MCID: SBC012
MIFTS: 46

Subcorneal Pustular Dermatosis

Categories: Skin diseases, Rare diseases

Aliases & Classifications for Subcorneal Pustular Dermatosis

MalaCards integrated aliases for Subcorneal Pustular Dermatosis:

Name: Subcorneal Pustular Dermatosis 12 59 55 15 73
Sneddon-Wilkinson Disease or Syndrome 12
Subcorneal Pustular Dermatitis 59
Sneddon-Wilkinson Disease 59
Pustulosis Subcornealis 59

Characteristics:

Orphanet epidemiological data:

59
subcorneal pustular dermatosis
Inheritance: Not applicable; Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 59  
Rare skin diseases


Summaries for Subcorneal Pustular Dermatosis

MalaCards based summary : Subcorneal Pustular Dermatosis, also known as sneddon-wilkinson disease or syndrome, is related to pemphigus and bullous pemphigoid, and has symptoms including pustular rash An important gene associated with Subcorneal Pustular Dermatosis is DSC3 (Desmocollin 3), and among its related pathways/superpathways are Developmental Biology and Keratinization. The drugs Interleukin 1 Receptor Antagonist Protein and Antirheumatic Agents have been mentioned in the context of this disorder. Affiliated tissues include skin, neutrophil and small intestine, and related phenotypes are hypothyroidism and hyperthyroidism

Related Diseases for Subcorneal Pustular Dermatosis

Diseases related to Subcorneal Pustular Dermatosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 67)
# Related Disease Score Top Affiliating Genes
1 pemphigus 28.8 CD79A COL17A1 DSC1 DSC3 DSG1 DSG3
2 bullous pemphigoid 28.2 CCL17 CD79A COL17A1 DSG1 DSG3 DST
3 transverse colon cancer 10.7 DSC2 DSC3
4 bullous impetigo 10.7 DSG1 DSG3
5 syndromic x-linked intellectual disability cabezas type 10.6 DSC2 DSC3
6 lichen planus pemphigoides 10.6 COL17A1 DST
7 pemphigoid gestationis 10.6 COL17A1 DST
8 pompholyx 10.6 ATP2A2 COL17A1
9 neurotic excoriation 10.5 COL17A1 DST
10 pemphigus vegetans 10.5 DSC3 DSG1 PPL
11 yellow nail syndrome 10.5 ATP2A2 CD79A
12 impetigo 10.5 DSG1 IL36RN
13 herpes gestationis 10.5 COL17A1 DST
14 arthritis 10.5
15 pyoderma 10.5
16 pyoderma gangrenosum 10.5
17 herpetiform pemphigus 10.4 DSG1 DSG3 DST
18 linear iga disease 10.4 CD79A COL17A1 DST
19 vesiculobullous skin disease 10.4 CD79A COL17A1 DST
20 psoriasis 10.4
21 pustular psoriasis 10.4
22 epidermolysis bullosa acquisita 10.4 CD79A COL17A1 DST
23 epidermolysis bullosa, junctional, non-herlitz type 10.4 CD79A COL17A1 DST
24 hypersensitivity reaction disease 10.4 CD79A COL17A1 DST
25 rheumatoid arthritis 10.4
26 impetigo herpetiformis 10.3 DST IL36RN
27 lichen planus 10.3 COL17A1 DSG3 DST
28 pustulosis of palm and sole 10.3 IL36RN TNFRSF1B
29 pneumonia 10.3
30 rhinoscleroma 10.3 CD79A TNFRSF1B
31 epidermolysis bullosa simplex with muscular dystrophy 10.2 COL17A1 DST
32 lichen disease 10.2 CD79A COL17A1 DSG1 DST
33 myeloma, multiple 10.2
34 dermatitis herpetiformis, familial 10.2
35 crohn's disease 10.2
36 diffuse scleroderma 10.2
37 dermatitis 10.2
38 dermatitis herpetiformis 10.2
39 norwegian scabies 10.1 ATP2A2 CD79A TNFRSF1B
40 pityriasis rubra pilaris 10.0 ATP2A2 IL36RN TNFRSF1B
41 multiple sclerosis 10.0
42 systemic lupus erythematosus 10.0
43 aging 10.0
44 aplastic anemia 10.0
45 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 10.0
46 ulcerative colitis 10.0
47 lymphoma 10.0
48 colitis 10.0
49 nodal marginal zone lymphoma 10.0
50 goiter 10.0

Graphical network of the top 20 diseases related to Subcorneal Pustular Dermatosis:



Diseases related to Subcorneal Pustular Dermatosis

Symptoms & Phenotypes for Subcorneal Pustular Dermatosis

Human phenotypes related to Subcorneal Pustular Dermatosis:

59 32 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hypothyroidism 59 32 occasional (7.5%) Occasional (29-5%) HP:0000821
2 hyperthyroidism 59 32 occasional (7.5%) Occasional (29-5%) HP:0000836
3 hyperpigmentation of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0000953
4 pruritus 59 32 frequent (33%) Frequent (79-30%) HP:0000989
5 rheumatoid arthritis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001370
6 systemic lupus erythematosus 59 32 occasional (7.5%) Occasional (29-5%) HP:0002725
7 multiple myeloma 59 32 occasional (7.5%) Occasional (29-5%) HP:0006775
8 increased antibody level in blood 59 32 occasional (7.5%) Occasional (29-5%) HP:0010702
9 erythema 59 32 hallmark (90%) Very frequent (99-80%) HP:0010783
10 pustule 59 32 hallmark (90%) Very frequent (99-80%) HP:0200039
11 autoimmunity 59 Occasional (29-5%)

UMLS symptoms related to Subcorneal Pustular Dermatosis:


pustular rash

GenomeRNAi Phenotypes related to Subcorneal Pustular Dermatosis according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.1 ATP2A2 CD79A PUM1 RPS6 TNFRSF1B ZAP70

MGI Mouse Phenotypes related to Subcorneal Pustular Dermatosis:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.28 ATP2A2 DSC1 DSC3 DSG1 DSG3 EVPL

Drugs & Therapeutics for Subcorneal Pustular Dermatosis

Drugs for Subcorneal Pustular Dermatosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Interleukin 1 Receptor Antagonist Protein Phase 2
2 Antirheumatic Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Anakinra for Inflammatory Pustular Skin Diseases Recruiting NCT01794117 Phase 2 Anakinra
2 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275

Search NIH Clinical Center for Subcorneal Pustular Dermatosis

Genetic Tests for Subcorneal Pustular Dermatosis

Anatomical Context for Subcorneal Pustular Dermatosis

MalaCards organs/tissues related to Subcorneal Pustular Dermatosis:

41
Skin, Neutrophil, Small Intestine, Spleen, Lymph Node

Publications for Subcorneal Pustular Dermatosis

Articles related to Subcorneal Pustular Dermatosis:

(show top 50) (show all 160)
# Title Authors Year
1
Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma. ( 29930923 )
2018
2
Subcorneal pustular dermatosis in the pediatric age. ( 29641711 )
2018
3
A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease). ( 29491542 )
2017
4
Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis. ( 28137903 )
2017
5
Clinical and immunological studies of 49 cases of various types of intercellular IgA dermatosis and 13 cases of classical subcorneal pustular dermatosis examined at Kurume University. ( 27258999 )
2016
6
Complete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with anti-myeloma therapy: association with disappearance of M-protein. ( 27516004 )
2016
7
Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. ( 27349653 )
2016
8
A case of subcorneal pustular dermatosis with IgG monoclonal gammopathy of undetermined significance: a rare association. ( 26341517 )
2015
9
Scabies Incognito Presenting as a Subcorneal Pustular Dermatosis-like Eruption. ( 26470936 )
2015
10
Subcorneal pustular dermatosis associated with Coccidioides immitis. ( 26437157 )
2015
11
Successful treatment of subcorneal pustular dermatosis with maxacalcitol. ( 25991581 )
2015
12
Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma. ( 24980095 )
2015
13
Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. ( 25128138 )
2014
14
Subcorneal pustular dermatosis triggered by Mycoplasma pneumoniae infection: a rare clinical association. ( 24650287 )
2014
15
Subcorneal pustular dermatosis in childhood: a case report and review of the literature. ( 23365765 )
2013
16
Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis. ( 23489057 )
2013
17
Subcorneal Pustular Dermatosis Exhibiting a High Serum TARC/CCL17 Level. ( 23525135 )
2013
18
Subcorneal pustular dermatosis masquerading as dermatophytosis. ( 23189262 )
2012
19
Subcorneal pustular dermatosis an immnohisto-pathological perspective. ( 21738824 )
2011
20
A case of subcorneal pustular dermatosis in association with monoclonal IgA gammopathy successfully treated with acitretin. ( 20136575 )
2010
21
Subcorneal pustular dermatosis treated successfully with adalimumab. ( 20395190 )
2010
22
Unilateral and distal subcorneal pustular dermatosis and persistent polyclonal immunoglobulin M hyperimmunoglobulinemia. ( 20860743 )
2010
23
A case of bullous pemphigoid clinically mimicking subcorneal pustular dermatosis. ( 18759797 )
2009
24
Subcorneal pustular dermatosis with pathergy phenomenon in a patient with Crohn's disease. ( 18624859 )
2009
25
Subcorneal pustular dermatosis in a patient with rheumatoid arthritis and diffuse scleroderma. ( 19379649 )
2009
26
Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma. ( 18627386 )
2009
27
Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. ( 19841404 )
2009
28
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) occurring in association with nodal marginal zone lymphoma: a case report. ( 19061566 )
2008
29
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with absence of desmoglein 1 and 3 antibodies: case report and literature review. ( 18092844 )
2008
30
Subcorneal pustular dermatosis: 50 years on. ( 18355359 )
2008
31
Successful treatment of long-standing, recalcitrant subcorneal pustular dermatosis with etanercept. ( 17786105 )
2007
32
A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone. ( 17373984 )
2007
33
A case of juvenile subcorneal pustular dermatosis successfully treated with acitretin. ( 16961541 )
2006
34
Subcorneal pustular dermatosis in association with a monoclonal IgA/k gammopathy: successful treatment with acitretin. ( 17101491 )
2006
35
Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. ( 16112379 )
2005
36
Early but not lasting improvement of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease) after infliximab therapy: relationships with variations in cytokine levels in suction blister fluids. ( 16197383 )
2005
37
Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. ( 16038746 )
2005
38
Subcorneal pustular dermatosis and SjAPgren's syndrome. ( 16336533 )
2005
39
Subcorneal pustular dermatosis associated with palmo-plantar pustular psoriasis: response to colchicine therapy. ( 15729873 )
2004
40
Inverse subcorneal pustular dermatosis. ( 12702084 )
2003
41
Subcorneal pustular dermatosis and mycoplasma pneumoniae respiratory infection. ( 14609116 )
2003
42
Subcorneal pustular dermatosis developing in a patient with rheumatoid arthritis; rheumatoid, antimicrosomal, and antimitochondrial autoantibodies; and a goiter. ( 14673283 )
2003
43
Subcorneal pustular dermatosis in association with chronic lymphocytic leukaemia. ( 12926811 )
2003
44
Subcorneal pustular dermatosis in a patient with AIDS. ( 12834471 )
2003
45
Juvenile subcorneal pustular dermatosis: a case report. ( 12558849 )
2003
46
IgA pemphigus of the subcorneal pustular dermatosis type associated with monoclonal IgA gammopathy. ( 14761150 )
2003
47
Subcorneal pustular dermatosis accompanied by seronegative arthritis. ( 12361148 )
2002
48
Subcorneal pustular dermatosis-type IgA pemphigus induced by thiol drugs. ( 11174137 )
2001
49
Infliximab (anti-tumor necrosis factor alpha antibody): a novel, highly effective treatment of recalcitrant subcorneal pustular dermatosis (Sneddon-Wilkinson disease). ( 11735706 )
2001
50
Pyoderma gangrenosum, subcorneal pustular dermatosis, IgA paraproteinaemia and IgG antiepithelial antibodies. ( 11736923 )
2001

Variations for Subcorneal Pustular Dermatosis

Expression for Subcorneal Pustular Dermatosis

Search GEO for disease gene expression data for Subcorneal Pustular Dermatosis.

Pathways for Subcorneal Pustular Dermatosis

Pathways related to Subcorneal Pustular Dermatosis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.91 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
2
Show member pathways
11.62 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
3
Show member pathways
11.17 DST EVPL PPL

GO Terms for Subcorneal Pustular Dermatosis

Cellular components related to Subcorneal Pustular Dermatosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell junction GO:0030054 9.85 CDH17 COL17A1 DSC1 DSC2 DSC3 DSG1
2 cell-cell junction GO:0005911 9.56 COL17A1 DSC3 DSG1 ZAP70
3 cornified envelope GO:0001533 9.5 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
4 hemidesmosome GO:0030056 9.32 COL17A1 DST
5 desmosome GO:0030057 9.17 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
6 membrane GO:0016020 10.25 ATP2A2 CD79A CDH17 COL17A1 DSC1 DSC2
7 plasma membrane GO:0005886 10.15 CD79A CDH17 COL17A1 DSC1 DSC2 DSC3

Biological processes related to Subcorneal Pustular Dermatosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.86 ATP2A2 CDH17 DSC1 DSC2 DSC3 DSG1
2 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 9.63 CDH17 DSC1 DSC2 DSC3 DSG1 DSG3
3 epidermis development GO:0008544 9.5 ATP2A2 COL17A1 EVPL
4 keratinization GO:0031424 9.5 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
5 cornification GO:0070268 9.17 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL

Molecular functions related to Subcorneal Pustular Dermatosis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 calcium ion binding GO:0005509 9.23 ATP2A2 CDH17 DSC1 DSC2 DSC3 DSG1

Sources for Subcorneal Pustular Dermatosis

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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