WCD
MCID: NDL003
MIFTS: 40

Nodular Nonsuppurative Panniculitis (WCD)

Categories: Rare diseases, Skin diseases
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Aliases & Classifications for Nodular Nonsuppurative Panniculitis

MalaCards integrated aliases for Nodular Nonsuppurative Panniculitis:

Name: Nodular Nonsuppurative Panniculitis 11 19 14 16 75
Relapsing Febrile Nodular Nonsuppurative Panniculitis 11 19 58
Weber-Christian Disease 11 58 75
Panniculitis, Nodular Nonsuppurative 43 71
Pfeiffer-Weber-Christian Syndrome 19 58
Idiopathic Nodular Panniculitis 19 58
Weber-Christian Panniculitis 19 58
Nodular Non-Suppurative Febrile Panniculitis 11
Relapsing Febrile Nodular Panniculitis 58
Nodular Non-Suppurative Panniculitis 58
Panniculitis Nodular Nonsuppurative 19
Idiopathic Lobular Panniculitis 58
Weber - Christian Disease 11
Wcd 58

Characteristics:


Age Of Onset:

Nodular Non-Suppurative Panniculitis: Adult 58

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 11 DOID:1525
MeSH 43 D010201
SNOMED-CT 68 203120002
ICD10 31 M35.6
MESH via Orphanet 44 D010201
ICD10 via Orphanet 32 M35.6
UMLS via Orphanet 72 C0030328
Orphanet 58 ORPHA33577
UMLS 71 C0030328

Summaries for Nodular Nonsuppurative Panniculitis

GARD: 19 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. 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 panniculitis and non-alcoholic fatty liver disease. An important gene associated with Nodular Nonsuppurative Panniculitis is SERPINA1 (Serpin Family A Member 1), and among its related pathways/superpathways is Development_Leptin signaling via JAK/STAT and MAPK cascades. Affiliated tissues include skin, eye and pancreas, and related phenotypes are nausea and vomiting and fever

Orphanet: 58 A rare skin disorder characterized by recurring inflammation in the subcutaneous layer of fat.

Wikipedia 75 Nodular nonsuppurative panniculitis: Weber-Christian disease, is a cutaneous condition characterized by recurrent subcutaneous nodules that... more...

Weber-christian disease: 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 206)
# Related Disease Score Top Affiliating Genes
1 panniculitis 31.7 SERPINA1 CRP
2 non-alcoholic fatty liver disease 29.7 TM6SF2 PNPLA3 CRP
3 mental retardation, skeletal dysplasia, and abducens palsy 10.4
4 cardiac conduction defect 10.4
5 aplastic anemia 10.4
6 alpha-1-antitrypsin deficiency 10.4
7 pancytopenia 10.4
8 pancreatic cancer 10.3
9 disseminated intravascular coagulation 10.3
10 peritonitis 10.3
11 vasculitis 10.3
12 igg4-related mesenteritis 10.3
13 ventricular fibrillation, paroxysmal familial, 1 10.3
14 glomerulonephritis 10.3
15 alopecia 10.3
16 systemic lupus erythematosus 10.3
17 nephrotic syndrome 10.3
18 histiocytosis 10.3
19 collagen disease 10.3
20 perforation of bile duct 10.2 SERPINA1 CRP
21 peroxisome biogenesis disorder 1a 10.2
22 viral laryngitis 10.2 SERPINA1 CRP
23 pleural lipoma 10.2 SERPINA1 CRP
24 constrictive pericarditis 10.2 SERPINA1 CRP
25 whipple disease 10.2 SERPINA1 CRP
26 bronchiectasis 1 10.2 SERPINA1 CRP
27 varicose veins 10.2
28 myeloma, multiple 10.2
29 deficiency anemia 10.2
30 erythema nodosum 10.2
31 mastitis 10.2
32 anterior uveitis 10.2
33 calcinosis 10.2
34 pituitary gland disease 10.2
35 retroperitoneal fibrosis 10.2
36 granulomatous mastitis 10.2
37 protein-losing enteropathy 10.2 SERPINA1 CRP
38 viral meningitis 10.2 SERPINA1 CRP
39 diverticulitis 10.2 SERPINA1 CRP
40 plasma protein metabolism disease 10.2 SERPINA1 CRP
41 coronary heart disease 1 10.1 LIPC CRP
42 pfeiffer syndrome 10.1
43 hashimoto thyroiditis 10.1
44 capillary malformation-arteriovenous malformation 1 10.1
45 leptin deficiency or dysfunction 10.1
46 leptin receptor deficiency 10.1
47 serum amyloid a amyloidosis 10.1
48 primary biliary cholangitis 10.1
49 uveitis 10.1
50 rheumatic fever 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 30 (show all 15)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 nausea and vomiting 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002017
2 fever 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001945
3 subcutaneous nodule 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001482
4 abdominal pain 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002027
5 arthralgia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0002829
6 myalgia 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0003326
7 aplasia/hypoplasia of the skin 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0008065
8 erythema 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0010783
9 weight loss 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001824
10 edema 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000969
11 panniculitis 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0012490
12 splenomegaly 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0001744
13 hepatomegaly 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002240
14 autoimmunity 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0002960
15 inflammatory abnormality of the eye 58 30 Occasional (7.5%) Occasional (29-5%)
HP:0100533

MGI Mouse Phenotypes related to Nodular Nonsuppurative Panniculitis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 liver/biliary system MP:0005370 9.02 CIDEC PNPLA3 POMC RNASEH2B TM6SF2

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

Organs/tissues related to Nodular Nonsuppurative Panniculitis:

MalaCards : Skin, Eye, Pancreas, Bone Marrow, Liver, Breast, Pituitary

Publications for Nodular Nonsuppurative Panniculitis

Articles related to Nodular Nonsuppurative Panniculitis:

(show top 50) (show all 487)
# Title Authors PMID Year
1
Panniculitis as a manifestation of metal-associated autoimmune/inflammatory syndrome induced by adjuvants: a case-based review. 62
34180012 2021
2
Investigating the presence of neutrophil extracellular traps in septal and lobular cutaneous panniculitides. 62
33580883 2021
3
Histobulin as a complementary but essential therapeutic for Intravenous Immune Globulin Therapy of Pfeiffer-Weber-Christian disease with multiple allergic diseases and its effects on allergic disease: A case report. 62
33598280 2021
4
Intravenous Immune Globulin (IVIG) Therapy After Unsuccessful Treatment with Corticosteroid and Cyclosporine A in Pfeifer-Weber-Christian Disease: A Case Report. 62
33390586 2021
5
[Autoimmunity and autoinflammation in pathogenesis of immunoinflammatory diseases]. 62
33720583 2020
6
Generalized chronic erythema nodosum: to consider Weber-Christian disease. 62
34002995 2020
7
Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices. 62
32528225 2020
8
Analysis of the clinical characteristics of thirteen patients with Weber-Christian panniculitis. 62
31402393 2019
9
Pfeifer-Weber-Christian disease and successful treatment with mycophenolate mofetil: a case report. 62
31122958 2019
10
[Weber-Christian's disease: a preleukemic disorder?] 62
31223415 2019
11
[Idiopathic lobular panniculitis: clinical and morphological correlations]. 62
31317929 2019
12
Weber-Christian disease presenting with lung nodules dramatically improved with corticosteroid therapy: one case report and literature review. 62
26224252 2018
13
Weber-Christian disease: a case report. 62
30590944 2018
14
Weber-Christian Panniculitis: Is it a Disorder of Immune System? 62
26944431 2016
15
Pfeifer-Weber-Christian Disease during Pregnancy Successfully Treated with Corticosteroids. 62
27688468 2016
16
Sclerosing mesenteric panniculitis in a young patient : common cause of diagnostic dilemma and treatment refractoriness. 62
27382948 2016
17
Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature. 62
25325705 2016
18
Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease. 62
28352826 2016
19
Diagnosis of extensive myocardial, pericardial, and coronary calcification in Weber-Christian disease. 62
25925218 2015
20
Nephrotic syndrome in a patient with Pfeifer-Weber-Christian disease. 62
25543272 2015
21
Fat-Removal Orbital Decompressing for Severe Bilateral Proptosis due to Weber-Christian Disease. 62
25978784 2015
22
Weber-Christian disease with ileocolonic involvement successfully treated with infliximab. 62
25954116 2015
23
Mycobacterium chelonae infection presenting as recurrent cutaneous and subcutaneous nodules--a presentation previously diagnosed as Weber Christian disease. 62
24945648 2014
24
Idiopatic lobular necrotizing paniculitis. 62
25023430 2014
25
The endemic mimic: blastomycosis an illness often misdiagnosed. 62
25125734 2014
26
Panniculitis in children. 62
23900159 2013
27
How to make a specific diagnosis of panniculitis on clinical grounds alone: an integrated pathway of general criteria and specific findings. 62
23900156 2013
28
[Weber-Christian disease]. 62
24417073 2013
29
Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report. 62
23216240 2012
30
A 38-year old woman with a dural based lesion. 62
22497617 2012
31
Weber-Christian disease developing into mediastinitis and pleuritis with massive pleural effusion. 62
22504256 2012
32
[Lipoatrophic panniculitis in adults: treatment with hydroxychloroquine]. 62
21978506 2011
33
[A case of idiopathic sclerosing mesenteritis with retroperitoneal fibrosis]. 62
22042424 2011
34
[Observation of Pfeiffer-Weber-Christian syndrome, simulating sepsis]. 62
21789991 2011
35
Weber-Christian disease associated with granulomatous mastitis: a variant type of Weber-Christian disease? 62
20922452 2011
36
Annular lipoatrophic panniculitis of the ankles. 62
21418290 2011
37
Unusual systemic signs in a dog with sterile neutrophilic-macrophagic lymphadenitis and nodular panniculitis. 62
21311075 2011
38
Skin lesions depicting a systemic disease. Weber-Christian disease. 62
21325702 2011
39
A case of Weber-Christian disease with later development of rheumatoid arthritis. 62
21372513 2011
40
Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature. 62
20701939 2010
41
[Weber-Christian disease: unknown etiology systemic panniculitis]. 62
21627887 2010
42
Mesenteric panniculitis in Merseyside: a case series and a review of the literature. 62
20615306 2010
43
Efficacy of cyclosporine A treatment in relapsing febrile lobular panniculitis associated with small vessel vasculitis. 62
19506878 2010
44
A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. 62
20105325 2010
45
Idiopathic lobular panniculitis: remission induced and maintained with infliximab. 62
19519831 2009
46
Chorioretinitis associated with weber-christian disease. 62
25391077 2009
47
Weber and Christian of Weber-Christian disease. 62
18401882 2008
48
Childhood Weber-Christian disease: clinical investigation and virus detection. 62
17888054 2007
49
Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. 62
16550303 2007
50
Case reports: nodular vasculitis responsive to mycophenolate mofetil. 62
17373149 2006

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
Show member pathways
10.1 POMC CRP

GO Terms for Nodular Nonsuppurative Panniculitis

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

# Name GO ID Score Top Affiliating Genes
1 triglyceride catabolic process GO:0019433 9.26 PNPLA3 LIPC
2 lipid droplet organization GO:0034389 8.92 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 9.46 PNPLA3 LIPC
2 low-density lipoprotein particle binding GO:0030169 9.26 LIPC CRP
3 lipoprotein lipase activity GO:0004465 8.92 PNPLA3 LIPC

Sources for Nodular Nonsuppurative Panniculitis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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