Barker Jonathan N, Casanova Emmylou, Choon Siew Eng, Foley Peter, Fujita Hideki, Gonzalez César, Gooderham Melinda, Marrakchi Slaheddine, Puig Luís, Romiti Ricardo, Thaçi Diamant, Zheng Min, Strober Bruce
St John's Institute of Dermatology, Faculty of Life Sciences and Medicine, King's College London, London, UK.
Patient author.
Br J Dermatol. 2025 Mar 18;192(4):706-716. doi: 10.1093/bjd/ljae491.
Generalized pustular psoriasis (GPP) is a chronic, systemic, neutrophilic inflammatory disease. A previous Delphi panel established areas of consensus on GPP, although patient perspectives were not included and aspects of treatment goals remained unclear.
To identify and achieve consensus on refined, specific treatment goals for GPP treatment via a Delphi panel with patient participation.
Statements were generated based on a systematic literature review and revised by a Steering Committee. Statements were categorized into overarching principles, and short- and long-term treatment goals. A global panel of 30 dermatologists and 3 patient representatives voted in agreement or disagreement with each statement. Consensus was defined as ≥ 80% approval by the panellists.
Consensus was reached in the first round of voting and ≥ 90% agreement was reached for 23 of 26 statements. In summary, GPP requires a timely, tailored treatment plan, co-developed by patients and physicians, that involves a multidisciplinary approach and addresses the complexity, heterogeneity and chronicity of the disease. Short-term treatment goals should include pustule clearance within 7 days and prevention of pustule recurrence, reduction of cutaneous symptom burden (-4 or more points on the Itch and Skin Pain Numeric Rating Scale), improvement in systemic symptoms (e.g. resolution of fever within 3 days of treatment initiation and reduced fatigue), prevention of life-threatening complications and progressive improvement of inflammatory biomarkers. In patients with comorbid psoriatic diseases, treatment decisions should prioritize GPP. Long-term treatment goals should include minimizing disease activity through flare prevention and symptom control between flares, sustained disease control, management of comorbidities and improvement in quality of life (QoL). Small differences in perception between patients and physicians regarding the importance of certain treatment goals (e.g. avoiding hair and/or nail loss to improve QoL), reflect the complexity of assessing treatment goals and emphasize the need for a patient-centred approach.
In the first global Delphi panel in GPP to include patient perspectives, consensus between dermatologists and patients was achieved on overarching principles of treatment, and short- and long-term treatment goals for GPP. These findings provide valuable insights for developing guidelines that consider the perspectives of patients and physicians in the treatment of GPP.
泛发性脓疱型银屑病(GPP)是一种慢性、全身性、嗜中性粒细胞炎症性疾病。先前的德尔菲小组已就GPP达成了共识领域,不过未纳入患者的观点,且治疗目标的某些方面仍不明确。
通过有患者参与的德尔菲小组,确定GPP治疗的细化、具体治疗目标并达成共识。
基于系统的文献综述生成陈述,并由指导委员会进行修订。陈述分为总体原则以及短期和长期治疗目标。由30名皮肤科医生和3名患者代表组成的全球小组对每条陈述投票表示同意或不同意。共识定义为小组成员中≥80%的批准率。
在第一轮投票中达成了共识,26条陈述中有23条达成了≥90%的一致意见。总之,GPP需要患者和医生共同制定一个及时、量身定制的治疗方案,该方案采用多学科方法,应对疾病的复杂性、异质性和慢性。短期治疗目标应包括7天内清除脓疱并预防脓疱复发,减轻皮肤症状负担(瘙痒和皮肤疼痛数字评定量表上降低4分或更多),改善全身症状(如治疗开始后3天内退热和减轻疲劳),预防危及生命的并发症以及炎症生物标志物的逐步改善。对于合并银屑病性疾病的患者,治疗决策应优先考虑GPP。长期治疗目标应包括通过预防发作和控制发作间期症状来最小化疾病活动,持续控制疾病,管理合并症以及改善生活质量(QoL)。患者和医生在某些治疗目标重要性方面的认知存在细微差异(例如避免脱发和/或指甲脱落以改善生活质量),这反映了评估治疗目标的复杂性,并强调了以患者为中心方法的必要性。
在首个纳入患者观点的GPP全球德尔菲小组中,皮肤科医生和患者就治疗的总体原则以及GPP的短期和长期治疗目标达成了共识。这些发现为制定在GPP治疗中考虑患者和医生观点的指南提供了有价值的见解。