Medizinonline Medizinonline
  • News
    • News
    • Market & Medicine
  • Patients
    • Disease patterns
    • Diagnostics
    • Therapy
  • Partner Content
    • Dermatology
      • Atopic dermatitis and psoriasis news
      • Dermatology News
    • Diabetes
      • Dia-Month Club – Type 2 Diabetes
      • Diabetes in Motion
      • Diabetes Podcasts
    • Gastroenterology
      • IBD matters
      • Forum Gastroenterology
      • Ozanimod: ulcerative colitis
      • Reflux Update
    • Immunology
      • Primary immunodeficiency – exchange of experience
    • Vaccinate
      • Herpes zoster
    • Infektiologie
    • Neurology
      • EXPERT ULTRASONIC: Introduction to ultrasound-guided injection
      • MS News
      • MS Therapy in Transition
    • Oncology
      • Swiss Oncology in motion
    • Orthopedics
      • Osteoporosis in motion
    • Phytotherapie
    • Practice Management
      • Aargau Cantonal Bank
      • Claraspital
    • Psychiatry
      • Geriatric Psychiatry
    • Rheumatology
  • Departments
    • Allergology and clinical immunology
    • General Internal Medicine
    • Anesthesiology
    • Angiology
    • Surgery
    • Dermatology and venereology
    • Endocrinology and Diabetology
    • Nutrition
    • Gastroenterology and Hepatology
    • Genetics
    • Geriatrics
    • Gynecology
    • Hematology
    • Infectiology
    • Cardiology
    • Nephrology
    • Neurology
    • Emergency and intensive care medicine
    • Nuclear Medicine
    • Oncology
    • Ophthalmology
    • ORL
    • Orthopedics
    • Pediatrics
    • Pharmacology and toxicology
    • Pharmaceutical medicine
    • Phlebology
    • Physical medicine and rehabilitation
    • Pneumology
    • Prevention and health care
    • Psychiatry and psychotherapy
    • Radiology
    • Forensic Medicine
    • Rheumatology
    • Sports Medicine
    • Traumatology and trauma surgery
    • Tropical and travel medicine
    • Urology
    • Dentistry
  • CME & Congresses
    • CME continuing education
    • Congress Reports
    • Congress calendar
  • Practice
    • Noctimed
    • Practice Management
    • Jobs
    • Interviews
  • Log In
  • Register
  • My account
  • Contact
  • English
    • Deutsch
    • Français
    • Italiano
    • Português
    • Español
  • Publications
  • Contact
  • Deutsch
  • English
  • Français
  • Italiano
  • Português
  • Español
Subscribe
Medizinonline Medizinonline
Medizinonline Medizinonline
  • News
    • News
    • Market & Medicine
  • Patienten
    • Krankheitsbilder
    • Diagnostik
    • Therapie
  • Partner Content
    • Dermatology
      • Atopic dermatitis and psoriasis news
      • Dermatology News
    • Diabetes
      • Dia-Month Club – Type 2 Diabetes
      • Diabetes in Motion
      • Diabetes Podcasts
    • Gastroenterology
      • IBD matters
      • Forum Gastroenterology
      • Ozanimod: ulcerative colitis
      • Reflux Update
    • Immunology
      • Primary immunodeficiency – exchange of experience
    • Vaccinate
      • Herpes zoster
    • Infektiologie
    • Neurology
      • EXPERT ULTRASONIC: Introduction to ultrasound-guided injection
      • MS News
      • MS Therapy in Transition
    • Oncology
      • Swiss Oncology in motion
    • Orthopedics
      • Osteoporosis in motion
    • Phytotherapie
    • Practice Management
      • Aargau Cantonal Bank
      • Claraspital
    • Psychiatry
      • Geriatric Psychiatry
    • Rheumatology
  • Departments
    • Fachbereiche 1-13
      • Allergology and clinical immunology
      • General Internal Medicine
      • Anesthesiology
      • Angiology
      • Surgery
      • Dermatology and venereology
      • Endocrinology and Diabetology
      • Nutrition
      • Gastroenterology and Hepatology
      • Genetics
      • Geriatrics
      • Gynecology
      • Hematology
    • Fachbereiche 14-26
      • Infectiology
      • Cardiology
      • Nephrology
      • Neurology
      • Emergency and intensive care medicine
      • Nuclear Medicine
      • Oncology
      • Ophthalmology
      • ORL
      • Orthopedics
      • Pediatrics
      • Pharmacology and toxicology
      • Pharmaceutical medicine
    • Fachbereiche 26-38
      • Phlebology
      • Physical medicine and rehabilitation
      • Phytotherapy
      • Pneumology
      • Prevention and health care
      • Psychiatry and psychotherapy
      • Radiology
      • Forensic Medicine
      • Rheumatology
      • Sports Medicine
      • Traumatology and trauma surgery
      • Tropical and travel medicine
      • Urology
      • Dentistry
  • CME & Congresses
    • CME continuing education
    • Congress Reports
    • Congress calendar
  • Practice
    • Noctimed
    • Practice Management
    • Jobs
    • Interviews
Login

Sie haben noch keinen Account? Registrieren

  • Case Report

Successful treatment of an acute GPP flare-up

    • Cases
    • Congress Reports
    • Dermatology and venereology
    • Rheumatology
    • RX
    • Studies
  • 4 minute read

Generalized pustular psoriasis (GPP) is a neutrophilic, inflammatory skin disease with a potentially life-threatening course. Recent findings on immunopathogenesis indicate that the group of interleukin (IL)-36 cytokines are central drivers of autoinflammatory reactions in GPP. The monoclonal antibody spesolimab inhibits the activation of the IL-36 receptor (IL-36R) and has also been approved in Switzerland since last year for the treatment of adult patients with GPP.

The European Rare and Severe Psoriasis Expert Network ( ERASPEN) defines generalized pustular psoriasis (GPP) as a disease with primary, sterile, macroscopically visible pustules on non-acral skin. GPP is characterized by flare-ups with extensive pustule formation, which can be accompanied by systemic inflammatory reactions** and serious extracutaneous complications. The criteria for systemic inflammation are fever >38 °C and leukocytosis£ [1]. A GPP relapse is usually followed by phases with little or no disease activity, but there are also courses with persistent symptoms (>3 months). During an acute flare-up, symptoms such as pain, itching and fatigue lead to a high level of stress for those affected and, as a result, their quality of life is severely restricted [2–4]. Overexpression of IL-36 cytokines plays a central role in the immunopathogenesis of GPP (Fig. 1) [5,6]. The anti-IL-36 receptor antibody spesolimab blocks the IL-36 receptor (R) signaling pathway. Binding of spesolimab to IL-36R prevents the subsequent activation of IL-36R by its ligands (IL36 α, β and γ) and the downstream activation of proinflammatory signaling pathways [7]. This dampens inflammatory reactions and attenuates the symptoms of GPP [8]. Spesolimab (Spevigo®) is indicated for the treatment of relapses of GPP in adult patients [9]. The recommended dose is a single dose of 900 mg (2 vials of 450 mg each), administered as an intravenous infusion [8].

** Definition according to the American Society of Chest Physicians
£ Leukocytosis = number of white blood cells >12×109/l

63-year-old patient: acute GPP relapse and reduced general condition

The patient presented at [10] with an acute episode of previously known GPP that had been present for three days. On admission, the patient was in a reduced general condition (AZ) and showed pronounced skin findings with extensive erythematous plaques with multiple, partly confluent pustules on the entire integument. Laboratory chemistry revealed a significant increase in inflammatory parameters.

Previous therapies: Since initial diagnosis in 2011, treatment with methotrexate, dimethyl fumarate and ustekinumab. The latter was discontinued 6 months ago after several years due to an operation.

Current course of therapy: Initial systemic steroid therapy with 80 mg/d prednisolone. Topically, steroid-containing topicals were used. This led to further extensive formation of new pustules, so that after carrying out the necessary preliminary examinations, treatment with Spesolimab 900 mg i.v. was administered as a single dose. A significant reduction in disease activity was observed on the following day with a complete reduction in pustules just three days after the infusion and a rapid improvement in the AZ. Within the following week, the plaques clearly faded and the inflammation values decreased, so that the patient could be discharged from hospital ten days after the infusion. Prednisolone could be reduced to 60 mg/d at the time of discharge. In the course of the treatment, an outpatient reintroduction of ustekinumab is planned as maintenance therapy. In summary, treatment with spesolimab in this case showed a rapid improvement in the symptoms of acute GPP relapse, which is consistent with the results of clinical studies.

Congress: Dermatology compact & practical

Literature:

  1. Bone RC, et al: Definitions for sepsis and organ failure and guidelines for the use of innovative therapies in sepsis. The ACCP/SCCM Consensus Conference Committee. American College of Chest Physicians/Society of Critical Care Medicine. Chest 1992; 101: 1644-1655.
  2. Burden AD, et al: Symptom Experience and Content Validity of the Psoriasis Symptom Scale (PSS) in Patients with Generalized Pustular Psoriasis (GPP). Dermatol Ther (Heidelberg) 2022; 12(6): 1367-1381.
  3. Lebwohl M, et al: The Disease Burden of Generalized Pustular Psoriasis: Real World Evidence From CorEvitas’ Psoriasis Registry. Journal of Psoriasis and Psoriatic Arthritis 2022; 7(2): 71-78.
  4. Reisner DV, et al: Impact of Generalized Pustular Psoriasis from the Perspective of People Living with the Condition: Results of an Online Survey. Am J Clin Dermatol 2022; 23(Suppl 1): 65-71.
  5. Marrakchi S, et al: Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med 2011; 365: 620-628.
  6. Gooderham MJ, Van Voorhees AS, Lebwohl MG: An update on generalized pustular psoriasis. Expert Rev Clin Immunol 2019; 15: 907-919.
  7. European Medicines Agency, https://ec.europa.eu/health/documents/community-register/2022/
    20221209157574/anx_157574_en.pdf
    , (last accessed 16.05.2024)
  8. Swissmedic: Spevigo® (active substance: spesolimab), Public Summary SwissPAR of 29.02.2024, first authorization in Switzerland: 09.08.2023
  9. Swissmedic: Medicinal product information, www.swissmedicinfo.ch,(last accessed 16.05.2024)
  10. Kluck R, et al: Treatment of an acute flare of generalized pustular psoriasis with spesolimab, P046. JDDG 2024; 22, Issue S1: 1-40.
  11. Samotij D, Szczęch J, Reich A: Generalized Pustular Psoriasis: Divergence of Innate and Adaptive Immunity. International Journal of Molecular Sciences. 2021; 22(16): 9048. www.mdpi.com/1422-0067/22/16/9048,(last accessed 16.05.2024).

DERMATOLOGIE PRAXIS 2024; 34(3): 26-27 (published on 14.6.24, ahead of print)

Autoren
  • Mirjam Peter, M.Sc.
Publikation
  • DERMATOLOGIE PRAXIS
Related Topics
  • Generalized pustular psoriasis
  • GPP
  • Interleukin (IL)-36
  • Spesolimab
Previous Article
  • Polycythaemia vera

Reduce the risk of thrombosis, increase the chance of survival

  • Hematology
  • Oncology
  • RX
  • Studies
View Post
Next Article
  • T helper 1 cells

Differentiation and activation of Th1 cells – a multi-omics approach

  • RX
  • CME continuing education
  • General Internal Medicine
  • Genetics
  • Prevention and health care
View Post
You May Also Like
View Post
  • 30 min
  • Chemsex - MSM, sex, chrystal meth & co.

Medical and psychosocial perspectives

    • CME continuing education
    • General Internal Medicine
    • Infectiology
    • Pharmacology and toxicology
    • Prevention and health care
    • Psychiatry and psychotherapy
    • RX
View Post
  • 3 min
  • Bladder cancer

Tuberculosis vaccination reduces recurrences

    • Congress Reports
    • Oncology
    • Pneumology
    • Prevention and health care
    • RX
    • Studies
    • Urology
View Post
  • 4 min
  • Oral JAK-i in atopic dermatitis

Benefits and risks: What does the current data say?

    • Allergology and clinical immunology
    • Congress Reports
    • Dermatology and venereology
    • RX
    • Studies
View Post
  • 9 min
  • Chronic lung diseases

Sarcopenia and malnutrition in the context of pneumological rehabilitation

    • CME continuing education
    • General Internal Medicine
    • Geriatrics
    • Nutrition
    • Pneumology
    • RX
    • Sports Medicine
    • Studies
View Post
  • 17 min
  • Sleep-wake disorders in pneumology practice

Patience, knowledge and persistence in therapy

    • CME continuing education
    • General Internal Medicine
    • Neurology
    • Pneumology
    • RX
View Post
  • 19 min
  • Cardiovascular risk and obesity

Pathomechanisms, secondary prevention and treatment options

    • Cardiology
    • CME continuing education
    • Endocrinology and Diabetology
    • Nutrition
    • RX
    • Sports Medicine
    • Studies
    • Training with partner
View Post
  • 9 min
  • Oxidative stress and neurodegeneration

Insights and therapeutic strategies for Parkinson’s disease

    • Education
    • Neurology
    • Phytotherapy
    • RX
    • Studies
View Post
  • 4 min
  • Biologics for atopic dermatitis

Th2 cytokines in focus: proven and new targets

    • Allergology and clinical immunology
    • Congress Reports
    • Dermatology and venereology
    • Pharmacology and toxicology
    • RX
    • Studies
Top Partner Content
  • Forum Gastroenterology

    Zum Thema
  • Herpes zoster

    Zum Thema
  • Dermatology News

    Zum Thema
Top CME content
  • 1
    Patience, knowledge and persistence in therapy
  • 2
    Sarcopenia and malnutrition in the context of pneumological rehabilitation
  • 3
    Medical and psychosocial perspectives
  • 4
    New nomenclature for non-alcoholic fatty liver disease
  • 5
    Examinations and considerations before therapy

Newsletter

Sign up and stay up to date

Subscribe
Medizinonline
  • Contact
  • General terms and conditions
  • Imprint

Input your search keywords and press Enter.