MCID: SBC012
MIFTS: 50

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 53 59 55 15 73
Subcorneal Pustular Dermatitis 53 59
Sneddon-Wilkinson Disease 53 59
Pustulosis Subcornealis 53 59
Sneddon-Wilkinson Disease or Syndrome 12
Sneddon Wilkinson Disease 53

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

NIH Rare Diseases : 53 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, bone and lymph node, and related phenotypes are hypothyroidism and pruritus

Wikipedia : 76 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 67)
# Related Disease Score Top Affiliating Genes
1 pemphigus 30.5 CD79A COL17A1 DSC1 DSC3 DSG1 DSG3
2 bullous pemphigoid 30.1 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 diffuse scleroderma 10.3
13 dermatitis 10.3
14 dermatitis herpetiformis 10.3
15 bullous impetigo 10.2 DSG1 DSG3
16 transverse colon cancer 10.2 DSC2 DSC3
17 bone inflammation disease 10.2
18 lichen planus pemphigoides 10.2 COL17A1 DST
19 pemphigoid gestationis 10.2 COL17A1 DST
20 multiple sclerosis 10.2
21 systemic lupus erythematosus 10.2
22 aplastic anemia 10.2
23 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 10.2
24 ulcerative colitis 10.2
25 lymphoma 10.2
26 colitis 10.2
27 nodal marginal zone lymphoma 10.2
28 lepromatous leprosy 10.2
29 goiter 10.2
30 sapho syndrome 10.2
31 polyneuropathy 10.2
32 monoclonal gammopathy of uncertain significance 10.2
33 hyperthyroidism 10.2
34 scabies 10.2
35 lupus erythematosus 10.2
36 dermatophytosis 10.2
37 neurotic excoriation 10.2 COL17A1 DST
38 pemphigus vegetans 10.2 DSC3 DSG1 PPL
39 herpes gestationis 10.2 COL17A1 DST
40 impetigo herpetiformis 10.2 DST IL36RN
41 discoid lupus erythematosus 10.1
42 herpetiform pemphigus 10.1 DSG1 DSG3 DST
43 linear iga disease 10.1 CD79A COL17A1 DST
44 pustulosis of palm and sole 10.1 IL36RN TNFRSF1B
45 dowling-degos disease 10.1 ATP2A2 DSC2 DSC3
46 lichen disease 10.1 COL17A1 DSG1 DST
47 lichen planus 10.1 COL17A1 DSG3 DST
48 solitary osseous plasmacytoma 10.1 CD79A MYOM2
49 epidermolysis bullosa simplex with muscular dystrophy 10.1 COL17A1 DST
50 osteosclerotic myeloma 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:

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 pruritus 59 32 frequent (33%) Frequent (79-30%) HP:0000989
3 hyperthyroidism 59 32 occasional (7.5%) Occasional (29-5%) HP:0000836
4 erythema 59 32 hallmark (90%) Very frequent (99-80%) HP:0010783
5 increased antibody level in blood 59 32 occasional (7.5%) Occasional (29-5%) HP:0010702
6 rheumatoid arthritis 59 32 occasional (7.5%) Occasional (29-5%) HP:0001370
7 systemic lupus erythematosus 59 32 occasional (7.5%) Occasional (29-5%) HP:0002725
8 pustule 59 32 hallmark (90%) Very frequent (99-80%) HP:0200039
9 hyperpigmentation of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0000953
10 multiple myeloma 59 32 occasional (7.5%) Occasional (29-5%) HP:0006775
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 (show all 12)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00106-A-0 10.33 RPS6
2 Decreased viability GR00107-A-1 10.33 ZAP70
3 Decreased viability GR00221-A-1 10.33 ZAP70
4 Decreased viability GR00221-A-2 10.33 ZAP70
5 Decreased viability GR00240-S-1 10.33 CCL17 MYOM2
6 Decreased viability GR00342-S-1 10.33 ZAP70
7 Decreased viability GR00342-S-2 10.33 ZAP70
8 Decreased viability GR00381-A-1 10.33 CCL17 CD79A
9 Decreased viability GR00381-A-3 10.33 CD79A
10 Decreased viability GR00402-S-2 10.33 ATP2A2 CCL17 CD79A CDH17 COL17A1 DSC1
11 no effect GR00402-S-1 9.93 ATP2A2 CCL17 CD79A CDH17 COL17A1 DSC1
12 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 cellular MP:0005384 9.65 ATP2A2 CCL17 CD79A DSC1 DSC3 DSG3
2 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, Bone, Lymph Node, Spleen, Colon, B Cells, Bone Marrow

Publications for Subcorneal Pustular Dermatosis

Articles related to Subcorneal Pustular Dermatosis:

(show top 50) (show all 169)
# 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
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
A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease). ( 29491542 )
2017
6
Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis. ( 28137903 )
2017
7
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
8
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
9
Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. ( 27349653 )
2016
10
A case of subcorneal pustular dermatosis with IgG monoclonal gammopathy of undetermined significance: a rare association. ( 26341517 )
2015
11
Scabies Incognito Presenting as a Subcorneal Pustular Dermatosis-like Eruption. ( 26470936 )
2015
12
Subcorneal pustular dermatosis associated with Coccidioides immitis. ( 26437157 )
2015
13
Successful treatment of subcorneal pustular dermatosis with maxacalcitol. ( 25991581 )
2015
14
Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma. ( 24980095 )
2015
15
Subcorneal pustular dermatosis triggered by Mycoplasma pneumoniae infection: a rare clinical association. ( 24650287 )
2015
16
Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. ( 25128138 )
2014
17
Subcorneal pustular dermatosis in childhood: a case report and review of the literature. ( 23365765 )
2013
18
Palmoplantar subcorneal pustular dermatosis following adalimumab therapy for rheumatoid arthritis. ( 23489057 )
2013
19
Subcorneal Pustular Dermatosis Exhibiting a High Serum TARC/CCL17 Level. ( 23525135 )
2013
20
Subcorneal pustular dermatosis masquerading as dermatophytosis. ( 23189262 )
2012
21
Subcorneal pustular dermatosis an immnohisto-pathological perspective. ( 21738824 )
2011
22
A case of subcorneal pustular dermatosis in association with monoclonal IgA gammopathy successfully treated with acitretin. ( 20136575 )
2010
23
Subcorneal pustular dermatosis treated successfully with adalimumab. ( 20395190 )
2010
24
Unilateral and distal subcorneal pustular dermatosis and persistent polyclonal immunoglobulin M hyperimmunoglobulinemia. ( 20860743 )
2010
25
A case of bullous pemphigoid clinically mimicking subcorneal pustular dermatosis. ( 18759797 )
2009
26
Subcorneal pustular dermatosis with pathergy phenomenon in a patient with Crohn's disease. ( 18624859 )
2009
27
Subcorneal pustular dermatosis in a patient with rheumatoid arthritis and diffuse scleroderma. ( 19379649 )
2009
28
Pyoderma gangrenosum associated with subcorneal pustular dermatosis and IgA myeloma. ( 18627386 )
2009
29
Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. ( 19841404 )
2009
30
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) occurring in association with nodal marginal zone lymphoma: a case report. ( 19061566 )
2008
31
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with absence of desmoglein 1 and 3 antibodies: case report and literature review. ( 18092844 )
2008
32
Subcorneal pustular dermatosis: 50 years on. ( 18355359 )
2008
33
Successful treatment of long-standing, recalcitrant subcorneal pustular dermatosis with etanercept. ( 17786105 )
2007
34
A case of subcorneal pustular dermatosis treated successfully with a combination of cyclosporin and prednisolone. ( 17373984 )
2007
35
A case of juvenile subcorneal pustular dermatosis successfully treated with acitretin. ( 16961541 )
2006
36
Subcorneal pustular dermatosis in association with a monoclonal IgA/k gammopathy: successful treatment with acitretin. ( 17101491 )
2006
37
Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. ( 16112379 )
2005
38
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
39
Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. ( 16038746 )
2005
40
Subcorneal pustular dermatosis and SjAPgren's syndrome. ( 16336533 )
2005
41
Subcorneal pustular dermatosis associated with palmo-plantar pustular psoriasis: response to colchicine therapy. ( 15729873 )
2004
42
Inverse subcorneal pustular dermatosis. ( 12702084 )
2003
43
Subcorneal pustular dermatosis and mycoplasma pneumoniae respiratory infection. ( 14609116 )
2003
44
Subcorneal pustular dermatosis developing in a patient with rheumatoid arthritis; rheumatoid, antimicrosomal, and antimitochondrial autoantibodies; and a goiter. ( 14673283 )
2003
45
Subcorneal pustular dermatosis in association with chronic lymphocytic leukaemia. ( 12926811 )
2003
46
Subcorneal pustular dermatosis in a patient with AIDS. ( 12834471 )
2003
47
Juvenile subcorneal pustular dermatosis: a case report. ( 12558849 )
2003
48
IgA pemphigus of the subcorneal pustular dermatosis type associated with monoclonal IgA gammopathy. ( 14761150 )
2003
49
Subcorneal pustular dermatosis accompanied by seronegative arthritis. ( 12361148 )
2002
50
Subcorneal pustular dermatosis-type IgA pemphigus induced by thiol drugs. ( 11174137 )
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 10.24 ATP2A2 CD79A CDH17 COL17A1 DSC1 DSC2
7 plasma membrane GO:0005886 10.18 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 9.61 ATP2A2 COL17A1 EVPL
4 wound healing GO:0042060 9.58 DST EVPL PPL
5 intermediate filament cytoskeleton organization GO:0045104 9.5 DST EVPL PPL
6 keratinization GO:0031424 9.5 DSC1 DSC2 DSC3 DSG1 DSG3 EVPL
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 9.23 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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