WCD
MCID: NDL003
MIFTS: 39

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 52 15 17
Relapsing Febrile Nodular Nonsuppurative Panniculitis 12 52 58
Weber-Christian Disease 12 74 58
Panniculitis, Nodular Nonsuppurative 43 71
Pfeiffer-Weber-Christian Syndrome 52 58
Idiopathic Nodular Panniculitis 52 58
Weber-Christian Panniculitis 52 58
Nodular Non-Suppurative Febrile Panniculitis 12
Relapsing Febrile Nodular Panniculitis 58
Nodular Non-Suppurative Panniculitis 58
Panniculitis Nodular Nonsuppurative 52
Idiopathic Lobular Panniculitis 58
Weber - Christian Disease 12
Wcd 58

Characteristics:

Orphanet epidemiological data:

58
nodular non-suppurative panniculitis
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:1525
MeSH 43 D010201
SNOMED-CT 67 33760009
ICD10 32 M35.6
MESH via Orphanet 44 D010201
ICD10 via Orphanet 33 M35.6
UMLS via Orphanet 72 C0030328
Orphanet 58 ORPHA33577
UMLS 71 C0030328

Summaries for Nodular Nonsuppurative Panniculitis

NIH Rare Diseases : 52 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 relapsing febrile nodular nonsuppurative panniculitis, 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 is Glycerolipid metabolism. Affiliated tissues include skin, eye and pancreas, and related phenotypes are fever and weight loss

Wikipedia : 74 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 152)
# Related Disease Score Top Affiliating Genes
1 localized lipodystrophy 11.7
2 panniculitis 10.9
3 cardiac conduction defect 10.6
4 mental retardation, skeletal dysplasia, and abducens palsy 10.5
5 ventricular fibrillation, paroxysmal familial, 1 10.5
6 vasculitis 10.4
7 glomerulonephritis 10.4
8 alopecia 10.4
9 cardiac arrest 10.3
10 alpha-1-antitrypsin deficiency 10.3
11 pancytopenia 10.3
12 exophthalmos 10.3
13 takayasu arteritis 10.2
14 dermatitis herpetiformis, familial 10.2
15 membranous nephropathy 10.2
16 bacterial infectious disease 10.2
17 autoimmune hepatitis 10.2
18 hepatitis 10.2
19 post-thrombotic syndrome 10.2
20 immune-complex glomerulonephritis 10.2
21 connective tissue disease 10.2
22 dermatitis herpetiformis 10.2
23 ulcerative colitis 10.2
24 alopecia areata 10.2
25 nephrotic syndrome 10.2
26 neutropenia 10.2
27 histiocytosis 10.2
28 collagen disease 10.2
29 lupus erythematosus 10.2
30 leptin deficiency or dysfunction 10.2
31 primary biliary cirrhosis 10.2
32 uveitis 10.2
33 rheumatic fever 10.2
34 thrombocytopenia 10.2
35 liver cirrhosis 10.2
36 thyroiditis 10.2
37 amyloidosis 10.2
38 diabetes mellitus 10.2
39 amyloidosis aa 10.2
40 parkes weber syndrome 10.2
41 weber syndrome 10.2
42 atrial standstill 1 10.1
43 progressive familial heart block, type ia 10.1
44 peripartum cardiomyopathy 10.1
45 left bundle branch hemiblock 10.1
46 dilated cardiomyopathy 10.1
47 hidradenitis suppurativa 10.1
48 hidradenitis 10.1
49 syncope 10.1
50 varicose veins 10.1

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:

58 31 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 58 31 hallmark (90%) Very frequent (99-80%) HP:0001945
2 weight loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0001824
3 edema 58 31 hallmark (90%) Very frequent (99-80%) HP:0000969
4 subcutaneous nodule 58 31 hallmark (90%) Very frequent (99-80%) HP:0001482
5 arthralgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0002829
6 erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0010783
7 nausea and vomiting 58 31 hallmark (90%) Very frequent (99-80%) HP:0002017
8 abdominal pain 58 31 hallmark (90%) Very frequent (99-80%) HP:0002027
9 myalgia 58 31 hallmark (90%) Very frequent (99-80%) HP:0003326
10 aplasia/hypoplasia of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0008065
11 panniculitis 58 31 hallmark (90%) Very frequent (99-80%) HP:0012490
12 splenomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0001744
13 hepatomegaly 58 31 occasional (7.5%) Occasional (29-5%) HP:0002240
14 autoimmunity 58 31 occasional (7.5%) Occasional (29-5%) HP:0002960
15 inflammatory abnormality of the eye 58 31 occasional (7.5%) Occasional (29-5%) HP:0100533

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:

40
Skin, Eye, Pancreas, Bone, Liver, Lung, Breast

Publications for Nodular Nonsuppurative Panniculitis

Articles related to Nodular Nonsuppurative Panniculitis:

(show top 50) (show all 417)
# Title Authors PMID Year
1
Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices. 61
32528225 2020
2
Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis. 61
31402393 2019
3
Pfeifer-Weber-Christian disease and successful treatment with mycophenolate mofetil: a case report. 61
31122958 2019
4
Weber-Christian disease presenting with lung nodules dramatically improved with corticosteroid therapy: one case report and literature review. 61
26224252 2018
5
Weber-Christian disease: a case report. 61
30590944 2018
6
Pfeifer-Weber-Christian Disease during Pregnancy Successfully Treated with Corticosteroids. 61
27688468 2016
7
Sclerosing mesenteric panniculitis in a young patient : common cause of diagnostic dilemma and treatment refractoriness. 61
27382948 2016
8
Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease. 61
28352826 2016
9
Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature. 61
25325705 2016
10
Diagnosis of extensive myocardial, pericardial, and coronary calcification in Weber-Christian disease. 61
25925218 2015
11
Nephrotic syndrome in a patient with Pfeifer-Weber-Christian disease. 61
25543272 2015
12
Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. 61
25954116 2015
13
Fat-Removal Orbital Decompressing for Severe Bilateral Proptosis due to Weber-Christian Disease. 61
25978784 2015
14
Mycobacterium chelonae infection presenting as recurrent cutaneous and subcutaneous nodules--a presentation previously diagnosed as Weber Christian disease. 61
24945648 2014
15
Idiopatic lobular necrotizing paniculitis. 61
25023430 2014
16
Panniculitis in children. 61
23900159 2013
17
[Weber-Christian disease]. 61
24417073 2013
18
Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report. 61
23216240 2012
19
A 38-year old woman with a dural based lesion. 61
22497617 2012
20
Weber-Christian disease developing into mediastinitis and pleuritis with massive pleural effusion. 61
22504256 2012
21
[Lipoatrophic panniculitis in adults: treatment with hydroxychloroquine]. 61
21978506 2011
22
[A case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis]. 61
22042424 2011
23
Weber-Christian disease associated with granulomatous mastitis: a variant type of Weber-Christian disease? 61
20922452 2011
24
A case of Weber-Christian disease with later development of rheumatoid arthritis. 61
21372513 2011
25
Skin lesions depicting a systemic disease. Weber-Christian disease. 61
21325702 2011
26
[Weber-Christian disease: unknown etiology systemic panniculitis]. 61
21627887 2010
27
Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. 61
20701939 2010
28
Mesenteric panniculitis in Merseyside: a case series and a review of the literature. 61
20615306 2010
29
Efficacy of cyclosporine A treatment in relapsing febrile lobular panniculitis associated with small vessel vasculitis. 61
19506878 2010
30
A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. 61
20105325 2010
31
Chorioretinitis associated with weber-christian disease. 61
25391077 2009
32
Weber and Christian of Weber-Christian disease. 61
18401882 2008
33
Childhood Weber-Christian disease: clinical investigation and virus detection. 61
17888054 2007
34
Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. 61
16550303 2007
35
Case reports: nodular vasculitis responsive to mycophenolate mofetil. 61
17373149 2006
36
A case of Weber-Christian disease with collapsing glomerulopathy. 61
16931223 2006
37
A case of Rothmann-Makai panniculitis successfully treated with tetracycline. 61
16681577 2006
38
Idiopathic lobular panniculitis (Weber Christian disease): a case report. 61
18603907 2006
39
Panniculitis in childhood and adolescence. 61
16490070 2006
40
Staphylococcus cohnii as a cause of multiple brain abscesses in Weber-Christian disease. 61
16054652 2005
41
Insulin resistance and clinical aspects of non-alcoholic steatohepatitis (NASH). 61
16198620 2005
42
Successful treatment of steroid-resistant Weber-Christian disease with biliary ductopenia using cyclosporin A. 61
15757971 2005
43
Panniculitis and liver disease (hepatic Weber Christian disease). 61
15629524 2005
44
Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS). 61
15479908 2004
45
Weber-Christian disease and pituitary dysfunction in a patient with polymyositis. 61
15638065 2004
46
Fatal cytophagic histiocytic panniculitis. 61
15165205 2004
47
A case of Weber-Christian disease associated with myelodysplastic syndrome. 61
17028810 2004
48
Weber-Christian disease presenting with ocular manifestations. 61
14576996 2003
49
Weber Christian disease. 61
14621048 2003
50
Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. 61
12745859 2003

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

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

# Super pathways Score Top Affiliating Genes
1 10.41 PNPLA3 LIPC

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 endoplasmic reticulum-Golgi intermediate compartment membrane GO:0033116 8.62 TM6SF2 SERPINA1

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

# Name GO ID Score Top Affiliating Genes
1 lipid homeostasis GO:0055088 9.32 TM6SF2 PNPLA3
2 acute-phase response GO:0006953 9.26 SERPINA1 CRP
3 RNA catabolic process GO:0006401 9.16 RNASEH2B RNASE6
4 triglyceride catabolic process GO:0019433 8.96 PNPLA3 LIPC
5 lipid droplet organization GO:0034389 8.62 PNPLA3 CIDEC

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 8.96 PNPLA3 LIPC
2 low-density lipoprotein particle binding GO:0030169 8.62 LIPC CRP

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
Content
Loading form....