Preventing generalised pustular psoriasis.

Generalised pustular psoriasis (GPP) is a rare disease variant characterised by relapsing flares of widespread pustules and painful erythema on skin, accompanied by systemic symptoms such as fever and fatigue. 1 Griffiths CEM Armstrong AW Gudjonsson JE Barker J Psoriasis. Lancet. 2021; 397: 1301-1315 Summary Full Text Full Text PDF PubMed Scopus (550) Google Scholar , 2 Prinz JC Choon SE Griffiths CEM et al. Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review. J Eur Acad Dermatol Venereol. 2023; 37: 256-273 Crossref PubMed Scopus (12) Google Scholar Meta-analyses have indicated greater risk in people with east Asian genetic backgrounds. 3 Feng JN Guo JZ Zhang Q et al. Higher prevalence of generalized pustular psoriasis in Asia? A population-based study using claim data in China and a systematic review. Dermatology. 2023; 239: 195-205 Crossref PubMed Scopus (3) Google Scholar GPP appears to present most frequently after the age of 40 years, although the juvenile form is not uncommon, with variable degrees of chronic, baseline skin inflammation. 4 Kharawala S Golembesky AK Bohn RL Esser D The clinical, humanistic, and economic burden of generalized pustular psoriasis: a structured review. Expert Rev Clin Immunol. 2020; 16: 239-252 Crossref PubMed Scopus (46) Google Scholar Flares can cause cardiovascular failure and even death, particularly in patients older than 50 years. 2 Prinz JC Choon SE Griffiths CEM et al. Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review. J Eur Acad Dermatol Venereol. 2023; 37: 256-273 Crossref PubMed Scopus (12) Google Scholar , 5 Zema CL Valdecantos WC Weiss J Krebs B Menter AM Understanding flares in patients with generalized pustular psoriasis documented in US electronic health records. JAMA Dermatol. 2022; 158: 1142-1148 Crossref PubMed Scopus (8) Google Scholar , 6 Ohn J Choi Y-G Yun J Jo SJ Identifying patients with deteriorating generalized pustular psoriasis: development of a prediction model. J Dermatol. 2022; 49: 675-681 Crossref PubMed Scopus (1) Google Scholar Efficacy and safety of subcutaneous spesolimab for the prevention of generalised pustular psoriasis flares (Effisayil 2): an international, multicentre, randomised, placebo-controlled trialHigh-dose spesolimab was superior to placebo in GPP flare prevention, significantly reducing the risk of a GPP flare and flare occurrence over 48 weeks. Given the chronic nature of GPP, a treatment for flare prevention is a significant shift in the clinical approach, and could ultimately lead to improvements in patient morbidity and quality of life. Full-Text PDF