MCID: NDL003
MIFTS: 46

Nodular Nonsuppurative Panniculitis

Categories: Rare diseases, Skin diseases, Muscle diseases

Aliases & Classifications for Nodular Nonsuppurative Panniculitis

MalaCards integrated aliases for Nodular Nonsuppurative Panniculitis:

Name: Nodular Nonsuppurative Panniculitis 12 53 15
Weber-Christian Disease 12 53 59
Relapsing Febrile Nodular Nonsuppurative Panniculitis 12 59
Panniculitis, Nodular Nonsuppurative 44 73
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
ICD10 33 M35.6
MeSH 44 D010201
Orphanet 59 ORPHA33577
MESH via Orphanet 45 D010201
UMLS via Orphanet 74 C0030328
ICD10 via Orphanet 34 M35.6
UMLS 73 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 localized lipodystrophy and phacolytic glaucoma. 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 lung, and related phenotypes are edema and subcutaneous nodule

Wikipedia : 76 Weber–Christian disease, also known as relapsing febrile nodular nonsuppurative panniculitis, is a... more...

Related Diseases for Nodular Nonsuppurative Panniculitis

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

(show all 39)
# Related Disease Score Top Affiliating Genes
1 localized lipodystrophy 11.4
2 phacolytic glaucoma 10.7 CRYAA SERPINA1
3 panniculitis 10.7
4 familial lipoprotein lipase deficiency 10.6 LIPC PNLIP
5 hepatic lipase deficiency 10.6 LIPC PNLIP
6 thyroid crisis 10.5 F8 POMC
7 congenital granular cell tumor 10.5 LYZ SERPINA1
8 sella turcica neoplasm 10.5 F8 POMC
9 tuberculum sellae meningioma 10.4 F8 POMC
10 adult xanthogranuloma 10.4 LYZ SERPINA1
11 rubeosis iridis 10.4 CRYAA SERPINF2 SNRPE
12 acute poststreptococcal glomerulonephritis 10.3 CFP SERPINF2
13 hemorrhagic disease 10.3 F8 SERPINA1 SERPINF2
14 mental retardation, skeletal dysplasia, and abducens palsy 10.2
15 cutaneous solitary mastocytoma 10.2 LYZ POMC SERPINA1
16 laryngeal tuberculosis 10.2 LYZ POMC
17 diffuse glomerulonephritis 10.2 CFP LYZ
18 biliary cirrhosis, primary, 1 10.0
19 rheumatic fever-related antigen 10.0
20 parkes weber syndrome 10.0
21 diabetes mellitus 10.0
22 primary biliary cirrhosis 10.0
23 jejunoileitis 10.0
24 uveitis 10.0
25 rheumatic fever 10.0
26 thyroiditis 10.0
27 amyloidosis 10.0
28 amyloidosis aa 10.0
29 primary biliary cholangitis 10.0
30 weber syndrome 10.0
31 blepharitis 10.0 CRYAA LYZ
32 cardiac tamponade 10.0 F8 TNFRSF1A
33 endocrine pancreas disease 9.8 IRS1 POMC SLC2A4
34 overnutrition 9.7 IRS1 POMC SLC2A4
35 glucose metabolism disease 9.7 IRS1 POMC SLC2A4
36 acquired metabolic disease 9.7 IRS1 POMC SLC2A4
37 periodic fever, familial, autosomal dominant 9.5 LYZ TNFRSF1A
38 cystic fibrosis 9.2 ELANE PNLIP SERPINA1 TNFRSF1A
39 body mass index quantitative trait locus 11 9.1 IRS1 LIPC PNLIP POMC SLC2A4

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 edema 59 32 hallmark (90%) Very frequent (99-80%) HP:0000969
2 subcutaneous nodule 59 32 hallmark (90%) Very frequent (99-80%) HP:0001482
3 splenomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0001744
4 weight loss 59 32 hallmark (90%) Very frequent (99-80%) HP:0001824
5 fever 59 32 hallmark (90%) Very frequent (99-80%) HP:0001945
6 nausea and vomiting 59 32 hallmark (90%) Very frequent (99-80%) HP:0002017
7 abdominal pain 59 32 hallmark (90%) Very frequent (99-80%) HP:0002027
8 hepatomegaly 59 32 occasional (7.5%) Occasional (29-5%) HP:0002240
9 arthralgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0002829
10 autoimmunity 59 32 occasional (7.5%) Occasional (29-5%) HP:0002960
11 myalgia 59 32 hallmark (90%) Very frequent (99-80%) HP:0003326
12 aplasia/hypoplasia of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0008065
13 erythema 59 32 hallmark (90%) Very frequent (99-80%) HP:0010783
14 panniculitis 59 32 hallmark (90%) Very frequent (99-80%) HP:0012490
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 10.1 KLK4 LIPC LYZ PNLIP POMC PPIA
2 cardiovascular system MP:0005385 10.02 CFP IRS1 LYZ POMC PPIA RASA1
3 immune system MP:0005387 9.93 CFP ELANE F8 IRS1 LYZ POMC
4 endocrine/exocrine gland MP:0005379 9.92 IRS1 LIPC LYZ POMC PPIA SLC2A4
5 adipose tissue MP:0005375 9.88 IRS1 LYZ PNLIP POMC SLC2A4 TNFRSF1A
6 liver/biliary system MP:0005370 9.56 TNFRSF1A IRS1 LYZ PNLIP POMC PPIA
7 mortality/aging MP:0010768 9.4 CFP ELANE F8 IRS1 KLK4 LYZ

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, Lung, Thyroid

Publications for Nodular Nonsuppurative Panniculitis

Articles related to Nodular Nonsuppurative Panniculitis:

(show all 24)
# Title Authors Year
1
Nodular nonsuppurative panniculitis in association with primary biliary cirrhosis and Hashimoto's thyroiditis. ( 8686385 )
1996
2
Cultivation of Borrelia burgdorferi from the blood and a subcutaneous lesion of a patient with relapsing febrile nodular nonsuppurative panniculitis. ( 1538171 )
1992
3
Amyloidosis (AA type) associated with nodular nonsuppurative panniculitis. ( 6625383 )
1983
4
Nodular nonsuppurative panniculitis associated with jejunoileal bypass surgery. ( 485188 )
1979
5
Generalized proteolysis in a young woman with Weber-Christian disease (nodular nonsuppurative panniculitis). ( 1215832 )
1975
6
Relapsing febrile nodular nonsuppurative panniculitis (Weber-Christian diseases). ( 14441580 )
1960
7
Nodular nonsuppurative panniculitis (Weber-Christian syndrome) with relapsing uveitis. ( 14409834 )
1959
8
Relapsing nodular nonsuppurative panniculitis with lung involvement: clinical and autopsy findings, with notes on pathogenesis. ( 13571854 )
1958
9
A follow-up on a case of nodular nonsuppurative panniculitis. ( 13410123 )
1957
10
Relapsing febrile nodular nonsuppurative panniculitis: Pfeifer-Weber-Christian disease; report of three cases. ( 13275145 )
1956
11
Relapsing febrile nodular nonsuppurative panniculitis (Weber-Christian syndrome): case report with response to roentgen therapy, and failure of cortisone. ( 14350473 )
1955
12
Weber-Christian disease: relapsing febrile nodular nonsuppurative panniculitis. ( 13115728 )
1954
13
Relapsing febrile nodular nonsuppurative panniculitis: Weber-Christian disease; case report. ( 12993371 )
1952
14
Relapsing febrile nodular nonsuppurative panniculitis (Weber-Christian syndrome). ( 14929371 )
1952
15
Relapsing febrile nodular nonsuppurative panniculitis (Weber-Christian disease); review of literature and report of case. ( 14830097 )
1951
16
Nodular nonsuppurative panniculitis (Weber-Christian disease). ( 14850312 )
1951
17
Chronic relapsing febrile nodular nonsuppurative panniculitis (Weber-Christian disease); relation to rheumatic fever and allied diseases. ( 14780325 )
1950
18
Relapsing febrile nodular nonsuppurative panniculitis; a report of a case with a review of the literature. ( 18140071 )
1949
19
Nodular nonsuppurative panniculitis, Weber-Christian disease; report of a case in which an infiltrate of polymorphonuclear leukocytes represented the earliest lesion. ( 18110556 )
1949
20
Nodular nonsuppurative panniculitis; Parkes-Weber syndrome. ( 18098648 )
1948
21
Nodular nonsuppurative panniculitis complicating diabetes mellitus. ( 18902469 )
1948
22
Relapsing febrile nodular nonsuppurative panniculitis; report of case treated with penicillin. ( 18892290 )
1948
23
Nodular nonsuppurative panniculitis treated with penicillin. ( 21022539 )
1946
24
Relapsing febrile nodular nonsuppurative panniculitis; with a report of a case. ( 21007324 )
1945

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 region GO:0005576 9.73 CFP ELANE F8 KLK4 LIPC LYZ
2 endoplasmic reticulum lumen GO:0005788 9.62 CFP F8 LIPC SERPINA1
3 secretory granule GO:0030141 9.54 ELANE KLK4 POMC
4 platelet alpha granule lumen GO:0031093 9.43 F8 SERPINA1 SERPINF2
5 extracellular space GO:0005615 9.36 CFP ELANE F8 LIPC LYZ PNLIP
6 specific granule lumen GO:0035580 9.33 CFP ELANE LYZ

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 9.55 CFP ELANE LYZ PPIA SERPINA1
2 platelet degranulation GO:0002576 9.5 F8 SERPINA1 SERPINF2
3 glucose homeostasis GO:0042593 9.43 IRS1 POMC SLC2A4
4 defense response to bacterium GO:0042742 9.26 CFP ELANE LYZ TNFRSF1A
5 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 protein binding GO:0005515 9.55 CFP CRYAA ELANE F8 IRS1 KLK4
2 triglyceride lipase activity GO:0004806 9.26 LIPC PNLIP
3 lipase activity GO:0016298 9.16 LIPC PNLIP
4 protease binding GO:0002020 9.13 ELANE SERPINA1 SERPINF2

Sources for Nodular Nonsuppurative Panniculitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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