WCD
MCID: NDL003
MIFTS: 45

Nodular Nonsuppurative Panniculitis (WCD)

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Nodular Nonsuppurative Panniculitis

MalaCards integrated aliases for Nodular Nonsuppurative Panniculitis:

Name: Nodular Nonsuppurative Panniculitis 12 53 15 17
Weber-Christian Disease 12 75 53 59
Relapsing Febrile Nodular Nonsuppurative Panniculitis 12 59
Panniculitis, Nodular Nonsuppurative 44 72
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
Wcd 59

Characteristics:

Orphanet epidemiological data:

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

Classifications:

Orphanet: 59  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:1525
MeSH 44 D010201
SNOMED-CT 68 33760009
ICD10 33 M35.6
MESH via Orphanet 45 D010201
ICD10 via Orphanet 34 M35.6
UMLS via Orphanet 73 C0030328
Orphanet 59 ORPHA33577
UMLS 72 C0030328

Summaries for Nodular Nonsuppurative Panniculitis

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 body mass index quantitative trait locus 11 and localized lipodystrophy. 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, eye and pancreas, and related phenotypes are nausea and vomiting and fever

Wikipedia : 75 Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue (the... more...

Related Diseases for Nodular Nonsuppurative Panniculitis

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

(show top 50) (show all 168)
# Related Disease Score Top Affiliating Genes
1 body mass index quantitative trait locus 11 28.8 SLC2A4 POMC PNLIP LIPC IRS1
2 localized lipodystrophy 11.7
3 panniculitis 10.9
4 cardiac conduction defect 10.6
5 cutaneous solitary mastocytoma 10.5 SERPINA1 POMC
6 mental retardation, skeletal dysplasia, and abducens palsy 10.5
7 hepatic lipase deficiency 10.5 PNLIP LIPC
8 ventricular fibrillation, paroxysmal familial, 1 10.5
9 familial lipoprotein lipase deficiency 10.5 PNLIP LIPC
10 acute poststreptococcal glomerulonephritis 10.4 SERPINF2 CFP
11 vasculitis 10.4
12 tuberculum sellae meningioma 10.4 POMC F8
13 glomerulonephritis 10.4
14 alopecia 10.4
15 sella turcica neoplasm 10.4 POMC F8
16 thyroid crisis 10.3 POMC F8
17 cholesterol ester storage disease 10.3 SERPINA1 PNLIP
18 cardiac arrest 10.3
19 alpha-1-antitrypsin deficiency 10.2
20 pancytopenia 10.2
21 exophthalmos 10.2
22 takayasu arteritis 10.2
23 dermatitis herpetiformis, familial 10.2
24 body mass index quantitative trait locus 9 10.2
25 body mass index quantitative trait locus 8 10.2
26 body mass index quantitative trait locus 4 10.2
27 body mass index quantitative trait locus 10 10.2
28 body mass index quantitative trait locus 7 10.2
29 body mass index quantitative trait locus 12 10.2
30 body mass index quantitative trait locus 14 10.2
31 membranous nephropathy 10.2
32 body mass index quantitative trait locus 18 10.2
33 body mass index quantitative trait locus 19 10.2
34 body mass index quantitative trait locus 20 10.2
35 bacterial infectious disease 10.2
36 autoimmune hepatitis 10.2
37 hepatitis 10.2
38 post-thrombotic syndrome 10.2
39 immune-complex glomerulonephritis 10.2
40 connective tissue disease 10.2
41 dermatitis herpetiformis 10.2
42 ulcerative colitis 10.2
43 alopecia areata 10.2
44 livedoid vasculitis 10.2 SERPINF2 F8
45 rh isoimmunization 10.2 POMC PNLIP
46 nephrotic syndrome 10.2
47 histiocytosis 10.2
48 collagen disease 10.2
49 lupus erythematosus 10.2
50 leptin deficiency or dysfunction 10.2

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



Diseases related to Nodular Nonsuppurative Panniculitis

Symptoms & Phenotypes for Nodular Nonsuppurative Panniculitis

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

MGI Mouse Phenotypes related to Nodular Nonsuppurative Panniculitis:

46
# 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

Drugs & Therapeutics for Nodular Nonsuppurative Panniculitis

Search Clinical Trials , NIH Clinical Center for Nodular Nonsuppurative Panniculitis

Cochrane evidence based reviews: panniculitis, nodular nonsuppurative

Genetic Tests for Nodular Nonsuppurative Panniculitis

Anatomical Context for Nodular Nonsuppurative Panniculitis

MalaCards organs/tissues related to Nodular Nonsuppurative Panniculitis:

41
Skin, Eye, Pancreas, Liver, Lung, Bone, Breast

Publications for Nodular Nonsuppurative Panniculitis

Articles related to Nodular Nonsuppurative Panniculitis:

(show top 50) (show all 416)
# Title Authors PMID Year
1
Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis. 38
31402393 2019
2
Pfeifer-Weber-Christian disease and successful treatment with mycophenolate mofetil: a case report. 38
31122958 2019
3
Weber-Christian disease presenting with lung nodules dramatically improved with corticosteroid therapy: one case report and literature review. 38
26224252 2018
4
Weber-Christian disease: a case report. 38
30590944 2018
5
Pfeifer-Weber-Christian Disease during Pregnancy Successfully Treated with Corticosteroids. 38
27688468 2016
6
Sclerosing mesenteric panniculitis in a young patient : common cause of diagnostic dilemma and treatment refractoriness. 38
27382948 2016
7
Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease. 38
28352826 2016
8
Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature. 38
25325705 2016
9
Diagnosis of extensive myocardial, pericardial, and coronary calcification in Weber-Christian disease. 38
25925218 2015
10
Nephrotic syndrome in a patient with Pfeifer-Weber-Christian disease. 38
25543272 2015
11
Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. 38
25954116 2015
12
Fat-Removal Orbital Decompressing for Severe Bilateral Proptosis due to Weber-Christian Disease. 38
25978784 2015
13
Mycobacterium chelonae infection presenting as recurrent cutaneous and subcutaneous nodules--a presentation previously diagnosed as Weber Christian disease. 38
24945648 2014
14
Idiopatic lobular necrotizing paniculitis. 38
25023430 2014
15
Panniculitis in children. 38
23900159 2013
16
[Weber-Christian disease]. 38
24417073 2013
17
Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report. 38
23216240 2012
18
A 38-year old woman with a dural based lesion. 38
22497617 2012
19
Weber-Christian disease developing into mediastinitis and pleuritis with massive pleural effusion. 38
22504256 2012
20
[Lipoatrophic panniculitis in adults: treatment with hydroxychloroquine]. 38
21978506 2011
21
[A case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis]. 38
22042424 2011
22
Weber-Christian disease associated with granulomatous mastitis: a variant type of Weber-Christian disease? 38
20922452 2011
23
A case of Weber-Christian disease with later development of rheumatoid arthritis. 38
21372513 2011
24
Skin lesions depicting a systemic disease. Weber-Christian disease. 38
21325702 2011
25
[Weber-Christian disease: unknown etiology systemic panniculitis]. 38
21627887 2010
26
Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. 38
20701939 2010
27
Mesenteric panniculitis in Merseyside: a case series and a review of the literature. 38
20615306 2010
28
Efficacy of cyclosporine A treatment in relapsing febrile lobular panniculitis associated with small vessel vasculitis. 38
19506878 2010
29
A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. 38
20105325 2010
30
Chorioretinitis associated with weber-christian disease. 38
25391077 2009
31
Weber and Christian of Weber-Christian disease. 38
18401882 2008
32
Childhood Weber-Christian disease: clinical investigation and virus detection. 38
17888054 2007
33
Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. 38
16550303 2007
34
Case reports: nodular vasculitis responsive to mycophenolate mofetil. 38
17373149 2006
35
A case of Weber-Christian disease with collapsing glomerulopathy. 38
16931223 2006
36
A case of Rothmann-Makai panniculitis successfully treated with tetracycline. 38
16681577 2006
37
Idiopathic lobular panniculitis (Weber Christian disease): a case report. 38
18603907 2006
38
Panniculitis in childhood and adolescence. 38
16490070 2006
39
Staphylococcus cohnii as a cause of multiple brain abscesses in Weber-Christian disease. 38
16054652 2005
40
Insulin resistance and clinical aspects of non-alcoholic steatohepatitis (NASH). 38
16198620 2005
41
Successful treatment of steroid-resistant Weber-Christian disease with biliary ductopenia using cyclosporin A. 38
15757971 2005
42
Panniculitis and liver disease (hepatic Weber Christian disease). 38
15629524 2005
43
Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS). 38
15479908 2004
44
Weber-Christian disease and pituitary dysfunction in a patient with polymyositis. 38
15638065 2004
45
Fatal cytophagic histiocytic panniculitis. 38
15165205 2004
46
A case of Weber-Christian disease associated with myelodysplastic syndrome. 38
17028810 2004
47
Weber-Christian disease presenting with ocular manifestations. 38
14576996 2003
48
Weber Christian disease. 38
14621048 2003
49
Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. 38
12745859 2003
50
The men behind the eponym: Frederick Parkes Weber and Henry a. Christian: Weber-Christian disease. 38
12454605 2002

Variations for Nodular Nonsuppurative Panniculitis

Expression for Nodular Nonsuppurative Panniculitis

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

Pathways for Nodular Nonsuppurative Panniculitis

GO Terms for Nodular Nonsuppurative Panniculitis

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 TNFRSF1A SERPINF2 SERPINA1 PPIA POMC PNLIP
2 endoplasmic reticulum lumen GO:0005788 9.62 SERPINA1 LIPC F8 CFP
3 collagen-containing extracellular matrix GO:0062023 9.56 SERPINF2 SERPINA1 ELANE CFP
4 secretory granule GO:0030141 9.54 POMC KLK4 ELANE
5 extracellular region GO:0005576 9.36 TNFRSF1A SERPINF2 SERPINA1 PPIA POMC PNLIP
6 platelet alpha granule lumen GO:0031093 9.33 SERPINF2 SERPINA1 F8

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 SERPINA1 PPIA ELANE CFP
2 platelet degranulation GO:0002576 9.33 SERPINF2 SERPINA1 F8
3 glucose homeostasis GO:0042593 9.13 SLC2A4 POMC IRS1
4 acute-phase response GO:0006953 8.8 SERPINF2 SERPINA1 F8

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 PNLIP LIPC
2 lipase activity GO:0016298 8.96 PNLIP LIPC
3 protease binding GO:0002020 8.8 SERPINF2 SERPINA1 ELANE

Sources for Nodular Nonsuppurative Panniculitis

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
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