WCD
MCID: NDL003
MIFTS: 50

Nodular Nonsuppurative Panniculitis (WCD)

Categories: Muscle diseases, Rare diseases, Skin diseases

Aliases & Classifications for Nodular Nonsuppurative Panniculitis

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:

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

Summaries for Nodular Nonsuppurative Panniculitis

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, eye and bone, 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...

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 82)
# 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 10.1
22 rheumatic fever-related antigen 10.1
23 diabetes mellitus 10.1
24 primary biliary cirrhosis 10.1
25 uveitis 10.1
26 rheumatic fever 10.1
27 thyroiditis 10.1
28 amyloidosis 10.1
29 amyloidosis aa 10.1
30 parkes weber syndrome 10.1
31 primary biliary cholangitis 10.1
32 weber syndrome 10.1
33 plasma protein metabolism disease 10.1 ELANE SERPINA1
34 livedoid vasculitis 10.1 F8 SERPINF2
35 mastitis 10.1
36 nephrotic syndrome 10.1
37 granulomatous mastitis 10.1
38 rh isoimmunization 10.1 PNLIP POMC
39 thrombophlebitis 10.1 F8 SERPINF2
40 cardiac tamponade 10.1 F8 TNFRSF1A
41 hidradenitis suppurativa 10.1
42 hidradenitis 10.1
43 mediastinal endodermal sinus tumors 10.1
44 factor xiii deficiency 10.1 F8 SERPINF2
45 hemorrhagic disease 10.1 F8 SERPINA1 SERPINF2
46 rubeosis iridis 10.0 SERPINF2 SNRPE
47 rheumatoid arthritis 10.0
48 varicose veins 10.0
49 reticulum cell sarcoma 10.0
50 focal segmental glomerulosclerosis 1 10.0

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:

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

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:

42
Skin, Eye, Bone, Lung, Brain, Colon, Bone Marrow

Publications for Nodular Nonsuppurative Panniculitis

Articles related to Nodular Nonsuppurative Panniculitis:

(show top 50) (show all 164)
# 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
Skin lesions depicting a systemic disease. Weber-Christian disease. ( 21325702 )
2011
11
A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report. ( 20105325 )
2010
12
Chorioretinitis associated with weber-christian disease. ( 25391077 )
2009
13
Weber and Christian of Weber-Christian disease. ( 18401882 )
2008
14
Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema. ( 16550303 )
2007
15
Childhood Weber-Christian disease: clinical investigation and virus detection. ( 17888054 )
2007
16
A case of Weber-Christian disease with collapsing glomerulopathy. ( 16931223 )
2006
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
Weber-Christian disease with severe polyarthritis and polyosteitis. ( 12022332 )
2002
23
The men behind the eponym: Frederick Parkes Weber and Henry a. Christian: Weber-Christian disease. ( 12454605 )
2002
24
Hepatic Weber-Christian disease. ( 11296691 )
2001
25
Osteoarthropathy associated with Weber-Christian disease. ( 11550989 )
2001
26
Weber Christian disease in a type 2 diabetic patient. ( 11584948 )
2001
27
Weber-Christian disease presenting with proptosis: a case report. ( 10803707 )
2000
28
Systemic Weber-Christian disease complicated by partial transverse myelopathy. ( 11070575 )
2000
29
Systemic Weber-Christian disease. ( 11179936 )
2000
30
Steroid-resistant Weber-Christian Disease. ( 10435355 )
1999
31
Central serous retinopathy associated with Weber-Christian disease. ( 10435428 )
1999
32
Weber-Christian disease associated with lupus anticoagulant and anticardiolipin antibodies. ( 10501442 )
1999
33
Treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil. ( 9738797 )
1998
34
Proptosis as the manifesting sign of Weber-Christian disease. ( 9222254 )
1997
35
Nodular nonsuppurative panniculitis in association with primary biliary cirrhosis and Hashimoto's thyroiditis. ( 8686385 )
1996
36
Weber-Christian disease with calcinosis: a case report. ( 8973879 )
1996
37
Recognizing Weber-Christian disease. ( 8987390 )
1996
38
Subarachnoid hemorrhage of unknown origin associated with Weber-Christian disease--case report. ( 7477690 )
1995
39
Weber-Christian disease with benign cytophagic histiocytes in the skin lesion. ( 7754752 )
1995
40
Glucocorticoid-unresponsive fever in a patient with Weber-Christian disease. ( 8031696 )
1994
41
Mammary calcifications in Weber-Christian disease. ( 8168588 )
1994
42
Cultivation of Borrelia burgdorferi from the blood and a subcutaneous lesion of a patient with relapsing febrile nodular nonsuppurative panniculitis. ( 1538171 )
1992
43
Myalgia as the major symptom in systemic panniculitis (Weber-Christian disease). ( 1490498 )
1992
44
Cardiac manifestations of Weber-Christian disease: report and review of the literature. ( 1865427 )
1991
45
Weber-Christian disease. An unusual occurrence in a 12-year-old child. ( 2361793 )
1990
46
Weber-Christian disease producing splenic vein occlusion and bleeding gastric varices: successful treatment with sclerotherapy. ( 2385562 )
1990
47
Cytophagic panniculitis and lupus panniculitis versus Weber-Christian disease. ( 2741978 )
1989
48
Systemic Weber-Christian disease with reversible bilateral ureteric obstruction. ( 2608584 )
1989
49
Dyslipoproteinemia and an inhibitor of lipolytic enzymes in Weber-Christian disease. ( 2739146 )
1989
50
Weber-Christian disease associated with multiple perforations of the ileum and colon. ( 2741893 )
1989

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 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

Sources for Nodular Nonsuppurative Panniculitis

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
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