes mellitus 10.2
25 primary biliary cirrhosis 10.2
26 uveitis 10.2
27 rheumatic fever 10.2
28 thyroiditis 10.2
29 amyloidosis 10.2
30 amyloidosis aa 10.2
31 parkes weber syndrome 10.2
32 primary biliary cholangitis 10.2
33 weber syndrome 10.2
34 plasma protein metabolism disease 10.1 ELANE SERPINA1
35 livedoid vasculitis 10.1 F8 SERPINF2
36 rh isoimmunization 10.1 PNLIP POMC
37 thrombophlebitis 10.1 F8 SERPINF2
38 mastitis 10.1
39 nephrotic syndrome 10.1
40 granulomatous mastitis 10.1
41 hidradenitis suppurativa 10.1
42 hidradenitis 10.1
43 mediastinal endodermal sinus tumors 10.1
44 cardiac tamponade 10.1 F8 TNFRSF1A
45 factor xiii deficiency 10.1 F8 SERPINF2
46 hemorrhagic disease 10.1 F8 SERPINA1 SERPINF2
47 rubeosis iridis 10.0 SERPINF2 SNRPE
48 niemann-pick disease 10.0
49 rheumatoid arthritis 10.0
50 varicose veins 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:

60 33 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nausea and vomiting 60 33 hallmark (90%) Very frequent (99-80%) HP:0002017
2 fever 60 33 hallmark (90%) Very frequent (99-80%) HP:0001945
3 subcutaneous nodule 60 33 hallmark (90%) Very frequent (99-80%) HP:0001482
4 arthralgia 60 33 hallmark (90%) Very frequent (99-80%) HP:0002829
5 abdominal pain 60 33 hallmark (90%) Very frequent (99-80%) HP:0002027
6 edema 60 33 hallmark (90%) Very frequent (99-80%) HP:0000969
7 weight loss 60 33 hallmark (90%) Very frequent (99-80%) HP:0001824
8 myalgia 60 33 hallmark (90%) Very frequent (99-80%) HP:0003326
9 aplasia/hypoplasia of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0008065
10 erythema 60 33 hallmark (90%) Very frequent (99-80%) HP:0010783
11 panniculitis 60 33 hallmark (90%) Very frequent (99-80%) HP:0012490
12 splenomegaly 60 33 occasional (7.5%) Occasional (29-5%) HP:0001744
13 hepatomegaly 60 33 occasional (7.5%) Occasional (29-5%) HP:0002240
14 autoimmunity 60 33 occasional (7.5%) Occasional (29-5%) HP:0002960
15 inflammatory abnormality of the eye 60 33 occasional (7.5%) Occasional (29-5%) HP:0100533

MGI Mouse Phenotypes related to Nodular Nonsuppurative Panniculitis:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 9.97 CFP ELANE F8 IRS1 KLK4 LIPC
2 adipose tissue MP:0005375 9.65 IRS1 PNLIP POMC SLC2A4 TNFRSF1A
3 immune system MP:0005387 9.61 CFP ELANE F8 IRS1 POMC PPIA
4 liver/biliary system MP:0005370 9.1 IRS1 PNLIP POMC PPIA SLC2A4 TNFRSF1A
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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:

42
Skin, Thyroid, Eye, Bone, Brain, Pituitary, Bone Marrow
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Publications for Nodular Nonsuppurative Panniculitis

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Articles related to Nodular Nonsuppurative Panniculitis:

(show top 50) (show all 180)
# 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
Weber-Christian disease: a case report. ( 30590944 )
2018
3
Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease. ( 28352826 )
2016
4
Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature. ( 25325705 )
2016
5
Pfeifer-Weber-Christian Disease during Pregnancy Successfully Treated with Corticosteroids. ( 27688468 )
2016
6
Nephrotic syndrome in a patient with Pfeifer-Weber-Christian disease. ( 25543272 )
2015
7
Diagnosis of extensive myocardial, pericardial, and coronary calcification in Weber-Christian disease. ( 25925218 )
2015
8
Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. ( 25954116 )
2015
9
Fat-Removal Orbital Decompressing for Severe Bilateral Proptosis due to Weber-Christian Disease. ( 25978784 )
2015
10
Mycobacterium chelonae infection presenting as recurrent cutaneous and subcutaneous nodules--a presentation previously diagnosed as Weber Christian disease. ( 24945648 )
2014
11
Weber-Christian disease developing into mediastinitis and pleuritis with massive pleural effusion. ( 22504256 )
2012
12
Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report. ( 23216240 )
2012
13
Weber-Christian disease associated with granulomatous mastitis: a variant type of Weber-Christian disease? ( 20922452 )
2011
14
Skin lesions depicting a systemic disease. Weber-Christian disease. ( 21325702 )
2011
15
A case of Weber-Christian disease with later development of rheumatoid arthritis. ( 21372513 )
2011
16
Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. ( 20701939 )
2010
17
A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. ( 20105325 )
2010
18
Chorioretinitis associated with weber-christian disease. ( 25391077 )
2009
19
Weber and Christian of Weber-Christian disease. ( 18401882 )
2008
20
Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. ( 16550303 )
2007
21
Childhood Weber-Christian disease: clinical investigation and virus detection. ( 17888054 )
2007
22
Idiopathic lobular panniculitis (Weber Christian disease): a case report. ( 18603907 )
2006
23
A case of Weber-Christian disease with collapsing glomerulopathy. ( 16931223 )
2006
24
Panniculitis and liver disease (hepatic Weber Christian disease). ( 15629524 )
2005
25
Successful treatment of steroid-resistant Weber-Christian disease with biliary ductopenia using cyclosporin A. ( 15757971 )
2005
26
Staphylococcus cohnii as a cause of multiple brain abscesses in Weber-Christian disease. ( 16054652 )
2005
27
A case of Weber-Christian disease associated with myelodysplastic syndrome. ( 17028810 )
2004
28
Weber-Christian disease and pituitary dysfunction in a patient with polymyositis. ( 15638065 )
2004
29
Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. ( 12745859 )
2003
30
Weber-Christian disease presenting with ocular manifestations. ( 14576996 )
2003
31
Weber Christian disease. ( 14621048 )
2003
32
Successful treatment of idiopathic febrile panniculitis (Weber-Christian disease) with thalidomide in a patient having failed multiple other medical therapies. ( 17041381 )
2002
33
Weber-Christian disease with severe polyarthritis and polyosteitis. ( 12022332 )
2002
34
The men behind the eponym: Frederick Parkes Weber and Henry a. Christian: Weber-Christian disease. ( 12454605 )
2002
35
Hepatic Weber-Christian disease. ( 11296691 )
2001
36
Osteoarthropathy associated with Weber-Christian disease. ( 11550989 )
2001
37
Weber Christian disease in a type 2 diabetic patient. ( 11584948 )
2001
38
Weber-Christian disease presenting with proptosis: a case report. ( 10803707 )
2000
39
Systemic Weber-Christian disease complicated by partial transverse myelopathy. ( 11070575 )
2000
40
Systemic Weber-Christian disease. ( 11179936 )
2000
41
Successful treatment of a patient with febrile, lobular panniculitis (Weber-Christian disease) with oral cyclosporin A: implications for pathogenesis and therapy. ( 10435371 )
1999
42
Steroid-resistant Weber-Christian Disease. ( 10435355 )
1999
43
Central serous retinopathy associated with Weber-Christian disease. ( 10435428 )
1999
44
Weber-Christian disease associated with lupus anticoagulant and anticardiolipin antibodies. ( 10501442 )
1999
45
Treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. ( 9738797 )
1998
46
Proptosis as the manifesting sign of Weber-Christian disease. ( 9222254 )
1997
47
Weber-Christian disease with calcinosis: a case report. ( 8973879 )
1996
48
Crystal-storing histiocytosis associated with lymphoplasmacytic lymphoma mimicking Weber-Christian disease: immunohistochemical, ultrastructural, and gene-rearrangement studies. ( 8543317 )
1996
49
Nodular nonsuppurative panniculitis in association with primary biliary cirrhosis and Hashimoto's thyroiditis. ( 8686385 )
1996
50
Recognizing Weber-Christian disease. ( 8987390 )
1996
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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
Complement and coagulation cascades 1 38
11.58 F8 SERPINA1 SERPINF2
2
Insulin resistance 38
Show member pathways
 11.37 IRS1 SLC2A4 TNFRSF1A
3
Metformin Pathway, Pharmacodynamics 62
10.96 IRS1 SLC2A4
4
Factors and pathways affecting insulin-like growth factor (IGF1)-Akt signaling 1
10.86 IRS1 TNFRSF1A
5
Adipocytokine signaling pathway 38
10.76 IRS1 POMC SLC2A4 TNFRSF1A
6
Transcription factor regulation in adipogenesis 1
10.66 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 space GO:0005615 9.65 CFP ELANE F8 LIPC PNLIP POMC
2 endoplasmic reticulum lumen GO:0005788 9.62 CFP F8 LIPC SERPINA1
3 collagen-containing extracellular matrix GO:0062023 9.56 CFP ELANE SERPINA1 SERPINF2
4 secretory granule GO:0030141 9.54 ELANE KLK4 POMC
5 extracellular region GO:0005576 9.36 CFP ELANE F8 KLK4 LIPC PNLIP
6 platelet alpha granule lumen GO:0031093 9.33 F8 SERPINA1 SERPINF2

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

# Name GO ID Score Top Affiliating Genes
1 neutrophil degranulation GO:0043312 9.56 CFP ELANE PPIA SERPINA1
2 platelet degranulation GO:0002576 9.33 F8 SERPINA1 SERPINF2
3 glucose homeostasis GO:0042593 9.13 IRS1 POMC SLC2A4
4 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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 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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