MCID: PST059
MIFTS: 40

Pustular Psoriasis

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

Aliases & Classifications for Pustular Psoriasis

MalaCards integrated aliases for Pustular Psoriasis:

Name: Pustular Psoriasis 53 37 55 73

Classifications:



External Ids:

KEGG 37 H01663
UMLS 73 C0152081

Summaries for Pustular Psoriasis

NIH Rare Diseases : 53 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.

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 IL36RN (Interleukin 36 Receptor Antagonist), and among its related pathways/superpathways are Matrix Metalloproteinases and NF-kappa B signaling pathway. The drugs Miconazole and Etanercept have been mentioned in the context of this disorder. Affiliated tissues include skin, monocytes and neutrophil.

Wikipedia : 76 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 123)
# Related Disease Score Top Affiliating Genes
1 psoriasis 14, pustular 34.4 AP1S3 IL36RN
2 pustulosis of palm and sole 32.8 IL36RN TNF
3 pustulosis palmaris et plantaris 31.5 AP1S3 ELANE IL36RN TNF
4 acrodermatitis 30.9 AP1S3 IL36RN
5 impetigo herpetiformis 30.7 CARD14 ELANE IL36RN PI3
6 geographic tongue 30.2 IL36RN TNF
7 psoriatic arthritis 29.9 IL36RN TNF
8 vasculitis 29.3 ELANE TNF
9 netherton syndrome 29.2 ELANE PI3
10 psoriasis 29.2 AP1S3 CARD14 IL36RN PI3 TNF
11 impetigo 29.0 ELANE IL36RN PI3
12 pneumonia 28.9 ELANE TNF
13 acute respiratory distress syndrome 28.7 ELANE PI3 TNF
14 skin disease 28.3 CARD14 IL36RN PI3 TNF
15 psoriasis 15, pustular 11.8
16 psoriasis 1 11.3
17 psoriasis 2 10.3
18 psoriasis 7 10.3
19 psoriasis 11 10.3
20 psoriasis 13 10.3
21 acute generalized exanthematous pustulosis 10.3
22 arthritis 10.3
23 pityriasis rubra pilaris 10.3 CARD14 IL36RN
24 pyoderma 10.2 IL36RN TNF
25 subcorneal pustular dermatosis 10.1
26 pemphigus 10.0
27 irritant dermatitis 10.0 PI3 TNF
28 systemic capillary leak syndrome 10.0
29 turner syndrome 10.0
30 cholangitis 10.0
31 capillary leak syndrome 10.0
32 hypoparathyroidism 10.0
33 crohn's disease 10.0
34 hepatitis 9.9
35 pemphigus foliaceus 9.9
36 rheumatoid arthritis 9.9
37 thyroiditis 9.9
38 bullous pemphigoid 9.9
39 arthus reaction 9.8 ELANE TNF
40 chronic recurrent multifocal osteomyelitis 9.7
41 diabetes mellitus 9.7
42 hemopericardium 9.7
43 hyperostosis 9.7
44 multiple carboxylase deficiency 9.7
45 igg4-related disease 9.7
46 hemophilia a 9.7
47 dilated cardiomyopathy 9.7
48 fissured tongue 9.7
49 relapsing polychondritis 9.7
50 maculopapular cutaneous mastocytosis 9.7

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 33)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
2
Etanercept Approved, Investigational Phase 4,Phase 3 185243-69-0
3 Analgesics Phase 4,Phase 3
4 Gastrointestinal Agents Phase 4,Phase 3
5 Analgesics, Non-Narcotic Phase 4,Phase 3
6 Cyclosporins Phase 4
7 Tin Fluorides Phase 4
8 Dermatologic Agents Phase 4,Phase 3,Phase 2
9 Peripheral Nervous System Agents Phase 4,Phase 3
10 Antibodies Phase 4,Phase 3
11 Immunoglobulins Phase 4,Phase 3
12 Antibodies, Monoclonal Phase 4,Phase 3
13 Immunosuppressive Agents Phase 4,Phase 3,Phase 2
14 Anti-Infective Agents Phase 4,Phase 3
15 Antifungal Agents Phase 4
16 Anti-Inflammatory Agents Phase 4,Phase 3
17 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 3
18 Antirheumatic Agents Phase 4,Phase 3,Phase 2
19 Calcineurin Inhibitors Phase 4
20
Menthol Approved Phase 3 2216-51-5 16666
21
Infliximab Approved Phase 3 170277-31-3
22
Adalimumab Approved Phase 3 331731-18-1 16219006
23
Benzocaine Approved, Investigational Phase 3,Phase 2 1994-09-7, 94-09-7 2337
24
Apremilast Approved, Investigational Phase 3 608141-41-9 11561674
25
Certolizumab pegol Approved Phase 2, Phase 3 428863-50-7
26
Ustekinumab Approved, Investigational Phase 3 815610-63-0
27 tannic acid Approved, Nutraceutical Phase 3,Phase 2
28 Angiogenesis Inhibitors Phase 3
29 Angiogenesis Modulating Agents Phase 3
30 Anti-Bacterial Agents Phase 3
31 Pharmaceutical Solutions Phase 2, Phase 3
32 Interleukin 1 Receptor Antagonist Protein Phase 2
33 tyrosine Nutraceutical Not Applicable

Interventional clinical trials:

(show all 22)
# Name Status NCT ID Phase Drugs
1 Evaluation of Etanercept in Patients With Plaque Psoriasis After Stopping Ciclosporin Therapy Completed NCT00581555 Phase 4 Etanercept
2 Raptiva in Palm and Sole Psoriasis Terminated NCT00972543 Phase 4
3 Palmoplantar Pustular Psoriasis Efficacy and Safety wIth Secukinumab Completed NCT02008890 Phase 3 Placebo
4 Study to Investigate Efficacy and Safety of Adalimumab in Japanese Subjects With Generalized Pustular Psoriasis (GPP) Completed NCT02533375 Phase 3 Adalimumab
5 Study to Assess the Efficacy, Safety and Tolerability of Secukinumab in Japanese Subjects With Generalized Pustular Psoriasis (GPP) Completed NCT01952015 Phase 3
6 An Open-label, Non-controlled Study of KHK4827 in Subjects With Psoriasis Completed NCT01782937 Phase 3 KHK4827
7 A Phase 3 Clinical Study of KHK 4827 Completed NCT02052609 Phase 3 KHK4827 140mg SC;KHK4827 210mg SC
8 A Clinical Study of Increased Dose of TA-650 in Patients With Psoriasis Completed NCT01680159 Phase 3 TA-650
9 Study to Evaluate Safety and Effectiveness of Oral Apremilast (CC-10004) in Patients With Moderate to Severe Plaque Psoriasis. Completed NCT01232283 Phase 3 Apremilast;Placebo
10 Study to Evaluate Safety and Effectiveness of Oral Apremilast (CC-10004) in Patients With Moderate to Severe Plaque Psoriasis Completed NCT01194219 Phase 3 Apremilast;Placebo;Topical treatments or phototherapy
11 Phase 3b Safety and Efficacy Study of Apremilast to Treat Moderate to Severe Plaque-plaque Psoriasis Completed NCT01690299 Phase 3 Apremilast;Etanercept;Placebo tablet;Placebo injection
12 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
13 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
14 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
15 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)
16 Determination of the Efficacy and Safety of Psirelax in the Relief of the Disease in Psoriasis Completed NCT01000714 Phase 2 Psirelax
17 Study to Evaluate the Efficacy of Alefacept to Treat Palmar Plantar Pustulosis Completed NCT00301002 Phase 2 Alefacept
18 Anakinra for Inflammatory Pustular Skin Diseases Recruiting NCT01794117 Phase 2 Anakinra
19 Initial Dosing of BI 655130 in Palmoplantar Pustulosis Patients Active, not recruiting NCT03135548 Phase 2 BI 655130 (low dose);Placebo;BI 655130 (high dose)
20 BI655130 Single Dose in Generalized Pustular Psoriasis Completed NCT02978690 Phase 1 BI655130
21 Role of Tyrosine Kinase Lyn and Cleaved Form by Caspases in Psoriasis Unknown status NCT01538342 Not Applicable
22 Observational Study of the Genetic Architecture of Neutrophil-Mediated Inflammatory Skin Diseases Recruiting NCT01952275

Search NIH Clinical Center for Pustular Psoriasis

Genetic Tests for Pustular Psoriasis

Anatomical Context for Pustular Psoriasis

MalaCards organs/tissues related to Pustular Psoriasis:

41
Skin, Monocytes, Neutrophil, Testes, Tongue, Thyroid, Bone

Publications for Pustular Psoriasis

Articles related to Pustular Psoriasis:

(show top 50) (show all 408)
# Title Authors Year
1
Paradoxical pustular psoriasis induced by ustekinumab in a patient with Crohn's disease-associated spondyloarthropathy. ( 29705819 )
2018
2
The heterogeneous mutational landscape of pustular psoriasis. ( 29595236 )
2018
3
RNA sequencing-based longitudinal transcriptomic profiling gives novel insights into the disease mechanism of generalized pustular psoriasis. ( 29871627 )
2018
4
Pustular psoriasis and related pustular skin diseases. ( 29333670 )
2018
5
Increased levels of lipocalin 2 in palmoplantar pustular psoriasis. ( 29395573 )
2018
6
Pustular psoriasis of pregnancy: current perspectives. ( 29520163 )
2018
7
An infant with generalized pustular psoriasis and geographic tongue had a heterozygous IL36RN mutation and IgG2 deficiency. ( 29454537 )
2018
8
A case of pustular psoriasis of pregnancy with positive maternal-fetal outcomes. ( 29763478 )
2018
9
Pediatric case of generalized pustular psoriasis developing acute pancreatitis. ( 29655246 )
2018
10
Acrodermatitis continua of Hallopeau and geographic tongue are variants of pustular psoriasis. ( 29687071 )
2018
11
ARDS complicating pustular psoriasis: treatment with low-dose corticosteroids, vitamin C and thiamine. ( 29420246 )
2018
12
Genetic background and therapeutic response in generalized pustular psoriasis patients treated with granulocyte and monocyte adsorption apheresis. ( 29380726 )
2018
13
Systemic Monotherapy Treatments for Generalized Pustular Psoriasis: A Systematic Review. ( 29707979 )
2018
14
Generalized pustular psoriasis: a review and update on treatment. ( 29573491 )
2018
15
Generalized Pustular Psoriasis ( 29630241 )
2018
16
Guselkumab, a human interleukin-23 monoclonal antibody in Japanese patients with generalized pustular psoriasis and erythrodermic psoriasis: Efficacy and safety analyses of a 52-week, phase 3, multicenter, open-label study. ( 29569397 )
2018
17
Case of generalized pustular psoriasis that might have progressed from terbinafine-induced acute generalized exanthematous pustulosis. ( 29766566 )
2018
18
Two cases of generalized pustular psoriasis complicated by IgG4-related disease. ( 29709076 )
2018
19
Generalized Pustular Psoriasis Induced by Infliximab in a Patient With Inflammatory Bowel Disease. ( 29484893 )
2018
20
Generalized pustular psoriasis - A model disease for specific targeted immunotherapy, systematic review. ( 29852521 )
2018
21
Generalized pustular psoriasis triggered by Zika virus infection. ( 29027743 )
2018
22
Association of IL36RN mutations with clinical features, therapeutic response to acitretin, and frequency of recurrence in patients with generalized pustular psoriasis. ( 29619998 )
2018
23
Successful treatment with interleukin-17A antagonists of generalized pustular psoriasis in patients without IL36RN mutations. ( 29655177 )
2018
24
Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis. ( 28967976 )
2018
25
Severe hypocalcemia-related pustular psoriasis in an 83-year-old woman with Fahr's syndrome: a case report. ( 29953675 )
2018
26
A Case of Ceftriaxone-Induced Acute Generalized Exanthematous Pustulosis/Generalized Pustular Psoriasis Overlap. ( 29681811 )
2018
27
A newly revealed IL36RN mutation in sibling cases complements our IL36RN mutation statistics for generalized pustular psoriasis. ( 27802907 )
2017
28
Generalized pustular psoriasis in a 92-year-old man with a homozygous nonsense mutation in IL36RN. ( 29215143 )
2017
29
A dramatic response to a single dose of infliximab in a patient with prolonged pustular psoriasis derived from inverse psoriasis. ( 28419647 )
2017
30
Case of generalized pustular psoriasis presenting with both pleural effusion and pericardial effusion collection. ( 28636215 )
2017
31
Psoriasis verrucosa complicated by generalized pustular psoriasis. ( 29235672 )
2017
32
Autosomal dominant familial generalized pustular psoriasis caused by a CARD14 mutation. ( 28295164 )
2017
33
Generalized Pustular Psoriasis. ( 28629688 )
2017
34
Juvenile generalized pustular psoriasis is a chronic recalcitrant disease: an analysis of 27 patients seen in a tertiary hospital in Johor, Malaysia. ( 28194751 )
2017
35
Generalized pustular psoriasis treated with apremilast in a patient with multiple medical comorbidities. ( 29034298 )
2017
36
Granulocyte and monocyte apheresis can control juvenile generalised pustular psoriasis with mutation of the IL36RN gene. ( 28369922 )
2017
37
Successful Secukinumab treatment of generalized pustular psoriasis and erythrodermic psoriasis. ( 28319281 )
2017
38
Annular pustular psoriasis in a 14-month-old girl: a therapeutic challenge. ( 27997815 )
2017
39
Acute Generalized Erythrodermic Pustular Psoriasis Associated with Bupropion/Naltrexone (Contrave(Ar)). ( 28130024 )
2017
40
Unopposed IL-36 activity promotes clonal CD4<sup>+</sup>T-cell responses with IL-17A production in generalized pustular psoriasis. ( 29288651 )
2017
41
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
42
Short- and Long-Term Management of an Acute Pustular Psoriasis Flare: A Case Report. ( 28535721 )
2017
43
Pediatric pustular psoriasis responsive to cyclosporine bridged to etanercept: A treatment approach. ( 29447638 )
2017
44
Acquired hemophilia A following generalized pustular psoriasis of pregnancy. ( 28771773 )
2017
45
European consensus statement on phenotypes of pustular psoriasis. ( 28585342 )
2017
46
Palmoplantar pustular psoriasis (PPPP) is characterized by activation of the IL-17A pathway. ( 27743912 )
2017
47
Generalized pustular psoriasis: A possible association with severe hypocalcaemia due to primary hypoparathyroidism. ( 28106254 )
2017
48
Generalised pustular psoriasis and neutrophilic cholangitis: An infrequently reported association with excellent response to tumour necrosis factor inhibitors. ( 28195322 )
2017
49
Rare diseases and costly treatments: the role of IL-1I^ in pustular psoriasis. ( 28098365 )
2017
50
Efficacy and safety of acitretin monotherapy in children with pustular psoriasis: results from 15 cases and a literature review. ( 29098909 )
2017

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
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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