MCID: SBC012
MIFTS: 44

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 52 58 54 15 17 71
Subcorneal Pustular Dermatitis 52 58 32
Sneddon-Wilkinson Disease 52 58
Pustulosis Subcornealis 52 58
Sneddon-Wilkinson Disease or Syndrome 12
Sneddon Wilkinson Disease 52

Characteristics:

Orphanet epidemiological data:

58
subcorneal pustular dermatosis
Inheritance: Not applicable; Age of onset: Adult; Age of death: normal life expectancy;

Classifications:

Orphanet: 58  
Rare skin diseases


Summaries for Subcorneal Pustular Dermatosis

NIH Rare Diseases : 52 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 the 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 pyoderma and pemphigus vegetans, and has symptoms including pustular rash An important gene associated with Subcorneal Pustular Dermatosis is DSC1 (Desmocollin 1), 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 hyperpigmentation of the skin

Wikipedia : 74 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 109)
# Related Disease Score Top Affiliating Genes
1 pyoderma 31.2 DSG3 DSG1
2 pemphigus vegetans 30.2 PPL DSG1 DSC3
3 thymoma 29.7 PPL EVPL DSP
4 iga pemphigus 29.6 PPL EVPL DSG3 DSG1 DSC3 DSC1
5 skin disease 29.0 DST DSP DSG3 DSG1 DSC3 DSC1
6 pemphigus foliaceus 29.0 PPL EVPL DST DSG3 DSG1
7 bullous pemphigoid 28.8 PPL EVPL DST DSP DSG3 DSG1
8 pemphigus 28.3 PPL EVPL DST DSP DSG3 DSG1
9 pemphigus vulgaris, familial 27.7 PPL EVPL DST DSP DSG3 DSG1
10 pyoderma gangrenosum 10.8
11 pustular psoriasis 10.7
12 pustulosis of palm and sole 10.7
13 psoriasis 10.7
14 rheumatoid arthritis 10.6
15 myeloma, multiple 10.5
16 arthritis 10.5
17 dermatitis herpetiformis, familial 10.5
18 hypopyon 10.5
19 dermatitis 10.5
20 dermatitis herpetiformis 10.5
21 psoriatic arthritis 10.4
22 inflammatory bowel disease 10.4
23 allergic hypersensitivity disease 10.4
24 diffuse scleroderma 10.4
25 systemic scleroderma 10.4
26 monoclonal gammopathy of uncertain significance 10.4
27 crohn's disease 10.4
28 seminoma 10.3
29 hepatocellular carcinoma 10.3
30 osteomyelitis 10.3
31 bone inflammation disease 10.3
32 acute generalized exanthematous pustulosis 10.3
33 autoimmune disease 10.2
34 multiple sclerosis 10.2
35 systemic lupus erythematosus 10.2
36 leprosy 3 10.2
37 thymoma, familial 10.2
38 dermatitis, atopic 10.2
39 aplastic anemia 10.2
40 human immunodeficiency virus type 1 10.2
41 psoriasis 14, pustular 10.2
42 pulmonary fibrosis and/or bone marrow failure, telomere-related, 2 10.2
43 deficiency anemia 10.2
44 marginal zone b-cell lymphoma 10.2
45 colitis 10.2
46 nodal marginal zone lymphoma 10.2
47 lepromatous leprosy 10.2
48 goiter 10.2
49 graves' disease 10.2
50 urethritis 10.2

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:

58 31 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 erythema 58 31 hallmark (90%) Very frequent (99-80%) HP:0010783
2 hyperpigmentation of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0000953
3 pustule 58 31 hallmark (90%) Very frequent (99-80%) HP:0200039
4 pruritus 58 31 frequent (33%) Frequent (79-30%) HP:0000989
5 hypothyroidism 58 31 occasional (7.5%) Occasional (29-5%) HP:0000821
6 hyperthyroidism 58 31 occasional (7.5%) Occasional (29-5%) HP:0000836
7 increased antibody level in blood 58 31 occasional (7.5%) Occasional (29-5%) HP:0010702
8 systemic lupus erythematosus 58 31 occasional (7.5%) Occasional (29-5%) HP:0002725
9 rheumatoid arthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001370
10 multiple myeloma 58 31 occasional (7.5%) Occasional (29-5%) HP:0006775
11 autoimmunity 58 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 Decreased viability after Maraba virus infection GR00252-A-1 9.23 CD79A DSG1 DSP ST18
2 Decreased viability after Maraba virus infection GR00252-A-2 9.23 CD79A DSG1
3 Decreased viability after Maraba virus infection GR00252-A-3 9.23 DSP ST18

MGI Mouse Phenotypes related to Subcorneal Pustular Dermatosis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.17 DSC1 DSC3 DSG1 DSG3 DSP 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 A Phase 2 Study of Anakinra in Inflammatory Pustular Dermatoses: Evaluation of Therapeutic Efficacy and Validation of Pathogenic Mechanisms Completed NCT01794117 Phase 2 Anakinra

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:

40
Skin, Neutrophil, Bone, Spleen, Lung, Lymph Node, Small Intestine

Publications for Subcorneal Pustular Dermatosis

Articles related to Subcorneal Pustular Dermatosis:

(show top 50) (show all 289)
# Title Authors PMID Year
1
Subcorneal Pustular Dermatosis: A Review of 30 Years of Progress. 61 52
27349653 2016
2
Subcorneal pustular dermatosis: 50 years on. 61 52
18355359 2008
3
Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. 54 61
19841404 2009
4
Immunoglobulin A pemphigus associated with immunoglobulin A gammopathy and lung cancer. 54 61
18578711 2008
5
Subcorneal pustular dermatosis (Sneddon-Wilkinson disease) with absence of desmoglein 1 and 3 antibodies: case report and literature review. 54 61
18092844 2008
6
IgA pemphigus--occurrence of anti-desmocollin 1 and anti-desmoglein 1 antibody reactivity in an individual patient. 54 61
17176412 2006
7
Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. 54 61
16112379 2005
8
Regression of subcorneal pustular dermatosis type of IgA pemphigus lesions with azithromycin. 54 61
16038746 2005
9
Two Brazilian cases of IgA pemphigus. 54 61
14739515 2003
10
Detection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus. 54 61
11405762 2001
11
Rapid response of IgA pemphigus of subcorneal pustular dermatosis type to treatment with isotretinoin. 54 61
11044824 2000
12
Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review. 54 61
10886149 2000
13
Intercellular IgA dermatosis (IgA pemphigus)--two cases illustrating the clinical heterogeneity of this disorder. 54 61
10606950 1999
14
Human desmocollin 1a transiently expressed in COS-7 cells and NIH 3T3-3 cells is reacted by IgG4 antibodies in a pemphigus foliaceus serum. 54 61
10468191 1999
15
Effect of colchicine in the subcorneal pustular dermatosis type of IgA pemphigus. 54 61
9922018 1999
16
Human desmocollin 1 (Dsc1) is an autoantigen for the subcorneal pustular dermatosis type of IgA pemphigus. 54 61
9242496 1997
17
Studies of autoantigens recognized by IgA anti-keratinocyte cell surface antibodies. 54 61
8740455 1996
18
Subcorneal pustular dermatosis associated with IgA myeloma and intraepidermal IgA deposits. 54 61
8049547 1994
19
Subcorneal pustular dermatosis associated with rheumatoid arthritis and raised IgA: simultaneous remission of skin and joint involvements with dapsone treatment. 54 61
2181947 1990
20
The Striking Appearance of a Rare Skin Condition: "Half-Half" Blisters (Subcorneal Pustular Dermatosis). 61
31611152 2020
21
Successful treatment of recalcitrant subcorneal pustular dermatosis with oral nicotinamide. 61
31187880 2019
22
Topical dapsone gel for treatment of axillary subcorneal pustular dermatosis. 61
31232481 2019
23
Sneddon-Wilkinson Disease and Monoclonal Gammopathy of Undetermined Significance in the Elderly: Case Report. 61
31427943 2019
24
Pentoxyfilline as a treatment for subcorneal pustular dermatosis. 61
30637905 2019
25
Chronic annular pustular psoriasis resembling subcorneal pustular dermatosis: A case report. 61
31258906 2019
26
Imatinib-induced IgA Pemphigus: Subcorneal Pustular Dermatosis Type. 61
30258803 2018
27
A Case of the Co-Existence of Subcorneal Pustular Dermatosis and Pyoderma Gangrenosum and a Review of the Literature. 61
30087735 2018
28
Neutrophilic dermatoses: a broad spectrum of disease. 61
29368857 2018
29
Subcorneal Pustular Dermatosis: A Case Report of a Patient with Diffuse Scleroderma. 61
29930923 2018
30
Subcorneal pustular dermatosis in the pediatric age. 61
29641711 2018
31
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. 61
27516004 2017
32
Bullous, pseudobullous, & pustular dermatoses. 61
28108048 2017
33
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. 61
27258999 2017
34
Interruption of Sneddon-Wilkinson Subcorneal Pustulation with Infliximab. 61
28559813 2017
35
Subcorneal pustular dermatosis and episcleritis associated with poorly controlled ulcerative colitis. 61
28137903 2017
36
A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease). 61
29491542 2017
37
Prolonged pustular eruption from hydroxychloroquine: an unusual case of acute generalized exanthematous pustulosis. 61
27023083 2016
38
Classification of Extrapulmonary Manifestations Due to Mycoplasma pneumoniae Infection on the Basis of Possible Pathogenesis. 61
26858701 2016
39
Successful treatment of subcorneal pustular dermatosis with maxacalcitol. 61
25991581 2016
40
A case of subcorneal pustular dermatosis with IgG monoclonal gammopathy of undetermined significance: a rare association. 61
26341517 2015
41
Scabies Incognito Presenting as a Subcorneal Pustular Dermatosis-like Eruption. 61
26470936 2015
42
Subcorneal pustular dermatosis associated with Coccidioides immitis. 61
26437157 2015
43
PUM I Revisited: Tradeoffs in Preservation and Discovery. 61
25998631 2015
44
Dinotefuran/pyriproxyfen/permethrin pemphigus-like drug reaction in three dogs. 61
25758668 2015
45
Subcorneal pustular dermatosis triggered by Mycoplasma pneumoniae infection: a rare clinical association. 61
24650287 2015
46
When Rings Are Not Ringworms: Case Reports and Review of Literature. 61
26244204 2015
47
Antidesmocollin 1 autoantibody negative subcorneal pustular dermatosis-type IgA pemphigus associated with multiple myeloma. 61
24980095 2015
48
Sneddon-Wilkinson disease induced by sorafenib in a patient with advanced hepatocellular carcinoma. 61
25786448 2015
49
The expanding spectrum of IgA pemphigus: a case report and review of the literature. 61
24601812 2014
50
Subcorneal pustular dermatosis associated with Mycoplasma pneumoniae infection. 61
25128138 2014

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 PPL EVPL DSP DSG3 DSG1 DSC3
2
Show member pathways
11.62 PPL EVPL DSP DSG3 DSG1 DSC3
3
Show member pathways
11.5 DSP DSG3 DSG1
4
Show member pathways
11.29 PPL EVPL DST DSP

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 membrane GO:0016020 10.13 PPL FCAR EVPL DST DSP DSG3
2 plasma membrane GO:0005886 10.06 PPL FCAR DST DSP DSG3 DSG1
3 cell junction GO:0030054 9.76 PPL EVPL DST DSP DSG3 DSG1
4 cell-cell junction GO:0005911 9.72 DSP DSG3 DSG1 DSC3 DSC1
5 intermediate filament GO:0005882 9.67 PPL EVPL DST DSP
6 ficolin-1-rich granule membrane GO:0101003 9.56 FCAR DSP DSG1 DSC1
7 desmosome GO:0030057 9.5 PPL EVPL DSP DSG3 DSG1 DSC3
8 cornified envelope GO:0001533 9.17 PPL EVPL DSP DSG3 DSG1 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 DST DSG3 DSG1 DSC3 DSC1
2 neutrophil degranulation GO:0043312 9.73 FCAR DSP DSG1 DSC1
3 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 9.67 DSG3 DSG1 DSC3 DSC1
4 cell-cell adhesion GO:0098609 9.65 DSP DSG3 DSG1 DSC3 DSC1
5 wound healing GO:0042060 9.62 PPL EVPL DST DSP
6 keratinization GO:0031424 9.5 PPL EVPL DSP DSG3 DSG1 DSC3
7 keratinocyte differentiation GO:0030216 9.48 EVPL DSP
8 peptide cross-linking GO:0018149 9.46 EVPL DSP
9 intermediate filament cytoskeleton organization GO:0045104 9.46 PPL EVPL DST DSP
10 cornification GO:0070268 9.17 PPL EVPL DSP DSG3 DSG1 DSC3

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 9.02 DST DSG3 DSG1 DSC3 DSC1
2 gamma-catenin binding GO:0045295 8.96 DSG1 DSC3

Sources for Subcorneal Pustular Dermatosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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