背景:全身性脓疱型银屑病(3GPP)是一种罕见且严重的炎症性皮肤病,其特征是复发性或间歇性耀斑。西班牙的流行病学和疾病管理数据有限。我们的目标是估计阵的流行病学,探索其管理,并就西班牙当前面临的挑战达成共识。
方法:向西班牙皮肤病学会银屑病工作组的皮肤科医生提交了一份电子调查。该小组在3GPP的管理方面经验丰富。它包括Delphi共识,以确定当前的挑战。
结果:共有33位皮肤科医生对调查做出了回应。5年患病率和发病率分别为每百万居民13.05和7.01例,分别,估计。根据受访者的说法,最常见的GMP症状是脓疱,红斑,和脱皮,而45%的患者出现>1次年度耀斑。共有45%的受访者表示,耀斑通常需要1至2周的停留时间。在存在耀斑的情况下,67%的受访者经常或总是开非生物系统治疗作为一线治疗[环孢素(55%);口服类维生素A(30%)],和45%的生物治疗[抗TNFα(52%);抗IL-17(39%)]。皮肤科医生一致认为,主要挑战是定义和建立具体的治疗目标来治疗疾病,包括患者对疾病管理的看法。
结论:我们的研究描述了西班牙的3GPP管理现状,增加目前对这种疾病的认识,并强调目前面临的挑战。
BACKGROUND: Generalized pustular psoriasis (GPP) is a rare and severe inflammatory skin disease characterised by recurrent or intermittent flares. Epidemiological and disease management data in Spain are limited. Our goal was to estimate the epidemiology of GPP, explore its management, and reach consensus on the current challenges faced in Spain.
METHODS: An electronic survey was submitted to dermatologists from the Spanish Academy of Dermatology and Venereology Psoriasis Working Group. This group is experienced in the management of GPP. It included a Delphi consensus to establish the current challenges.
RESULTS: A total of 33 dermatologists responded to the survey. A 5-year prevalence and incidence of 13.05 and 7.01 cases per million inhabitants, respectively, were estimated. According to respondents, the most common GPP symptoms are pustules, erythema, and desquamation, while 45% of patients present > 1 annual flares. A total of 45% of respondents indicated that flares often require a length of stay between 1 and 2 weeks. In the presence of a flare, 67% of respondents often or always prescribe a non-biological systemic treatment as the first-line therapy [cyclosporine (55%); oral retinoid (30%)], and 45% a biological treatment [anti-TNFα (52%); anti-IL-17 (39%)]. The dermatologists agreed that the main challenges are to define and establish specific therapeutic goals to treat the disease including the patients\' perspective on the management of the disease.
CONCLUSIONS: Our study describes the current situation on the management of GPP in Spain, increasing the present knowledge on the disease, and highlighting the current challenges faced at the moment.