MCID: SBC012
MIFTS: 47

Subcorneal Pustular Dermatosis

Categories: Blood diseases, Rare diseases, Skin diseases

Aliases & Classifications for Subcorneal Pustular Dermatosis

MalaCards integrated aliases for Subcorneal Pustular Dermatosis:

Name: Subcorneal Pustular Dermatosis 12 54 60 56 15 17 74
Subcorneal Pustular Dermatitis 54 60
Sneddon-Wilkinson Disease 54 60
Pustulosis Subcornealis 54 60
Sneddon-Wilkinson Disease or Syndrome 12
Sneddon Wilkinson Disease 54

Characteristics:

Orphanet epidemiological data:

60
subcorneal pustular dermatosis
Inheritance: Not applicable; Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 60  
Rare skin diseases


Summaries for Subcorneal Pustular Dermatosis

NIH Rare Diseases : 54 Subcorneal pustular dermatosis (SPD) is a rare skin disease in which pus-filled pimples or blisters (pustules) form under the top (subcorneal) layer of the skin. It is most common in middle-aged adults (particularly women) but can develop in children. Pustules usually appear over a few hours and grow together to form round or wavy patterns. They most often form in areas where the skin may touch or rub together, such as the groin area, underarms, inside the elbows, and behind the knees. The pustules may be mildly itchy or painful, but despite being pus filled, are not infected. The diagnosis of SPD is made based on the appearance of the pustules and theĀ  results of a skin biopsy (histologic findings). The cause of SPD is not known. There is currently no evidence it is inherited (no familial cases have been reported) and it is not contagious. SPD may be associated with other diseases or health problems including several autoimmune diseases, blood (hematologic) diseases, infections, and cancers. Rarely, it has been associated with taking certain medications (drug-induced SPD). SPD may be treated with is oral dapsone, which often improves symptoms within one month. However, the pustules may return when treatment is stopped. Other therapies have been tried with mixed results. While SPD may cause discomfort and cosmetic concerns, it typically does not affect overall health.

MalaCards based summary : Subcorneal Pustular Dermatosis, also known as subcorneal pustular dermatitis, 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 bone, and related phenotypes are erythema and pustule

Wikipedia : 77 IgA pemphigus is a subtype of pemphigus with two distinct... more...

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 64)
# Related Disease Score Top Affiliating Genes
1 pemphigus 30.1 CD79A COL17A1 DSC1 DSC3 DSG1 DSG3
2 bullous pemphigoid 29.9 CD79A COL17A1 DSG1 DSG3 DST EVPL
3 pyoderma 10.6
4 pyoderma gangrenosum 10.6
5 arthritis 10.6
6 psoriasis 10.6
7 pustular psoriasis 10.6
8 rheumatoid arthritis 10.5
9 myeloma, multiple 10.3
10 dermatitis herpetiformis, familial 10.3
11 crohn's disease 10.3
12 systemic scleroderma 10.3
13 diffuse scleroderma 10.3
14 dermatitis 10.3
15 dermatitis herpetiformis 10.3
16 bullous impetigo 10.3 DSG1 DSG3
17 transverse colon cancer 10.3 DSC2 DSC3
18 acute poststreptococcal glomerulonephritis 10.3 C5 CD79A
19 lichen planus pemphigoides 10.3 COL17A1 DST
20 pemphigoid gestationis 10.3 COL17A1 DST
21 bone inflammation disease 10.2
22 neurotic excoriation 10.2 COL17A1 DST
23 pemphigus vegetans 10.2 DSC3 DSG1 PPL
24 multiple sclerosis 10.2
25 systemic lupus erythematosus 10.2
26 aplastic anemia 10.2
27 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 10.2
28 marginal zone b-cell lymphoma 10.2
29 ulcerative colitis 10.2
30 lymphoma 10.2
31 colitis 10.2
32 nodal marginal zone lymphoma 10.2
33 lepromatous leprosy 10.2
34 goiter 10.2
35 sapho syndrome 10.2
36 polyneuropathy 10.2
37 monoclonal gammopathy of uncertain significance 10.2
38 hyperthyroidism 10.2
39 scabies 10.2
40 lupus erythematosus 10.2
41 dermatophytosis 10.2
42 herpetiform pemphigus 10.2 DSG1 DSG3 DST
43 linear iga disease 10.2 CD79A COL17A1 DST
44 discoid lupus erythematosus 10.2
45 lichen disease 10.2 COL17A1 DSG1 DST
46 impetigo 10.2 DSG1 MYOM2
47 autoimmune disease 10.1 COL17A1 DSG1 ZAP70
48 epidermolysis bullosa simplex with muscular dystrophy 10.1 COL17A1 DST
49 lichen planus 10.1 COL17A1 DSG3 DST
50 solitary osseous plasmacytoma 10.1 CD79A MYOM2

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:

60 33 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 erythema 60 33 hallmark (90%) Very frequent (99-80%) HP:0010783
2 pustule 60 33 hallmark (90%) Very frequent (99-80%) HP:0200039
3 hyperpigmentation of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0000953
4 pruritus 60 33 frequent (33%) Frequent (79-30%) HP:0000989
5 hypothyroidism 60 33 occasional (7.5%) Occasional (29-5%) HP:0000821
6 hyperthyroidism 60 33 occasional (7.5%) Occasional (29-5%) HP:0000836
7 increased antibody level in blood 60 33 occasional (7.5%) Occasional (29-5%) HP:0010702
8 rheumatoid arthritis 60 33 occasional (7.5%) Occasional (29-5%) HP:0001370
9 systemic lupus erythematosus 60 33 occasional (7.5%) Occasional (29-5%) HP:0002725
10 multiple myeloma 60 33 occasional (7.5%) Occasional (29-5%) HP:0006775
11 autoimmunity 60 Occasional (29-5%)

UMLS symptoms related to Subcorneal Pustular Dermatosis:


pustular rash

MGI Mouse Phenotypes related to Subcorneal Pustular Dermatosis:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.17 DSC1 DSC3 DSG1 DSG3 EVPL TNFRSF1B

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:

42
Skin, Neutrophil, Bone, Lymph Node, Spleen, Small Intestine, Bone Marrow

Publications for Subcorneal Pustular Dermatosis

Articles related to Subcorneal Pustular Dermatosis:

(show top 50) (show all 171)
# Title Authors Year
1
Pentoxyfilline as a treatment for subcorneal pustular dermatosis. ( 30637905 )
2019
2
Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma. ( 29930923 )
2018
3
Imatinib-induced IgA Pemphigus: Subcorneal Pustular Dermatosis Type. ( 30258803 )
2018
4
A Case of the Co-Existence of Subcorneal Pustular Dermatosis and Pyoderma Gangrenosum and a Review of the Literature. ( 30087735 )
2018
5
Subcorneal pustular dermatosis in the pediatric age. ( 29641711 )
2018
6
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 )
2017
7
Complete remission of skin lesions in a patient with subcorneal pustular dermatosis (Sneddon-Wilkinson disease) treated with antimyeloma therapy: association with disappearance of M-protein. ( 27516004 )
2017
8
Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis. ( 28137903 )
2017
9
A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease). ( 29491542 )
2017
10
Successful treatment of subcorneal pustular dermatosis with maxacalcitol. ( 25991581 )
2016
11
Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. ( 27349653 )
2016
12
Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma. ( 24980095 )
2015
13
Scabies Incognito Presenting as a Subcorneal Pustular Dermatosis-like Eruption. ( 26470936 )
2015
14
Subcorneal pustular dermatosis triggered by Mycoplasma pneumoniae infection: a rare clinical association. ( 24650287 )
2015
15
A case of subcorneal pustular dermatosis with IgG monoclonal gammopathy of undetermined significance: a rare association. ( 26341517 )
2015
16
Subcorneal pustular dermatosis associated with Coccidioides immitis. ( 26437157 )
2015
17
Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. ( 25128138 )
2014
18
Subcorneal pustular dermatosis in childhood: a case report and review of the literature. ( 23365765 )
2013
19
Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis. ( 23489057 )
2013
20
Subcorneal Pustular Dermatosis Exhibiting a High Serum TARC/CCL17 Level. ( 23525135 )
2013
21
Subcorneal pustular dermatosis masquerading as dermatophytosis. ( 23189262 )
2012
22
Subcorneal pustular dermatosis an immnohisto-pathological perspective. ( 21738824 )
2011
23
A case of subcorneal pustular dermatosis in association with monoclonal IgA gammopathy successfully treated with acitretin. ( 20136575 )
2010
24
Subcorneal pustular dermatosis treated successfully with adalimumab. ( 20395190 )
2010
25
Unilateral and distal subcorneal pustular dermatosis and persistent polyclonal immunoglobulin M hyperimmunoglobulinemia. ( 20860743 )
2010
26
A case of bullous pemphigoid clinically mimicking subcorneal pustular dermatosis. ( 18759797 )
2009
27
Subcorneal pustular dermatosis in a patient with rheumatoid arthritis and diffuse scleroderma. ( 19379649 )
2009
28
Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. ( 19841404 )
2009
29
Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma. ( 18627386 )
2009
30
Subcorneal pustular dermatosis with pathergy phenomenon in a patient with Crohn's disease. ( 18624859 )
2009
31
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) occurring in association with nodal marginal zone lymphoma: a case report. ( 19061566 )
2008
32
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with absence of desmoglein 1 and 3 antibodies: case report and literature review. ( 18092844 )
2008
33
Subcorneal pustular dermatosis: 50 years on. ( 18355359 )
2008
34
A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone. ( 17373984 )
2007
35
Successful treatment of long-standing, recalcitrant subcorneal pustular dermatosis with etanercept. ( 17786105 )
2007
36
A case of juvenile subcorneal pustular dermatosis successfully treated with acitretin. ( 16961541 )
2006
37
Subcorneal pustular dermatosis in association with a monoclonal IgA/k gammopathy: successful treatment with acitretin. ( 17101491 )
2006
38
Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. ( 16038746 )
2005
39
Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. ( 16112379 )
2005
40
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
41
Subcorneal pustular dermatosis and Sjögren's syndrome. ( 16336533 )
2005
42
Subcorneal pustular dermatosis associated with palmo-plantar pustular psoriasis: response to colchicine therapy. ( 15729873 )
2004
43
Subcorneal pustular dermatosis developing in a patient with rheumatoid arthritis; rheumatoid, antimicrosomal, and antimitochondrial autoantibodies; and a goiter. ( 14673283 )
2003
44
IgA pemphigus of the subcorneal pustular dermatosis type associated with monoclonal IgA gammopathy. ( 14761150 )
2003
45
Juvenile subcorneal pustular dermatosis: a case report. ( 12558849 )
2003
46
Inverse subcorneal pustular dermatosis. ( 12702084 )
2003
47
Subcorneal pustular dermatosis in a patient with AIDS. ( 12834471 )
2003
48
Subcorneal pustular dermatosis in association with chronic lymphocytic leukaemia. ( 12926811 )
2003
49
Subcorneal pustular dermatosis and mycoplasma pneumoniae respiratory infection. ( 14609116 )
2003
50
Subcorneal pustular dermatosis accompanied by seronegative arthritis. ( 12361148 )
2002

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 CD79A CDH17 COL17A1 DSC1 DSC2 DSC3
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 9.8 CDH17 DSC1 DSC2 DSC3 DSG1 DSG3
2 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 9.63 CDH17 DSC1 DSC2 DSC3 DSG1 DSG3
3 wound healing GO:0042060 9.58 DST EVPL PPL
4 intermediate filament cytoskeleton organization GO:0045104 9.5 DST EVPL PPL
5 keratinization GO:0031424 9.5 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
6 B cell activation GO:0042113 9.43 CD79A ZAP70
7 hemidesmosome assembly GO:0031581 9.37 COL17A1 DST
8 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 9.17 CDH17 DSC1 DSC2 DSC3 DSG1 DSG3

Sources for Subcorneal Pustular Dermatosis

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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