MCID: PST059
MIFTS: 40

Pustular Psoriasis

Categories: Rare diseases, Genetic diseases, Blood diseases, Skin diseases, Bone diseases

Aliases & Classifications for Pustular Psoriasis

MalaCards integrated aliases for Pustular Psoriasis:

Name: Pustular Psoriasis 49 36 51 69

Classifications:



External Ids:

KEGG 36 H01663
UMLS 69 C0152081

Summaries for Pustular Psoriasis

NIH Rare Diseases : 49 Pustular psoriasis is a rare form of psoriasis that is characterized by widespread pustules and reddish skin. This condition can occur alone or with plaque-type psoriasis. Most cases of pustular psoriasis are thought to be "multifactorial" or associated with the effects of multiple genes in combination with lifestyle and environmental factors. There are several triggers for this conditions including withdrawal from corticosteroids, exposure to various medications and/or infections. Some cases of the generalized form are caused by changes (mutations) in the IL36RN gene and are inherited in an autosomal recessive pattern. In severe cases, hospitalization may be required. Treatment aims to alleviate the associated symptoms and may include certain medications and/or phototherapy. Last updated: 11/11/2015

MalaCards based summary : Pustular Psoriasis is related to psoriasis 14, pustular and pustulosis of palm and sole. An important gene associated with Pustular Psoriasis is AP1S3 (Adaptor Related Protein Complex 1 Sigma 3 Subunit), and among its related pathways/superpathways are Matrix Metalloproteinases and NF-kappa B signaling pathway. The drugs Antibodies, Monoclonal and Immunoglobulins have been mentioned in the context of this disorder. Affiliated tissues include skin, monocytes and neutrophil.

Wikipedia : 72 The term pustular psoriasis is used for a heterogeneous group of diseases that share pustular skin... more...

Related Diseases for Pustular Psoriasis

Diseases in the Pustular Psoriasis family:

Psoriasis 14, Pustular Psoriasis 15, Pustular

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

(show top 50) (show all 119)
# Related Disease Score Top Affiliating Genes
1 psoriasis 14, pustular 34.2 AP1S3 IL36RN
2 pustulosis of palm and sole 32.9 IL36RN TNF
3 pustulosis palmaris et plantaris 32.2 AP1S3 ELANE IL36RN TNF
4 impetigo herpetiformis 31.4 CARD14 ELANE IL36RN PI3
5 acrodermatitis 30.6 AP1S3 IL36RN
6 vasculitis 29.5 ELANE TNF
7 skin disease 29.5 CARD14 IL36RN TNF
8 netherton syndrome 29.5 ELANE PI3
9 impetigo 29.4 ELANE IL36RN PI3
10 acute respiratory distress syndrome 29.2 ELANE PI3 TNF
11 psoriasis 29.2 AP1S3 CARD14 ELANE IL36RN PI3 TNF
12 pneumonia 28.9 ELANE TNF
13 psoriasis 15, pustular 11.8
14 psoriasis 1 11.3
15 psoriasis 7 10.3
16 psoriasis 11 10.3
17 psoriasis 13 10.3
18 psoriasis 2 10.3
19 arthritis 10.3
20 acute generalized exanthematous pustulosis 10.3
21 psoriatic arthritis 10.2
22 subcorneal pustular dermatosis 10.1
23 pityriasis rubra pilaris 10.1 CARD14 IL36RN
24 geographic tongue 10.1 IL36RN TNF
25 pemphigus 10.1
26 irritant dermatitis 10.0 PI3 TNF
27 cholangitis 10.0
28 turner syndrome 10.0
29 capillary leak syndrome 10.0
30 hypoparathyroidism 10.0
31 hepatitis 9.9
32 pemphigus foliaceus 9.9
33 thyroiditis 9.9
34 rheumatoid arthritis 9.9
35 bullous pemphigoid 9.9
36 crohn's disease 9.9
37 arthus reaction 9.8 ELANE TNF
38 pulmonary disease, chronic obstructive 9.8 ELANE TNF
39 leukocyte disease 9.8 ELANE TNF
40 adult respiratory distress syndrome 9.8 ELANE TNF
41 chorioamnionitis 9.8 ELANE TNF
42 gingivitis 9.8 ELANE TNF
43 pasteurellosis 9.8 ELANE PI3
44 bronchial disease 9.8 ELANE TNF
45 bronchopneumonia 9.8 ELANE PI3
46 autoinflammation, lipodystrophy, and dermatosis syndrome 9.8 ELANE TNF
47 dilated cardiomyopathy 9.8
48 inflammatory spondylopathy 9.8
49 dermatitis 9.8
50 iridocyclitis 9.8

Graphical network of the top 20 diseases related to Pustular Psoriasis:



Diseases related to Pustular Psoriasis

Symptoms & Phenotypes for Pustular Psoriasis

Drugs & Therapeutics for Pustular Psoriasis

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

(show all 17)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Antibodies, Monoclonal Phase 4,Phase 3
2 Immunoglobulins Phase 4,Phase 3
3 Antibodies Phase 4,Phase 3
4
Adalimumab Approved Phase 3 331731-18-1 16219006
5
Infliximab Approved Phase 3 170277-31-3
6
Menthol Approved Phase 3 2216-51-5 16666
7
Benzocaine Approved, Investigational Phase 2, Phase 3 1994-09-7, 94-09-7 2337
8
Certolizumab pegol Approved Phase 2, Phase 3 428863-50-7
9
Ustekinumab Approved, Investigational Phase 3 815610-63-0
10 tannic acid Approved, Nutraceutical Phase 2, Phase 3
11 Dermatologic Agents Phase 3,Phase 2
12 Anti-Inflammatory Agents Phase 3
13 Antirheumatic Agents Phase 3,Phase 2
14 Pharmaceutical Solutions Phase 2, Phase 3
15 Immunosuppressive Agents Phase 2, Phase 3
16 Interleukin 1 Receptor Antagonist Protein Phase 2
17 tyrosine Nutraceutical

Interventional clinical trials:

(show all 18)

# Name Status NCT ID Phase Drugs
1 Raptiva in Palm and Sole Psoriasis Terminated NCT00972543 Phase 4
2 Palmoplantar Pustular Psoriasis Efficacy and Safety wIth Secukinumab Completed NCT02008890 Phase 3 Placebo
3 Study to Assess the Efficacy, Safety and Tolerability of Secukinumab in Japanese Subjects With Generalized Pustular Psoriasis (GPP) Completed NCT01952015 Phase 3
4 Study to Investigate Efficacy and Safety of Adalimumab in Japanese Subjects With Generalized Pustular Psoriasis (GPP) Completed NCT02533375 Phase 3 Adalimumab
5 An Open-label, Non-controlled Study of KHK4827 in Subjects With Psoriasis Completed NCT01782937 Phase 3 KHK4827
6 A Phase 3 Clinical Study of KHK 4827 Completed NCT02052609 Phase 3 KHK4827 140mg SC;KHK4827 210mg SC
7 A Clinical Study of Increased Dose of TA-650 in Patients With Psoriasis Completed NCT01680159 Phase 3 TA-650
8 A Study to Assess Efficacy and Safety of Two Different Dose Regimens of Risankizumab Administered Subcutaneously in Japanese Subjects With Generalized Pustular Psoriasis or Erythrodermic Psoriasis Active, not recruiting NCT03022045 Phase 3 risankizumab
9 An Efficacy and Safety Study of CNTO1959 (Guselkumab) in the Treatment of Participants With Generalized Pustular Psoriasis or Erythrodermic Psoriasis Active, not recruiting NCT02343744 Phase 3 Guselkumab
10 A Study to Test the Efficacy and Safety of Certolizumab Pegol in Japanese Subjects With Moderate to Severe Chronic Psoriasis Active, not recruiting NCT03051217 Phase 2, Phase 3 Certolizumab Pegol
11 Safety and Efficacy of Ustekinumab in Patients With Palmo-Plantar Pustular Psoriasis or With Palmo-Plantar Pustulosis Terminated NCT01091051 Phase 3 Ustekinumab;Placebo (Soduim Chloride)
12 Determination of the Efficacy and Safety of Psirelax in the Relief of the Disease in Psoriasis Completed NCT01000714 Phase 2 Psirelax
13 Study to Evaluate the Efficacy of Alefacept to Treat Palmar Plantar Pustulosis Completed NCT00301002 Phase 2 Alefacept
14 Initial Dosing of BI 655130 in Palmoplantar Pustulosis Patients Recruiting NCT03135548 Phase 2 BI 655130 (low dose);Placebo;BI 655130 (high dose)
15 Anakinra for Inflammatory Pustular Skin Diseases Recruiting NCT01794117 Phase 2 Anakinra
16 BI655130 Single Dose in Generalized Pustular Psoriasis Completed NCT02978690 Phase 1 BI655130
17 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275
18 Role of Tyrosine Kinase Lyn and Cleaved Form by Caspases in Psoriasis Recruiting NCT01538342

Search NIH Clinical Center for Pustular Psoriasis

Genetic Tests for Pustular Psoriasis

Anatomical Context for Pustular Psoriasis

MalaCards organs/tissues related to Pustular Psoriasis:

38
Skin, Monocytes, Neutrophil, Testes, Thyroid, Bone, Tongue

Publications for Pustular Psoriasis

Articles related to Pustular Psoriasis:

(show top 50) (show all 382)
# Title Authors Year
1
ARDS complicating pustular psoriasis: treatment with low-dose corticosteroids, vitamin C and thiamine. ( 29420246 )
2018
2
Increased levels of lipocalin 2 in palmoplantar pustular psoriasis. ( 29395573 )
2018
3
Pustular psoriasis and related pustular skin diseases. ( 29333670 )
2018
4
Genetic background and therapeutic response in generalized pustular psoriasis patients treated with granulocyte and monocyte adsorption apheresis. ( 29380726 )
2018
5
Generalized pustular psoriasis triggered by Zika virus infection. ( 29027743 )
2018
6
Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis. ( 28967976 )
2018
7
A dramatic response to a single dose of infliximab in a patient with prolonged pustular psoriasis derived from inverse psoriasis. ( 28419647 )
2017
8
Pediatric pustular psoriasis responsive to cyclosporine bridged to etanercept: A treatment approach. ( 29447638 )
2017
9
Generalised pustular psoriasis and neutrophilic cholangitis: An infrequently reported association with excellent response to tumour necrosis factor inhibitors. ( 28195322 )
2017
10
Highly Resistant Acrodermatitis Continua of Hallopeau and Pustular Psoriasis. ( 29177142 )
2017
11
Generalized pustular psoriasis: A possible association with severe hypocalcaemia due to primary hypoparathyroidism. ( 28106254 )
2017
12
Granulocyte and monocyte apheresis can control juvenile generalised pustular psoriasis with mutation of the IL36RN gene. ( 28369922 )
2017
13
Clinical features of von Zumbusch type of generalized pustular psoriasis in children: a retrospective study of 26 patients in southwestern China. ( 29186241 )
2017
14
Acute Generalized Erythrodermic Pustular Psoriasis Associated with Bupropion/Naltrexone (Contrave(Ar)). ( 28130024 )
2017
15
Recalcitrant infantile generalized pustular psoriasis successfully treated with acitretin and narrowband ultraviolet B. ( 28106270 )
2017
16
Generalized Pustular Psoriasis. ( 28629688 )
2017
17
Palmoplantar pustular psoriasis (PPPP) is characterized by activation of the IL-17A pathway. ( 27743912 )
2017
18
Annular pustular psoriasis in a 14-month-old girl: a therapeutic challenge. ( 27997815 )
2017
19
Psoriasis verrucosa complicated by generalized pustular psoriasis. ( 29235672 )
2017
20
European consensus statement on phenotypes of pustular psoriasis. ( 28585342 )
2017
21
Interleukin-17 blockade in generalized pustular psoriasis - new hope for severely ill patients. ( 28300318 )
2017
22
Generalized pustular psoriasis treated with apremilast in a patient with multiple medical comorbidities. ( 29034298 )
2017
23
Autosomal dominant familial generalized pustular psoriasis caused by a CARD14 mutation. ( 28295164 )
2017
24
Clearance of annular pustular psoriasis with ustekinumab. ( 28202422 )
2017
25
Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia. ( 28194751 )
2017
26
A newly revealed IL36RN mutation in sibling cases complements our IL36RN mutation statistics for generalized pustular psoriasis. ( 27802907 )
2017
27
Acquired hemophilia A following generalized pustular psoriasis of pregnancy. ( 28771773 )
2017
28
Generalized pustular psoriasis in a 92-year-old man with a homozygous nonsense mutation in IL36RN. ( 29215143 )
2017
29
Unopposed IL-36 activity promotes clonal CD4<sup>+</sup>T-cell responses with IL-17A production in generalized pustular psoriasis. ( 29288651 )
2017
30
Rare diseases and costly treatments: the role of IL-1I^ in pustular psoriasis. ( 28098365 )
2017
31
Short- and Long-Term Management of an Acute Pustular Psoriasis Flare: A Case Report. ( 28535721 )
2017
32
Generalized Pustular Psoriasis With IL-36 Receptor Antagonist Mutation Successfully Treated With Granulocyte and Monocyte Adsorption Apheresis Accompanied by Reduced Serum IL-6 Level. ( 28913961 )
2017
33
Successful Secukinumab treatment of generalized pustular psoriasis and erythrodermic psoriasis. ( 28319281 )
2017
34
Efficacy and safety of acitretin monotherapy in children with pustular psoriasis: results from 15 cases and a literature review. ( 29098909 )
2017
35
Case of generalized pustular psoriasis presenting with both pleural effusion and pericardial effusion collection. ( 28636215 )
2017
36
Secukinumab in the Treatment of Palmoplantar, Nail, Scalp, and Pustular Psoriasis. ( 28439342 )
2016
37
Generalized pustular psoriasis associated with Turner syndrome and dilated cardiomyopathy. ( 26870904 )
2016
38
Efficacy and safety of brodalumab in patients with generalized pustular psoriasis and psoriatic erythroderma: results from a 52-week, open-label study. ( 27106510 )
2016
39
Efficacy and safety of ixekizumab treatment for Japanese patients with moderate to severe plaque psoriasis, erythrodermic psoriasis and generalized pustular psoriasis: Results from a 52-week, open-label, phase 3 study (UNCOVER-J). ( 27726163 )
2016
40
Perianal streptococcal infection precipitating pustular psoriasis in an adult. ( 27504479 )
2016
41
Imiquimod Treatment Causes Systemic Disease in Mice Resembling Generalized Pustular Psoriasis in an IL-1 and IL-36 Dependent Manner. ( 28057979 )
2016
42
Pustular Psoriasis Occurring on the Striae Distensae: An Umpteenth Example of Immunocompromised Cutaneous District. ( 27688451 )
2016
43
IL-1 and IL-36 are dominant cytokines in generalized pustular psoriasis. ( 28043870 )
2016
44
Correction: Based on Molecular Profiling of Gene Expression, Palmoplantar Pustulosis and Palmoplantar Pustular Psoriasis Are Highly Related Diseases that Appear to Be Distinct from Psoriasis Vulgaris. ( 27326461 )
2016
45
Ustekinumab in the treatment of palmoplantar pustular psoriasis - a case series of nine patients. ( 27879067 )
2016
46
Coincidence of annular pustular psoriasis, pemphigus foliaceus, and leukocytoclastic vasculitis associated with chronic cholecystitis. ( 27509423 )
2016
47
Therapeutic Efficacy of Interleukin 12/Interleukin 23 Blockade in Generalized Pustular Psoriasis Regardless of IL36RN Mutation Status. ( 27096382 )
2016
48
Immunohistochemical Comparison of IL-36 and the IL-23/Th17 Axis of Generalized Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis. ( 27489427 )
2016
49
Pustular Psoriasis of Pregnancy with Acrodermatitis Continua of Hallopeau. ( 26955145 )
2016
50
Generalized pustular psoriasis induced by systemic steroid dose reduction. ( 27828647 )
2016

Variations for Pustular Psoriasis

Expression for Pustular Psoriasis

Search GEO for disease gene expression data for Pustular Psoriasis.

Pathways for Pustular Psoriasis

Pathways related to Pustular Psoriasis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.25 ELANE TNF
2 11.13 CARD14 TNF
4 10.3 ELANE TNF

GO Terms for Pustular Psoriasis

Biological processes related to Pustular Psoriasis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of NF-kappaB transcription factor activity GO:0051092 9.43 CARD14 TNF
2 tumor necrosis factor-mediated signaling pathway GO:0033209 9.4 CARD14 TNF
3 positive regulation of MAP kinase activity GO:0043406 9.37 ELANE TNF
4 positive regulation of smooth muscle cell proliferation GO:0048661 9.32 ELANE TNF
5 antimicrobial humoral response GO:0019730 9.26 ELANE PI3
6 negative regulation of interleukin-6 production GO:0032715 9.16 IL36RN TNF
7 negative regulation of growth of symbiont in host GO:0044130 8.96 ELANE TNF
8 positive regulation of interleukin-8 biosynthetic process GO:0045416 8.62 ELANE TNF

Molecular functions related to Pustular Psoriasis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 8.62 ELANE TNF

Sources for Pustular Psoriasis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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