MCID: PST059
MIFTS: 37

Pustular Psoriasis

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

Aliases & Classifications for Pustular Psoriasis

MalaCards integrated aliases for Pustular Psoriasis:

Name: Pustular Psoriasis 20 36 54 70

Classifications:



External Ids:

KEGG 36 H01663
UMLS 70 C0152081

Summaries for Pustular Psoriasis

KEGG : 36 Pustular psoriasis is a form of psoriasis [DS:H01656] characterized by multiple tender sterile pustules with an underlying, blotchy, erythematous base. It has been classified into localized and generalized forms. Generalized pustular psoriasis (GPP) is the development of extensive sterile pustules with widespread erythema. The von Zumbusch type often starts abruptly and can be associated with painful skin, fever, and chills. The disease course varies from a benign, chronic process to an acute life-threatening episode, and as such optimal treatment depends on severity. Diseases considered within the spectrum of GPP include: impetigo herpetiformis and childhood GPP. Acrodermatitis continua of Hallopeau and palmoplantar pustulosis are two distinct forms of localized pustular psoriasis. Treatment should be governed by the extent of involvement and severity of disease. Acitretin, cyclosporine, methotrexate, and infliximab are considered to be first-line therapies for those with GPP. Adalimumab, etanercept, and psoralen plus ultraviolet A are second-line modalities in this setting. Pustular psoriasis in children, in pregnant women, and in localized forms alter which agents are first-line modalities as concerns such as teratogenicity need to be factored into the decisionmaking for the individual patient.

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 amb2 Integrin signaling. The drugs Ixekizumab and Brodalumab have been mentioned in the context of this disorder. Affiliated tissues include neutrophil, tongue and skin.

GARD : 20 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.

Wikipedia : 73 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 264)
# Related Disease Score Top Affiliating Genes
1 psoriasis 14, pustular 32.9 TNF IL36RN AP1S3
2 pustulosis of palm and sole 32.3 TNF IL36RN AP1S3
3 pustulosis palmaris et plantaris 31.6 TNF IL36RN ELANE AP1S3
4 psoriasis 15 31.6 ELANE AP1S3
5 impetigo herpetiformis 31.5 PI3 IL36RN ELANE
6 acrodermatitis 31.2 IL36RN AP1S3
7 psoriasis 30.3 TNF SLPI PI3 IL36RN ELANE AP1S3
8 skin disease 30.3 TNF PI3 IL36RN AP1S3
9 impetigo 30.2 PI3 IL36RN ELANE
10 pityriasis rubra pilaris 30.2 TNF IL36RN
11 pyoderma 29.9 TNF IL36RN
12 allergic contact dermatitis 29.9 TNF PI3
13 adult respiratory distress syndrome 29.7 TNF PI3 ELANE
14 spondyloarthropathy 29.7 TNF SLPI
15 netherton syndrome 29.7 PI3 ELANE
16 periodontitis 29.5 TNF ELANE
17 pneumonia 29.5 TNF SLPI ELANE
18 alpha-1-antitrypsin deficiency 29.2 TNF SLPI ELANE
19 acute pancreatitis 29.2 TNF ELANE
20 lung disease 28.7 TNF SLPI PI3 ELANE
21 psoriasis 1 11.3
22 psoriasis 15, pustular 11.2
23 psoriatic arthritis 10.6
24 acute generalized exanthematous pustulosis 10.6
25 exanthem 10.5
26 subcorneal pustular dermatosis 10.4
27 vasculitis 10.4
28 arthritis 10.3
29 autoimmune disease 10.3
30 palmoplantar keratoderma, punctate type ii 10.3
31 pemphigoid 10.3
32 punctate porokeratosis 10.3
33 hypoparathyroidism 10.3
34 gonadal dysgenesis 10.3
35 turner syndrome 10.3
36 crohn's disease 10.3
37 pemphigus 10.3
38 nasopharyngitis 10.2
39 inflammatory spondylopathy 10.2
40 geographic tongue 10.2
41 dermatitis 10.2
42 spondylitis 10.2
43 cholangitis 10.2
44 spondyloarthropathy 1 10.2
45 systemic lupus erythematosus 10.2
46 inflammatory bowel disease 10.2
47 capillary leak syndrome 10.2
48 urticaria 10.2
49 hyperostosis 10.2
50 arthropathy 10.2

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
Ixekizumab Approved, Investigational Phase 4 1143503-69-8
2
Brodalumab Approved, Investigational Phase 4 1174395-19-7
3 Dermatologic Agents Phase 4
4
Adalimumab Approved, Experimental Phase 3 331731-18-1 16219006
5
tannic acid Approved Phase 3 1401-55-4
6
Benzocaine Approved, Investigational Phase 3 1994-09-7, 94-09-7 2337
7
Ustekinumab Approved, Investigational Phase 3 815610-63-0
8 Anti-Inflammatory Agents Phase 3
9 Antibodies Phase 3
10 Immunoglobulins Phase 3
11 Antibodies, Monoclonal Phase 3
12 Immunologic Factors Phase 3
13 Vaccines Phase 2
14 Pharmaceutical Solutions Phase 2
15
Infliximab Approved 170277-31-3
16 Antirheumatic Agents
17 Gastrointestinal Agents

Interventional clinical trials:

(show all 19)
# Name Status NCT ID Phase Drugs
1 A Multicenter, Open-Label, Post Marketing Clinical Trial to Evaluate the Efficacy And Safety Of Ixekizumab in Patients With Generalized Pustular Psoriasis and Erythrodermic Psoriasis Completed NCT03942042 Phase 4 Ixekizumab
2 An Extension Study of Brodalumab in Subjects With Plaque Psoriasis (Psoriasis Vulgaris, Psoriatic Arthritis), Pustular Psoriasis (Generalized) and Psoriatic Erythroderma Completed NCT04183881 Phase 4 Brodalumab 210mg SC
3 A Multicenter, Open-Label Study of Adalimumab in Japanese Subjects With Generalized Pustular Psoriasis Completed NCT02533375 Phase 3 Adalimumab
4 A Phase 3, Multicenter, Open-Label Study Evaluating the Efficacy and Safety of CNTO1959 (Guselkumab) in the Treatment of Subjects With Generalized Pustular Psoriasis or Erythrodermic Psoriasis Completed NCT02343744 Phase 3 Guselkumab
5 A 52-week, Multicenter, Randomized, Double-blind, Placebo-controlled Study of Subcutaneous Secukinumab to Demonstrate Efficacy as Assessed by Palmoplantar Pustulosis Psoriasis Area and Severity Index (ppPASI) at 16 Weeks of Treatment, Compared to Placebo, and to Assess Long-term Safety, Tolerability, and Efficacy in Subjects With Moderate to Severe Chronic Palmoplantar Pustular Psoriasis - Amended With an Optional Extension Treatment Period of up to a Total of 148 Weeks Completed NCT02008890 Phase 3
6 A Phase 3, Randomized, Open-Label 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 Completed NCT03022045 Phase 3 risankizumab
7 A Long-Term Study of KHK4827 in Subjects With Pustular Psoriasis (Generalized) and Psoriatic Erythroderma Completed NCT01782937 Phase 3 KHK4827
8 An Extension Study of KHK4827 in Subjects With Plaque Psoriasis (Psoriasis Vulgaris, Psoriatic Arthritis), Pustular Psoriasis (Generalized) and Psoriatic Erythroderma Completed NCT02052609 Phase 3 KHK4827 140mg SC;KHK4827 210mg SC
9 A Multi-center, Open Label Study of Subcutaneous Secukinumab in Prefilled Syringes as Mono- or Co-therapy to Assess the Efficacy, Safety and Tolerability in Japanese Subjects With Generalized Pustular Psoriasis Completed NCT01952015 Phase 3
10 A Phase 3b, Multicenter, Interventional, Randomized, Placebo-controlled Study Investigating the Efficacy and Safety of Guselkumab for the Treatment of Palmoplantar-non-Pustular Psoriasis Active, not recruiting NCT03998683 Phase 3 Guselkumab 100 mg;Placebo
11 Safety and Efficacy of Ustekinumab in the Treatment of Patients With Palmo-Plantar Pustular Psoriasis or With Palmo-Plantar Pustulosis Terminated NCT01091051 Phase 3 Ustekinumab;Placebo (Soduim Chloride)
12 A Single Arm Multiple Dose Study to Assess the Efficacy and Safety of ANB019 in Subjects With Generalized Pustular Psoriasis Completed NCT03619902 Phase 2 ANB019
13 Effisayil™ 1:Multi-center, Double-blind, Randomised, Placebo-controlled, Phase II Study to Evaluate Efficacy, Safety and Tolerability of a Single Intravenous Dose of BI 655130 in Patients With Generalized Pustular Psoriasis (GPP) Presenting With an Acute Flare of Moderate to Severe Intensity. Completed NCT03782792 Phase 2 Spesolimab;Placebo
14 Effisayil™ 2: Multi-center, Randomized, Parallel Group, Double Blind, Placebo Controlled, Phase IIb Dose-finding Study to Evaluate Efficacy and Safety of BI 655130 (Spesolimab) Compared to Placebo in Preventing Generalized Pustular Psoriasis (GPP) Flares in Patients With History of GPP Recruiting NCT04399837 Phase 2 Spesolimab;Placebo
15 An Open-label, Long Term Extension Study to Assess the Safety and Efficacy of BI 655130 Treatment in Patients With Generalized Pustular Psoriasis (GPP) Recruiting NCT03886246 Phase 2 Spesolimab;Spesolimab
16 Multi-centre, Open-label, Single Arm, Phase I Study to Investigate Safety, Tolerability, Pharmacokinetics, Pharmacogenomics and Efficacy of a Single Intravenous Dose of BI 655130 in Patients With Active Generalized Pustular Psoriasis. Completed NCT02978690 Phase 1 BI655130
17 Infliximab BS for Intravenous Drip Infusion 100 mg "Pfizer" General Investigation (Psoriasis Vulgaris, Psoriasis Arthropathica, Pustular Psoriasis, or Erythrodermic Psoriasis) Recruiting NCT03885089 Infliximab [infliximab biosimilar 3]
18 Palmoplantar Pustulosis and Generalized Pustular Psoriasis: A National Population-based Analysis of Prevalence Active, not recruiting NCT04566471
19 International Rare And Severe Psoriasis Expert Network (IRASPEN) - A Prospective Registry With Genotype-Phenotype Correlation Not yet recruiting NCT04359394

Search NIH Clinical Center for Pustular Psoriasis

Genetic Tests for Pustular Psoriasis

Anatomical Context for Pustular Psoriasis

MalaCards organs/tissues related to Pustular Psoriasis:

40
Neutrophil, Tongue, Skin, T Cells, Endothelial, Thyroid, Myeloid

Publications for Pustular Psoriasis

Articles related to Pustular Psoriasis:

(show top 50) (show all 1401)
# Title Authors PMID Year
1
Treatment of acrodermatitis continua of Hallopeau with TNF-blocking agents: case report and review. 54 61
20110631 2010
2
Occurrence of plantar pustular psoriasis during treatment with infliximab. 54 61
19298770 2009
3
Pustular psoriasis following treatment of rheumatoid arthritis with TNF-alpha inhibitors. 54 61
19112764 2008
4
Manifestation of palmoplantar pustulosis during or after infliximab therapy for plaque-type psoriasis: report on five cases. 61 54
18239925 2008
5
Treatment of pustular psoriasis with infliximab. 61 54
17451384 2007
6
Tumor necrosis factor inhibitor-induced pustular psoriasis? 54 61
17224374 2007
7
Sequential use of infliximab and etanercept in generalized pustular psoriasis. 61 54
17036664 2006
8
Treatment of recalcitrant pustular psoriasis with infliximab: effective reduction of chemokine expression. 54 61
15149518 2004
9
Levels of proelafin peptides in the sera of the patients with generalized pustular psoriasis and pustulosis palmoplantaris. 61 54
10877128 2000
10
SKALP/elafin gene polymorphisms are not associated with pustular forms of psoriasis. 54 61
9727750 1998
11
Skin-derived antileukoproteinase (SKALP) is decreased in pustular forms of psoriasis. A clue to the pathogenesis of pustule formation? 61 54
8931865 1996
12
Topographic relation between skin-derived antileukoproteinase (SKALP) and leukocyte elastase in a case of annular pustular psoriasis. 61 54
7604637 1995
13
Is sustained production of tumor necrosis factor-alpha relevant to the development of pustular psoriasis? 61 54
8358109 1993
14
Pustular psoriasis of pregnancy managed with labor induction. 61
33637333 2021
15
Facial involvement in annular pustular psoriasis: a rare clinical presentation. 61
33247839 2021
16
Unmet Medical Needs in the Treatment and Management of Generalized Pustular Psoriasis Flares: Evidence from a Survey of Corrona Registry Dermatologists. 61
33638115 2021
17
Novel IL36RN Mutation Identified in Pediatric-Onset Generalized Pustular Psoriasis Causes IL36 Antagonist Degradation. 61
33415665 2021
18
Drug survival in the treatment of generalized pustular psoriasis: A retrospective multicenter study. 61
33497511 2021
19
Study protocol of the global Effisayil 1 Phase II, multicentre, randomised, double-blind, placebo-controlled trial of spesolimab in patients with generalized pustular psoriasis presenting with an acute flare. 61
33785490 2021
20
Economic burden of generalized pustular psoriasis and palmoplantar pustulosis in the United States. 61
33622113 2021
21
Pustular Psoriasis and Associated Musculoskeletal Disorders. 61
33722939 2021
22
Validation of psoriasis disability index (PDI) questionnaire Sinhala version. 61
33675385 2021
23
Successful treatment of paediatric generalized pustular psoriasis with secukinumab: a case series. 61
33706651 2021
24
Case of generalized pustular psoriasis preceded by acute generalized pustular bacterid-like eruptions on the palms and soles. 61
33715211 2021
25
[Successful treatment of generalized pustular psoriasis with certolizumab]. 61
33687481 2021
26
Similarity and difference between palmoplantar pustulosis and pustular psoriasis. 61
33650702 2021
27
Perspectives on the pharmacological management of psoriasis in pediatric and adolescent patients. 61
33784929 2021
28
Salt for the treatment of pyogenic granuloma in a patient with pustular psoriasis of palms. 61
32750179 2021
29
Response to "New-onset pustular psoriasis in the setting of severe acute respiratory syndrome coronavirus 2 infection causing coronavirus disease 2019". 61
33688574 2021
30
Pustular psoriasis associated with bullous pemphigoid with fluctuating anti-BP180 antibody titre according to the condition of pustular lesions. 61
33648907 2021
31
CCR4-positive peripheral T-cell lymphoma presenting as eosinophilic pneumonia and developing from prolonged pustular psoriasis. 61
33641488 2021
32
Treatment of acrodermatitis continua of hallopeau with ixekizumab. 61
31184530 2021
33
"Autoinflammatory psoriasis"-genetics and biology of pustular psoriasis. 61
32814870 2021
34
Involvement of the cytokine TWEAK in the pathogenesis of psoriasis vulgaris, pustular psoriasis, and erythrodermic psoriasis. 61
33302223 2021
35
Efficacy and safety of secukinumab in moderate to severe palmoplantar pustular psoriasis over 148 weeks: Extension of the 2PRECISE study. 61
32565211 2021
36
Significance of IL36RN mutation analyses in the management of impetigo herpetiformis: A case report and review of published cases. 61
33599999 2021
37
Psoriasis Flares Following Systemic Glucocorticoid Exposure in Patients With a History of Psoriasis. 61
33206132 2021
38
The interleukin-1 family cytokines in psoriasis: pathogenetic role and therapeutic perspectives. 61
33538202 2021
39
Prologue: 2020 Annual Meeting of the Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA). 61
33589551 2021
40
Improved quality of life of patients with generalized pustular psoriasis in Japan: A cross-sectional survey. 61
33099802 2021
41
Excellent response to secukinumab in an infant with severe generalized pustular psoriasis. 61
33543522 2021
42
Generalized Pustular Psoriasis in Patients with Interferon Gamma (IFN-γ) Receptor Deficiency and Mycobacterial Infection. 61
33502705 2021
43
Generalized pustular psoriasis following COVID-19. 61
33244885 2021
44
HLA-Cw1 and Psoriasis. 61
33460021 2021
45
Generalized pustular psoriasis associated with systemic lupus erythematosus successfully treated with secukinumab. 61
33063316 2021
46
Interleukin-36: Structure, Signaling and Function. 61
32026417 2021
47
Simultaneous occurrence of Pyoderma Gangrenosum and Palmoplantar Pustular Psoriasis: Is it an association or coincidental findings? 61
33489191 2021
48
Cutaneous T-cell lymphoma mimicking palmoplantar pustular psoriasis: A rare variant. 61
33386610 2021
49
Enhanced IL-36R signaling promotes barrier impairment and inflammation in skin and intestine. 61
33443029 2020
50
New-onset pustular psoriasis in the setting of severe acute respiratory syndrome coronavirus 2 infection causing coronavirus disease 2019. 61
33102669 2020

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
10.95 TNF ELANE
2 10.3 TNF ELANE

GO Terms for Pustular Psoriasis

Cellular components related to Pustular Psoriasis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.35 TNF SLPI PI3 IL36RN ELANE
2 specific granule lumen GO:0035580 9.16 SLPI ELANE
3 extracellular space GO:0005615 9.02 TNF SLPI PI3 IL36RN ELANE

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

# Name GO ID Score Top Affiliating Genes
1 innate immune response GO:0045087 9.65 SLPI PI3 IL36RN
2 immune response GO:0006955 9.63 TNF SLPI IL36RN
3 negative regulation of interleukin-6 production GO:0032715 9.43 TNF IL36RN
4 positive regulation of smooth muscle cell proliferation GO:0048661 9.4 TNF ELANE
5 positive regulation of MAP kinase activity GO:0043406 9.37 TNF ELANE
6 positive regulation of interleukin-8 production GO:0032757 9.32 TNF ELANE
7 antimicrobial humoral response GO:0019730 9.26 PI3 ELANE
8 antibacterial humoral response GO:0019731 9.16 SLPI PI3
9 negative regulation of viral genome replication GO:0045071 8.96 TNF SLPI
10 defense response to bacterium GO:0042742 8.8 TNF SLPI ELANE

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

# Name GO ID Score Top Affiliating Genes
1 protease binding GO:0002020 9.16 TNF ELANE
2 serine-type endopeptidase inhibitor activity GO:0004867 8.96 SLPI PI3
3 endopeptidase inhibitor activity GO:0004866 8.62 SLPI PI3

Sources for Pustular Psoriasis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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