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Any psoriasis in which the cause of the disease is a mutation in the IL36RN gene.
Features include always present findings: Parakeratosis, Epidermal acanthosis, Elevated CRP (inflammation marker) (elevated circulating c-reactive protein concentration), and Increased total neutrophil count and others; and sometimes findings: Geographic tongue, Polyarticular arthritis, Furrowed tongue, and Oligoarthritis and others. 15 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Skin | 3 | Erythema, Nail dystrophy, Psoriasiform dermatitis |
Blood and immune system | 2 | Increased total neutrophil count, Elevated white blood cell count (increased total leukocyte count) |
Bones and joints | 1 | Polyarticular arthritis |
Lab test results | 1 | Elevated CRP (inflammation marker) (elevated circulating c-reactive protein concentration) |
Metabolism | 1 | Fever |
Age of onset: adulthood.
IL36RN encodes interleukin 36 receptor antagonist (155 aa). Inhibits the activity of interleukin-36 (IL36A,IL36B and IL36G) by binding to receptor IL1RL2 and preventing its association with the coreceptor IL1RAP for signaling. Highest expression in Skin Sun Exposed Lower leg (82.0 TPM) and Skin Not Sun Exposed Suprapubic (49.1 TPM).
Psoriasis 14, pustular is associated with mutations in the IL36RN gene on chromosome 2.
The IL36RN protein participates in Interleukin-36 pathway pathway.
IL36RN is classified as a druggable target (Druggable Genome category) with score 0.0.
Genetic testing for IL36RN is available. Testing is considered confirmatory for diagnosis.
Biomarker and diagnostic research for psoriasis 14, pustular has been reported in the published literature.
No approved treatments are currently available for psoriasis 14, pustular. An additional 1 compound holds orphan drug designation.
While no drugs are FDA-approved specifically for psoriasis 14, pustular, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for psoriasis 14, pustular. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor | Designated | Exclusivity End | Designation Status |
|---|---|---|---|---|---|
Spevigo | spesolimab-sbzo | Boehringer Ingelheim Pharmaceuticals, Inc. (BI) | 2018 | 2031 | Designated (drug approved for other indication) |
Gene therapy approaches for psoriasis 14, pustular have been reported in the published literature.
20 trials found
Phenotype severity distribution: 6 always present features.
Estimated prevalence: <1 in 1,000,000 (VERY_RARE).
20 clinical trials registered, 9 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 2 PHASE4, 4 PHASE3, 5 PHASE2. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT06100991](https://clinicaltrials.gov/study/NCT06100991) | CorEvitas Generalized Pustular Psoriasis (GPP) Drug Safety and Effectiveness Registry | — | CorEvitas | RECRUITING |
[NCT06477536](https://clinicaltrials.gov/study/NCT06477536) | Long-Term Safety and Efficacy of HB0034 in Subjects With Generalized Pustular Psoriasis | PHASE2 | Shanghai Huaota Biopharmaceutical Co., Ltd. | RECRUITING |
[NCT07013201](https://clinicaltrials.gov/study/NCT07013201) | A 16-week Trial to Investigate the Efficacy and Safety of Delgocitinib Cream 20 mg/g in Adult Participants With Mild to Severe Palmoplantar Pustulosis | PHASE2 | LEO Pharma | RECRUITING |
[NCT07270003](https://clinicaltrials.gov/study/NCT07270003) | A Prospective, Single-arm, Open-label Clinical Trial to Evaluate the Efficacy and Safety of Ivarmacitinib in the Treatment of Palmoplantar Pustulosis | NA | Xijing Hospital | RECRUITING |
[NCT05994976](https://clinicaltrials.gov/study/NCT05994976) | Collection of Samples From Subjects With Various Skin Conditions and Healthy Volunteers | — | Innovaderm Research Inc. | RECRUITING |
202 publications have been identified in PubMed for psoriasis 14, pustular. Research spans Epidemiology / Natural History (20%), Review / Meta-Analysis (20%), and Case Report / Case Series (20%).
Research Type | Count | % of Total |
|---|---|---|
Disease patterns and progression | 41 | 20% |
Research summaries | 40 | 20% |
Patient case studies | 40 | 20% |
Clinical study results | 38 | 19% |
Laboratory research | 20 | 10% |
Other research | 12 |
Takahashi Y (2026). [PMID: 41670195](https://pubmed.ncbi.nlm.nih.gov/41670195/). *J Dermatol*. [Epidemiology / Natural History]
Cravo JM (2026). [PMID: 41489172](https://pubmed.ncbi.nlm.nih.gov/41489172/). *J Dermatol*. [Case Report / Case Series]
Gleeson D (2026). [PMID: 40452648](https://pubmed.ncbi.nlm.nih.gov/40452648/). *Journal of the European Academy of Dermatology and Venereology : JEADV*. [Review / Meta-Analysis]
Lee H (2026). [PMID: 41740929](https://pubmed.ncbi.nlm.nih.gov/41740929/). *J Allergy Clin Immunol*. [Basic Science / Preclinical]
Mori Y (2026). [PMID: 41845909](https://pubmed.ncbi.nlm.nih.gov/41845909/). *J Dermatol*. [Other]
Delmotte A (2026). [PMID: 41713591](https://pubmed.ncbi.nlm.nih.gov/41713591/). *J Am Acad Dermatol*. [Epidemiology / Natural History]
Zheng YX (2026). [PMID: 41061969](https://pubmed.ncbi.nlm.nih.gov/41061969/). *J Am Acad Dermatol*. [Epidemiology / Natural History]
Mai SZ (2026). [PMID: 41549598](https://pubmed.ncbi.nlm.nih.gov/41549598/). *J Dtsch Dermatol Ges*. [Case Report / Case Series]
Kishimoto M (2026). [PMID: 42076875](https://pubmed.ncbi.nlm.nih.gov/42076875/). *J Dermatol*. [Basic Science / Preclinical]
Tao Y (2026). [PMID: 42183095](https://pubmed.ncbi.nlm.nih.gov/42183095/). *JAAD Case Rep*. [Case Report / Case Series]
Data assembled from 8 of 12 sources · Last updated Sep 20, 2026, 11:14 AM UTC
Online Mendelian Inheritance in Man
European rare disease database
Genetic and Rare Diseases Info Center
Testing and diagnosis research | 6 | 3% |
New treatment approaches | 5 | 2% |
AI-curated news mentioning psoriasis 14, pustular
Updated Sep 3, 2026
A Phase 2 randomized trial demonstrates the efficacy and safety of recibokibart, an interleukin-36 receptor antibody, in treating generalized pustular psoriasis. This study contributes valuable data to the understanding of targeted therapies for this rare skin condition.
A recent study identifies a Behçet's-like syndrome linked to psoriasis and lichen sclerosus, associated with a variant in the NF-κB1 gene. This discovery may provide insights into the genetic underpinnings of these conditions.
A recent study published in PubMed evaluates the effectiveness and safety of spesolimab in treating adolescents and adults with generalized pustular psoriasis. The findings contribute to the understanding of treatment options for this rare skin condition.
Takeda's investigational psoriasis pill demonstrated total skin clearance in over 35% of patients at 16 weeks, outperforming Bristol Myers Squibb's Sotyktu by more than 2.5 times. This promising data positions Takeda for a potential FDA submission.
A new long-acting psoriasis therapy shows promising results in mid-stage trials, potentially offering a more effective treatment option with less frequent dosing compared to AbbVie's Skyrizi. This advancement could significantly impact the psoriasis treatment landscape.