MCID: PST059
MIFTS: 41

Pustular Psoriasis

Categories: Blood diseases, Bone diseases, Genetic diseases, Rare diseases, Skin 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 palmaris et plantaris. 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 Etanercept and Miconazole 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 139)
# Related Disease Score Top Affiliating Genes
1 psoriasis 14, pustular 34.3 AP1S3 IL36RN
2 pustulosis palmaris et plantaris 32.9 AP1S3 ELANE IL36RN TNF
3 pustulosis of palm and sole 32.6 AP1S3 IL36RN TNF
4 impetigo herpetiformis 32.3 CARD14 ELANE IL36RN PI3
5 psoriasis 31.2 AP1S3 CARD14 IL36RN PI3 TNF
6 acrodermatitis 30.8 AP1S3 IL36RN
7 psoriatic arthritis 30.5 IL36RN TNF
8 geographic tongue 30.3 IL36RN TNF
9 impetigo 30.0 ELANE IL36RN PI3
10 vasculitis 30.0 ELANE TNF
11 pneumonia 29.9 ELANE TNF
12 acute respiratory distress syndrome 29.9 ELANE PI3 TNF
13 netherton syndrome 29.8 ELANE PI3
14 skin disease 29.3 CARD14 IL36RN PI3 TNF
15 psoriasis 15, pustular 12.0
16 psoriasis 1 11.4
17 arthritis 10.5
18 psoriasis 2 10.5
19 psoriasis 7 10.5
20 psoriasis 11 10.5
21 psoriasis 13 10.5
22 acute generalized exanthematous pustulosis 10.4
23 subcorneal pustular dermatosis 10.3
24 dermatitis 10.2
25 hepatitis 10.2
26 turner syndrome 10.2
27 amyloidosis 10.2
28 pemphigus 10.2
29 crohn's disease 10.1
30 hypoparathyroidism 10.1
31 capillary leak syndrome 10.1
32 hepatitis c 10.1
33 contact dermatitis 10.1
34 allergic contact dermatitis 10.1
35 thyroiditis 10.1
36 bullous pemphigoid 10.1
37 dermatophytosis 10.1
38 cholangitis 10.1
39 systemic capillary leak syndrome 10.1
40 pityriasis rubra pilaris 10.1 CARD14 IL36RN
41 pyoderma 10.1 IL36RN TNF
42 systemic lupus erythematosus 10.0
43 rheumatoid arthritis 10.0
44 pemphigus foliaceus 10.0
45 spondylitis 10.0
46 lupus erythematosus 10.0
47 viral infectious disease 10.0
48 acquired hemophilia 10.0
49 trichuriasis 10.0 PI3 TNF
50 irritant dermatitis 10.0 PI3 TNF

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 36)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Etanercept Approved, Investigational Phase 4,Phase 3 185243-69-0
2
Miconazole Approved, Investigational, Vet_approved Phase 4 22916-47-8 4189
3 Antifungal Agents Phase 4
4 Gastrointestinal Agents Phase 4,Phase 3
5 Anti-Inflammatory Agents Phase 4,Phase 3
6 Immunologic Factors Phase 4,Phase 3,Phase 2
7 Antirheumatic Agents Phase 4,Phase 3,Phase 2
8 Anti-Infective Agents Phase 4,Phase 3
9 Cyclosporins Phase 4
10 Immunosuppressive Agents Phase 4,Phase 2,Phase 3
11 Calcineurin Inhibitors Phase 4
12 Anti-Inflammatory Agents, Non-Steroidal Phase 4,Phase 3
13 Dermatologic Agents Phase 4,Phase 3,Phase 2
14 Analgesics Phase 4,Phase 3
15 Peripheral Nervous System Agents Phase 4,Phase 3
16 Tin Fluorides Phase 4
17 Analgesics, Non-Narcotic Phase 4,Phase 3
18 Antibodies, Monoclonal Phase 4,Phase 3,Phase 2
19 Antibodies Phase 4,Phase 3,Phase 2
20 Immunoglobulins Phase 4,Phase 3,Phase 2
21
Adalimumab Approved Phase 3 331731-18-1 16219006
22 tannic acid Approved Phase 2, Phase 3,Phase 3
23
Benzocaine Approved, Investigational Phase 2, Phase 3,Phase 3 94-09-7, 1994-09-7 2337
24
Certolizumab pegol Approved Phase 2, Phase 3 428863-50-7
25
Infliximab Approved Phase 3 170277-31-3
26
Apremilast Approved, Investigational Phase 3 608141-41-9 11561674
27
Ustekinumab Approved, Investigational Phase 3 815610-63-0
28 Pharmaceutical Solutions Phase 2, Phase 3
29 Anti-Bacterial Agents Phase 3
30 Angiogenesis Modulating Agents Phase 3
31 Angiogenesis Inhibitors Phase 3
32 Immunoglobulin A Phase 3
33 Vaccines Phase 2
34 Interleukin 1 Receptor Antagonist Protein Phase 2
35
BCG vaccine Investigational
36 tyrosine Not Applicable

Interventional clinical trials:

(show all 27)
# 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 An Efficacy and Safety Study of CNTO1959 (Guselkumab) in the Treatment of Participants With Generalized Pustular Psoriasis or Erythrodermic Psoriasis Completed NCT02343744 Phase 3 Guselkumab
4 Palmoplantar Pustular Psoriasis Efficacy and Safety With Secukinumab Completed NCT02008890 Phase 3
5 Study to Investigate Efficacy and Safety of Adalimumab in Japanese Subjects With Generalized Pustular Psoriasis (GPP) Completed NCT02533375 Phase 3 Adalimumab
6 Study to Assess the Efficacy, Safety and Tolerability of Secukinumab in Japanese Subjects With Generalized Pustular Psoriasis (GPP) Completed NCT01952015 Phase 3
7 An Open-label, Non-controlled Study of KHK4827 in Subjects With Psoriasis Completed NCT01782937 Phase 3 KHK4827
8 A Phase 3 Clinical Study of KHK 4827 Completed NCT02052609 Phase 3 KHK4827 140mg SC;KHK4827 210mg SC
9 A Study to Test the Efficacy and Safety of Certolizumab Pegol in Japanese Subjects With Moderate to Severe Chronic Psoriasis Completed NCT03051217 Phase 2, Phase 3 Certolizumab Pegol
10 A Clinical Study of Increased Dose of TA-650 in Patients With Psoriasis Completed NCT01680159 Phase 3 TA-650
11 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
12 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
13 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
14 Extension Study of Secukinumab Prefilled Syringes in Subjects With Moderate to Severe Chronic Plaque-type Psoriasis Completing Preceding Psoriasis Phase III Studies With Secukinumab Completed NCT01544595 Phase 3 Secukinumab (AIN457);Placebo
15 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
16 Efficacy and Safety Study of Apremilast (CC-10004) in Pediatric Subjects From 6 Through 17 Years of Age With Moderate to Severe Plaque Psoriasis Not yet recruiting NCT03701763 Phase 3 Apremilast (CC-10004)
17 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)
18 Initial Dosing of BI 655130 in Palmoplantar Pustulosis Patients Completed NCT03135548 Phase 2 BI 655130 (low dose);Placebo;BI 655130 (high dose)
19 Determination of the Efficacy and Safety of Psirelax in the Relief of the Disease in Psoriasis Completed NCT01000714 Phase 2 Psirelax
20 Study to Evaluate the Efficacy of Alefacept to Treat Palmar Plantar Pustulosis Completed NCT00301002 Phase 2 Alefacept
21 A Study to Evaluate the Efficacy and Safety of ANB019 in Subjects With Generalized Pustular Psoriasis Recruiting NCT03619902 Phase 2 ANB019
22 Anakinra for Inflammatory Pustular Skin Diseases Recruiting NCT01794117 Phase 2 Anakinra
23 A Study to Test BI 655130 in Patients With a Flare-up of a Skin Disease Called Generalized Pustular Psoriasis Not yet recruiting NCT03782792 Phase 2 BI 655130;Placebo
24 BI655130 Single Dose in Generalized Pustular Psoriasis Completed NCT02978690 Phase 1 BI655130
25 Role of Tyrosine Kinase Lyn and Cleaved Form by Caspases in Psoriasis Unknown status NCT01538342 Not Applicable
26 The Psoriasis, Atherosclerosis, and Cardiometabolic Disease Initiative (PACI) Recruiting NCT01778569
27 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, Bone, Tongue, Thyroid, Lung

Publications for Pustular Psoriasis

Articles related to Pustular Psoriasis:

(show top 50) (show all 655)
# Title Authors Year
1
Secukinumab-induced paradoxical pustular psoriasis. ( 30129214 )
2019
2
Paradoxical pustular psoriasis induced by ustekinumab in a patient with Crohn's disease-associated spondyloarthropathy. ( 29705819 )
2018
3
The heterogeneous mutational landscape of pustular psoriasis. ( 29595236 )
2018
4
RNA sequencing-based longitudinal transcriptomic profiling gives novel insights into the disease mechanism of generalized pustular psoriasis. ( 29871627 )
2018
5
Pustular psoriasis and related pustular skin diseases. ( 29333670 )
2018
6
Increased levels of lipocalin 2 in palmoplantar pustular psoriasis. ( 29395573 )
2018
7
Pustular psoriasis of pregnancy: current perspectives. ( 29520163 )
2018
8
An infant with generalized pustular psoriasis and geographic tongue had a heterozygous IL36RN mutation and IgG2 deficiency. ( 29454537 )
2018
9
A case of pustular psoriasis of pregnancy with positive maternal-fetal outcomes. ( 29763478 )
2018
10
Pediatric case of generalized pustular psoriasis developing acute pancreatitis. ( 29655246 )
2018
11
Acrodermatitis continua of Hallopeau and geographic tongue are variants of pustular psoriasis. ( 29687071 )
2018
12
ARDS complicating pustular psoriasis: treatment with low-dose corticosteroids, vitamin C and thiamine. ( 29420246 )
2018
13
Genetic background and therapeutic response in generalized pustular psoriasis patients treated with granulocyte and monocyte adsorption apheresis. ( 29380726 )
2018
14
Systemic Monotherapy Treatments for Generalized Pustular Psoriasis: A Systematic Review. ( 29707979 )
2018
15
Generalized pustular psoriasis: a review and update on treatment. ( 29573491 )
2018
16
Generalized Pustular Psoriasis ( 29630241 )
2018
17
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
18
Case of generalized pustular psoriasis that might have progressed from terbinafine-induced acute generalized exanthematous pustulosis. ( 29766566 )
2018
19
Two cases of generalized pustular psoriasis complicated by IgG4-related disease. ( 29709076 )
2018
20
Generalized Pustular Psoriasis Induced by Infliximab in a Patient With Inflammatory Bowel Disease. ( 29484893 )
2018
21
Generalized pustular psoriasis - A model disease for specific targeted immunotherapy, systematic review. ( 29852521 )
2018
22
Generalized pustular psoriasis triggered by Zika virus infection. ( 29027743 )
2018
23
Association of IL36RN mutations with clinical features, therapeutic response to acitretin, and frequency of recurrence in patients with generalized pustular psoriasis. ( 29619998 )
2018
24
Successful treatment with interleukin-17A antagonists of generalized pustular psoriasis in patients without IL36RN mutations. ( 29655177 )
2018
25
Highlighting Interleukin-36 Signalling in Plaque Psoriasis and Pustular Psoriasis. ( 28967976 )
2018
26
Severe hypocalcemia-related pustular psoriasis in an 83-year-old woman with Fahr's syndrome: a case report. ( 29953675 )
2018
27
A Case of Ceftriaxone-Induced Acute Generalized Exanthematous Pustulosis/Generalized Pustular Psoriasis Overlap. ( 29681811 )
2018
28
Investigation of Psoriasis Susceptibility Loci in Psoriatic Arthritis and a Generalized Pustular Psoriasis Cohort. ( 30471759 )
2018
29
Recalcitrant annular pustular psoriasis associated with psoriatic arthritis successfully treated with secukinumab. ( 30246137 )
2018
30
Generalized pustular psoriasis complicated with bullous pemphigoid. ( 30516289 )
2018
31
Successful therapy with secukinumab in a patient with generalized pustular psoriasis carrying homozygous IL36RN p.His32Arg mutation. ( 30430544 )
2018
32
Gastrointestinal bleeding with severe mucosal involvement in a patient with generalized pustular psoriasis without IL36RN mutation. ( 30474867 )
2018
33
Secukinumab in Generalized Pustular Psoriasis. ( 30505796 )
2018
34
Palmar pustular psoriasis associated with teriflunomide treatment. ( 30513502 )
2018
35
Treatment of pustular psoriasis with anakinra: a statistical analysis plan for stage 1 of an adaptive two-staged randomised placebo-controlled trial. ( 30285894 )
2018
36
Adalimumab treatment in Japanese patients with generalized pustular psoriasis: Results of an open-label phase 3 study. ( 30302793 )
2018
37
Long-term efficacy and safety of ixekizumab in Japanese patients with erythrodermic or generalized pustular psoriasis: subgroup analyses of an open-label, phase 3 study (UNCOVER-J). ( 30317671 )
2018
38
Variants of CARD14 are predisposing factors for generalized pustular psoriasis (GPP) with psoriasis vulgaris but not for GPP alone in a Chinese population. ( 30387497 )
2018
39
Japanese guidelines for the management and treatment of generalized pustular psoriasis: The new pathogenesis and treatment of GPP. ( 30230572 )
2018
40
Successful treatment of refractory juvenile generalized pustular psoriasis with secukinumab monotherapy: A case report and review of published work. ( 30230584 )
2018
41
Genetic polymorphism of IL36RN in Han patients with generalized pustular psoriasis in Sichuan region of China: A case-control study. ( 30075588 )
2018
42
Generalized pustular psoriasis as an unusual cause of acquired haemophilia A. ( 30105997 )
2018
43
Successful treatment of palmoplantar pustular psoriasis with brodalumab. ( 30129522 )
2018
44
Refractory Palmoplantar Pustular Psoriasis Treated With High-Dose Ustekinumab After Initial Failure. ( 30141337 )
2018
45
Terbinafine-induced generalized pustular psoriasis in a patient carrying CARD14 mutation. ( 30144153 )
2018
46
A small population, randomised, placebo-controlled trial to determine the efficacy of anakinra in the treatment of pustular psoriasis: study protocol for the APRICOT trial. ( 30157880 )
2018
47
Clinical and genetic differences between pustular psoriasis subtypes. ( 30036598 )
2018
48
A newly revealed IL36RN mutation in sibling cases complements our IL36RN mutation statistics for generalized pustular psoriasis. ( 27802907 )
2017
49
Generalized pustular psoriasis in a 92-year-old man with a homozygous nonsense mutation in IL36RN. ( 29215143 )
2017
50
A dramatic response to a single dose of infliximab in a patient with prolonged pustular psoriasis derived from inverse psoriasis. ( 28419647 )
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 9.48 CARD14 TNF
2 positive regulation of JNK cascade GO:0046330 9.46 IL36RN TNF
3 tumor necrosis factor-mediated signaling pathway GO:0033209 9.43 CARD14 TNF
4 positive regulation of MAP kinase activity GO:0043406 9.4 ELANE TNF
5 positive regulation of interleukin-6 production GO:0032755 9.37 IL36RN TNF
6 positive regulation of smooth muscle cell proliferation GO:0048661 9.32 ELANE TNF
7 antimicrobial humoral response GO:0019730 9.26 ELANE PI3
8 negative regulation of interleukin-6 production GO:0032715 9.16 IL36RN TNF
9 negative regulation of growth of symbiont in host GO:0044130 8.96 ELANE TNF
10 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
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10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
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32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
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62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
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74 UMLS via Orphanet
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