Abstract
Background
Generalized pustular psoriasis (GPP) is a rare, serious, chronic, neutrophilic skin disease, distinct from plaque psoriasis, characterized by recurrent flares of cutaneous erythema and widespread sterile pustules. Patient-centric data on the quality of life (QoL) impacts of GPP remain limited.
Objective
To evaluate the physical and emotional QoL impacts of GPP.
Methods
We used a web-based survey and 90-minute telephone interviews with open-ended questions to evaluate the experiences of adults with GPP across China, Japan, the UK, and the USA. Quantitative measures included the Dermatology Life Quality Index (DLQI), Psoriasis Symptom Scale (PSS), and Worst GPP Pain Numeric Rating Scale (NRS).
Results
21 participants completed the survey; 9 completed telephone interviews. The journey to diagnosis was complicated, with 48% of participants seeing ≥4 physicians before the diagnosis. Impacts of GPP on participants physical and mental QoL were substantial, such as the anxiety of not being able to plan life and work with confidence, both in general and during flares. The mean DLQI was 10.2, and the PSS and Worst GPP Pain NRS were highest in participants experiencing recent flares. Participants’ biggest worries included risk of flares, distress from symptoms, inadequate treatment, and passing on the disease to their children. Limitations included a small sample size and short recall timeframe.
Conclusion
Participants reported multiple impacts of GPP on their daily lives even in the absence of acute flares, highlighting the need for greater disease awareness and understanding among healthcare professionals.
Keywords: generalized pustular psoriasis, quality of life, patient experience, disease burden, physical impacts, emotional impacts
Plain Language Summary
Why was the study done?
Generalized pustular psoriasis (GPP) is a life-long condition that causes skin redness and inflammation, sterile blisters (referred to as pustules), as well as fever, tiredness, and pain. It is known that GPP symptoms have a negative effect on people’s quality of life. However, the lived experience of people with GPP has not been investigated in depth. Our study aimed to better understand how people feel about their diagnosis and GPP’s impact on their lives.
What did the researchers do?
A total of 21 people with GPP completed an internet-based survey. They provided direct feedback about their symptoms and quality of life using different scales. Nine people were also interviewed by telephone.
What did the researchers find?
Participants generally reported difficulties in receiving a diagnosis of GPP; almost half of them had seen at least 4 different doctors before a GPP diagnosis (mean time to diagnosis was 2.4 years). Using a standard scale called the Dermatology Life Quality Index, the average score (10.2) showed a moderate impact of GPP on their lives. Other scales and patient interviews revealed that GPP had a substantial negative impact on people’s quality of life (all participants reported emotional and physical function impacts, and 75% reported depression). It affected the physical and mental health aspects of their lives, not only when they were having a flare but also in general. Symptoms and pain associated with GPP were worst in those who recently had a GPP flare. Participants most often reported that their biggest concerns were the risk of having another GPP flare, the GPP symptoms, receiving inadequate treatment, and the risk of passing on the disease to their children.
What do the findings mean?
There is a high unmet need to better understand people’s lived experience of GPP, which is not always clearly demonstrated by the standard measurement scales. Our study allowed participants to directly report the effects of GPP on their lives. These results will help to increase the awareness and understanding of GPP among clinicians and other healthcare professionals.
Graphical Abstract.
Introduction
Generalized pustular psoriasis (GPP) is a rare, serious, chronic, neutrophilic, and systemic inflammatory disease, distinct from plaque psoriasis, characterized by macroscopically visible sterile pustules on an erythematous base that are not restricted to the acral region or within psoriatic plaques. 1 In addition to causing these widespread sterile, neutrophilic pustules, GPP can cause extracutaneous symptoms, including fever, chills and malaise, arthritis and arthralgia, and asthenia and fatigue. 2 Periods of GPP flaring can vary in frequency and severity,3,4 with the most severe flares potentially causing life-threatening complications, such as sepsis/septic shock and cardiovascular complications, if left untreated.2,5,6 The journey to GPP diagnosis can be convoluted, with multiple differential diagnoses and frequent comorbidities to navigate, and involvement of multiple specialists.3,6-9
Due to its chronic and unpredictable nature, GPP requires continuous systemic treatments to control symptoms and reduce the risk of flares.2,10,11 In a recent US survey, only about one-third of patients with GPP felt their condition was well controlled. 3 Moreover, claims data in Japan indicate that many patients need changes to treatment with relative frequency, with each treatment lasting a median of ∼3 months and a maximum of ∼50 months. 12 A systematic analysis of small studies using the Dermatology Life Quality Index (DLQI) indicated a moderate-to-very-large effect on health-related quality of life (QoL), 13 with GPP commonly affecting daily activities, even in periods between flares. 3
Despite insights from previous studies using patient-reported outcomes (PROs), the lived experiences of patients with GPP have not been explored in depth nor are they well documented in the literature. Through a quantitative survey and qualitative interviews, we aimed to understand the real-world impact of GPP on patients’ physical and emotional well-being, including their paths to diagnosis and treatment, particularly in relation to flares.
Methods
Design Overview
This was a cross-sectional, non-interventional, international study using a web-based survey and qualitative telephone interviews to gather the experiences of adults (aged ≥18 years) with GPP and palmoplantar pustulosis (PPP) across Canada, China, Japan, Spain, the UK, and the USA. This analysis focuses on GPP. Inclusion criteria for participants with GPP included a diagnosis of GPP ≥12 months before screening, ≥1 flare in the past 12 months, and a diagnosis of GPP per the European Rare and Severe Psoriasis Expert Network criteria. 14 Main exclusion criteria included conditions with overlapping symptoms, enrollment in a clinical trial for GPP or PPP, or any impairment that would interfere with participation. The study was performed in compliance with the protocol, the principles outlined in the Declaration of Helsinki, and Guidelines for Good Pharmacoepidemiology Practice.
Recruitment and Screening
Given the rarity of GPP, participants were recruited through patient advocacy groups (PAGs), recruitment vendors, and clinical sites. All recruitment procedures complied with country-specific regulations and were approved by an institutional review board/ethics committee. In the UK, clinical site staff prescreened participants seen routinely in clinics. Informed consent was obtained from all eligible, potential participants for participation in the web-based survey and optional telephone interviews.
Web-Based Survey
The web-based survey was developed through a literature review, study reports, advisory board transcripts, and PAG feedback; data collection was conducted between October 17, 2021, and June 30, 2023. PRO measures included the Psoriasis Symptom Scale (PSS; 4 items, recall period past 24 hours), DLQI (10 items, past week), Worst GPP Pain Numeric Rating Scale (NRS; single-item; past 24 hours), Patient Global Impression of Severity (PGI-S; single-item, 5-point, at this time), and 12-Item Short-Form Survey (SF-12®; 12 items, same day/past week). According to country-specific regulations, only participants in China were remunerated for their time.
Qualitative Interviews
Trained interviewers used a semi-structured qualitative interview guide to pose open-ended questions. Interviews lasted ∼90 minutes and were audio recorded with interviewees’ permission. Non-English interviews were conducted by fluent bilingual interviewers, and transcripts were translated into English.
Data Analysis and Handling of Missing Data
All quantitative analysis was performed using SAS version 9.4 (SAS Institute Inc., Cary, NC, USA), with unanswered questions coded as missing unless prespecified by PRO instrument developers. Qualitative analysis of transcripts was performed using ATLAS.ti version 22 (ATLAS.ti Scientific Software Development GmbH, Berlin, Germany).
Results
Study Participation and Sociodemographic Data
Of the 21 participants who completed the web survey, 9 also completed telephone interviews. The mean age (±standard deviation [SD]) was 40.7 (12.0) years; the mean time since diagnosis was 16.1 (15.0) years (Table 1). Supplemental Figures S1A and C, S2, and S3A, D, and F show results by country.
Table 1.
Sociodemographic Characteristics of Survey Participants
| Characteristic | |
|---|---|
| Age, years (mean [SD]) | 40.7 [12.0] |
| Time since diagnosis, years (mean [SD]) | 16.1 [15.0] |
| Time of first symptoms, years (mean [SD]) | 27.6 [16.7] |
| Time of diagnosis, years (mean [SD]) | 29.3 [17.1] |
| Female (n (%)) | 12 (57.1%) |
| Married (n (%)) | 11 (52.4%) |
| Living with partner, spouse, family, friend (n (%)) | 14 (66.7%) |
| Completed | |
| High school education (n (%)) | 21 (100%) |
| Higher education (n (%)) | 9 (42.9%) |
| Worked | |
| Full-time (n (%)) | 12 (57.1%) |
| Part-time (n (%)) | 3 (14.3%) |
Abbreviations: SD, standard deviation.
Journey to Diagnosis
The mean (±SD) time to diagnosis was 2.4 (5.5) years, with the majority (61.9%) of participants receiving ≥1 misdiagnosis, most commonly eczema (n = 3/13, 23.1%) and plaque psoriasis/psoriasis vulgaris or impetigo (n = 2/13, 15.4%; for each). Delays to diagnosis were concerning and frustrating to participants, with one stating that “…they still don’t know what it is, why are they still not able to make a diagnosis, and I was worrying how long this would go on.” Overall, 10 participants (47.6%) saw ≥4 physicians before receiving a diagnosis and 45.0% did not receive educational materials after their GPP diagnosis.
In total, 62.5% (n = 5/8) of interviewees expressed concerns or fears about their diagnosis (Figure 1A). One participant described their long-term fears as “…my skin’s condition was quite bad so I was quite terrified when I thought that this disease, as the doctor had said, would never go away, and that I would have to spend the rest of my life like this.”
Figure 1.
Quantitative and qualitative data on the impact of GPP.
(A) Interview data on participant concerns about the diagnosis or diagnostic procedure. (B) Main interviewee-reported impacts of GPP and flares on daily life. (C) Interview data on participant concerns about the diagnosis or diagnostic procedure. (D) Physical function impacts. aOnly frequencies >15% were included. Emotional function impacts reported by 12.5% of participants (n = 1/8) were decreased self-confidence/self-esteem, discomfort, distress, embarrassment, fear, loneliness, panic, paranoia, shock, tension, traumatic experience, uneasiness, and worry. Abbreviation: GPP, generalized pustular psoriasis
Symptoms
In the survey, 9 participants (42.9%) indicated they experienced GPP symptoms every day, with >50% experiencing pustules, itching, not feeling well/discomfort, inflammation, fatigue, mental discomfort/irritability, redness, dryness/dry skin, peeling skin, or fever/shivering in the previous 12 months. Participants most commonly listed pustules, itching, and not feeling well/discomfort as their top 3 most bothersome symptoms in the past 12 months (Supplemental Figure S4).
In the 24 hours before the survey, the mean PSS total score (SD) was 5.2 (4.7; range: 0 [no symptoms] to 16 [very severe symptoms]), indicating moderate symptoms; the mean Worst GPP Pain NRS was 3.4 (3.8; range: 0 [no pain] to 10 [worst possible pain]), indicating mild pain intensity (Supplemental Figure S1A and C). However, 11 participants (57.2%) had moderate or higher overall severity on the PGI-S (Supplemental Figure S2).
Flares
All participants experienced ≥1 flare within the past 12 months, with a mean (±SD) of 2.1 (1.8) flares. Four participants were experiencing a flare at the time of the survey, with 4 participants experiencing a flare 1-3 months ago and 10 participants >3 months ago (Supplemental Figure S1B). Participants often stated their flares varied in their duration: “It could be anywhere from four to 6 weeks to months.” Across participants who provided details, flares could range from 4-7 days to >6 months. Commonly reported triggers for flares included stress (n = 15, 71.4%), sudden withdrawal of medications (n = 8, 38.1%), and sunlight/ultraviolet light or an infection (n = 5, 23.8%; for each).
Of surveyed participants, 56.3% reported that their most recent flare had a moderate impact on their daily activities, with 31.3% reporting a severe impact. Pustules were the most commonly reported as one of the most bothersome symptoms during their most recent flare (47.6%).
Participants who reported the highest mean (±SD) PSS score were currently experiencing a flare (n = 4, PSS 6.0 [3.6]) or had experienced one in the past 1-3 months (n = 4, PSS 6.5 [6.5]), with lower values for participants who had experienced a flare >3 months ago (n = 10, PSS 4.9 [4.8]; Supplemental Figure S1B). Similarly, the mean Worst GPP Pain NRS (moderate score: 4-6; mild: 1-3) was also highest in those currently experiencing a flare (n = 15, mean 5.0 [3.6] or those who had a flare in the past 1-3 months (n = 6, 5.7 [4.5]), with lower scores in those with less recent flares (n = 17, 2.7 [3.4]; Supplemental Figure S1D).
Qualitative Data on QoL
All 9 interviewees stated that GPP impacted their emotional and physical function, either generally or due to flares (Figure 1B). Greatest impacts were noted on sleep (88.9%), dressing/clothes choices, finances, social life, and work (77.8%; for each) (Figure 2).
Figure 2.
Interviewee-cited concepts regarding the impact of GPP on daily activities. Abbreviation: GPP, Generalized Pustular Psoriasis
Function Impacts
All interviewees reported emotional impacts of GPP generally or due to flares, with 8 (88.9%) reporting emotional impacts outside of flares — most commonly depression (75.0%) and stress (37.5%) (Figure 1C). For example, a participant reported “I could feel… extreme depression” and that “my appearance was very stressful for me.” Other participants reported anxiety “When the fever occurred, I know I was about to have an occurrence, I would be very anxious.”
Similarly, all interviewees reported physical impacts of GPP generally or due to flares, with 7 (77.8%) experiencing at least 1 impact outside of flares. The most frequently reported physical impacts were difficulty/inability to walk (85.7%), followed by a difficulty/inability to use their hands (42.9%; Figure 1D), such as “opening a soda can or twisting a bottle. I have very bad difficulty with it.”
Treatment Impacts
All interviewees were receiving treatment for GPP. In total, 77.8% reported side effects of their current treatment, 55.6% expressed worries over lack of efficacy or treatment consequences, and 33.3% expressed worries regarding the risk of potential treatment consequences or side effects (Figure 3A).
Figure 3.
Participant-reported worries over GPP during telephone interviews. (A) Reported concerns over treatments. (B) Quotes indicating the biggest worries for participants with GPP. Abbreviation: GPP, Generalized Pustular Psoriasis
Over half of participants surveyed had previously used or were currently using topical corticosteroids (76.2%), biologics (61.9%), and methotrexate or emollients (52.4%; for each). All participants were at least somewhat satisfied with biologics, whereas satisfaction for topical corticosteroids, emollients, and oral or injectable corticosteroids was predominately negative for symptom control and mixed for symptom prevention. The length of time that participants had been on their current and previous treatments varied greatly, ranging from <1 month to >5 years.
Frequency of visits to healthcare providers varied from monthly through to > every 6 months, with 3 participants having no routine follow-up. Overall, most participants (83%) reported spending 30 minutes to 2 hours traveling to their appointments and 57% reported spending up to 60 minutes a day on applying medications.
Participants’ Biggest Worries Regarding GPP
Five interviewees described their biggest worries regarding GPP: risk of a future flare and organ failure, distress from skin symptoms, pain associated with skin symptoms, fear of passing GPP to their children, and feeling that there is no ideal treatment for them (Figure 3B). These feelings were often combined with potential social impacts (eg, embarrassment or stigma; Figure 3B, quote 2) and conflicting emotions (eg, Figure 3B, quote 5).
Quantitative Findings on QoL Impact
DLQI
The mean DLQI total score (±SD) was 10.2 (10.4; range: 0-30), indicating a moderate effect of GPP on participants’ QoL (Figure 4A). Based on total score categories, the scale of impact varied between participants, with the highest impacts in those experiencing flares in the last 3 months (Supplemental Figure S3B). The mean scores (±SD) for the subdomains’ symptoms and feelings, daily activities, and leisure were >1 point higher for participants with a current flare vs those with a flare >3 months ago (Figure 4A; Supplemental Figure S3C).
Figure 4.
PRO scores for the 7 days prior to the survey.
(A) Overall DLQI and domain scores. (B) Impact of skin condition on different aspects of daily life. (C) SF-12 mean physical and mental health scores. (D) SF-12 items regarding ways in which skin condition was reported to limit impact on daily activities. Abbreviations: DLQI, Dermatology Life Quality Index; GPP, generalized pustular psoriasis; PRO, patient-reported outcome; SD, standard deviation; SF-12, 12-Item Short-Form Survey.
Many participants reported being embarrassed or self-conscious because of the condition of their skin “very much” or “a lot of the time” in the week before the survey, as well as interfering with daily activities such as shopping and working/studying (Figure 4B).
SF-12
The mean (±SD) SF-12 physical and mental component scores were 41.1 (11.5) and 41.2 (15.8), respectively (Figure 4C). Participant responses varied, with a notable proportion indicating poor health, physical limitations, or emotional impacts of their skin condition (Figure 4D).
Discussion
This study results reveal that patients’ experiences on GPP varied, reflecting the condition’s heterogeneous clinical course. Most participants had a moderate severity of disease, with chronic symptoms and a moderate-to-severe impact on their QoL and daily activities. Even mild symptoms (eg, pain) had substantial impacts on QoL, with many interviewees reporting symptoms that fluctuate between mild and severe. The biggest worries participants had about GPP included the risk of a future flare and organ failure, distress from skin symptoms, pain, feeling that the treatment does not meet their needs, and passing the disease to their children.
For many participants, the journey to diagnosis was long, involving misdiagnoses and visits to multiple physicians. Only half of the participants received educational materials after their diagnosis, highlighting an unmet need to ensure that participants are informed and provided with the necessary support and care networks to manage their condition.
Overall, the impacts of GPP on participants’ physical and mental QoL were substantial, both during and outside of flares, with symptoms having significant impacts on their daily life. Avoidance was common, with 1 participant stating “I generally try not to go to places where there are many people and where my skin needs to be exposed, such as swimming pools,” while another stated “I probably avoid social situations…I get more embarrassed by it.” In line with this, both depression and stress were common among participants, potentially creating a vicious cycle because stress was also identified as one of the leading triggers of flares. Nearly all participants reported that flares had a moderate-to-severe impact on their daily activities. As most participants experienced flares for 1-3 months, with a mean of 2 flares per year, many participants therefore experienced flares for at least half the year — a significant disease burden. The unpredictability of flares has been noted to be one of the major factors contributing to the mental health burden of patients with GPP15,16; therefore, any treatment that can reduce the risk of flares can potentially improve patients’ emotional and mental QoL. A recent global consensus study concluded that long-term GPP treatment goals should include prevention of flares 17 ; participants’ concerns over chronic symptoms, flares, and existing treatments in our study highlight the need for effective, continuous treatment strategies that provide sustained symptom relief and improve their QoL.
While few studies have qualitatively evaluated patients who experienced GPP, some have previously quantitatively evaluated the impacts on QoL. In a survey of 83 patients in Japan, patients with GPP had lower SF-36 scores for physical functioning, physical roles, body pain, general health, and emotional roles than the Japanese national standard values. 18 While we used a more concise SF-12 PRO, these findings align with our results, where many patients with GPP report limitations to both physical and emotional function. Similarly, the DLQI total and component scores found in our study correspond to those found in other studies, indicating that our study population was reasonably representative of patients with GPP.13,19
Moreover, a survey of 66 patients with GPP living in the USA found themes broadly similar to those of the current study with regard to flare triggers, delays in diagnosis, emotional impacts, and treatment effectiveness. The study concluded that GPP impacted patient lives even in the absence of flares and that many of the patients felt that their physician was unaware of emotional, psychological, and physical impacts of the disease. 3
Many physicians are unaware of the true burden of diseases such as GPP to patients. While PROs allow us to quantify patient experiences, they do not truly capture the whole patient experience. Qualitative research, such as that conducted in this study, can add a more nuanced element to aid dermatologists in a more comprehensive understanding of the patient experience, particularly their specific concerns and worries. For example, we found that even mild GPP symptoms can have a substantial impact on patients’ QoL, symptoms that may be overlooked with quantitative PRO measures. One of the main strengths of this study was the ability to combine both quantitative and qualitative evidence to give an overall picture of the impacts of GPP from a patient perspective. Despite this, there remain a few limitations in our study, including the small sample size, which precluded statistical analysis. As GPP is a rare disease, recruitment was unfortunately challenging, with sample sizes for the survey and interviews of 60% and 64% of planned numbers, respectively. Additionally, for some participants, there was a time lag of 1-4 months between the survey and interview, which may have resulted in some discrepancies in responses. Further limitations included this being a cross-sectional web survey performed at a single point in time; therefore, not all participants had symptoms or flares at the time the survey was conducted, and completion was biased towards people who were comfortable using an online platform. As the PROs used in the survey have a short recall timeframe of a day to a week, responses may have been dependent on symptoms at the time of the survey and may underestimate the impact of GPP during flare periods. Finally, discussion on personal, mental, and physical health, as well as the shame associated with the disease, potentially reduced the number of patients willing to participate in surveys, particularly from certain cultures, and/or limited the number of questions they felt comfortable responding to.
Given the concerns shared by the participants of this study, there is a need to identify and develop educational materials for patients. Despite available therapies for GPP, patients remain concerned about potential side effects and/or lack of efficacy. Future GPP clinical trials should routinely include PROs to assess whether treatments can improve QoL and reduce the burden of this chronic, systemic disease on the lives of patients. Additionally, further research on specific impacts of flares on patient experiences is warranted, as there are currently little available data focusing specifically on this aspect of the disease.
Conclusion
In this cross-sectional, international, non-interventional study, participants reported feelings of fear and related negative emotions throughout the disease course of GPP, with nearly all participants reporting multiple impacts on their daily lives. Although larger effects were seen in participants who had flares more recently, many participants reported chronic symptoms and QoL-related burden in the past year. Participants’ concerns over chronic symptoms, flares, and existing treatments highlight the need for effective, continuous treatment strategies that provide sustained symptom relief and improve their QoL. Overall, this study provides a better understanding of the symptom burden and emotional impact of GPP, especially important due to the ongoing need for greater disease awareness and understanding among healthcare professionals.
Supplemental Material
Supplemental Material for Patient Experiences on the Diagnosis, Management, and Burden of Generalized Pustular Psoriasis: An International Web Survey and Qualitative Interview Study by Mark Lebwohl, Hideki Fujita, Kilian Eyerich, Andrew E. Pink, Min Zheng, Iris Chen-Yin Lai, David Trigos, Anne M. Skalicky, Julia R. Correll, Ana C. Hernandez Daly, and Tadashi Terui in Journal of Psoriasis and Psoriatic Arthritis®
Acknowledgments
The authors would like to give a heartfelt thanks to all patients with GPP who took part in this research, as well as the PAGs, including IFPA, CORD, NPF, and EUROPSO, who played a pivotal role in participants recruitment. The authors meet criteria for authorship as recommended by the International Committee of Medical Journal Editors (ICMJE) and did not receive payment related to the development of this manuscript. Authors David Trigos and Iris Chen-Yin Lai are patient authors who advised on the study design. Boehringer Ingelheim was given the opportunity to review the manuscript for medical and scientific accuracy, as well as intellectual property considerations. Maria Coimbra-Dores, PhD, provided writing, editorial, and formatting support, which was contracted and funded by Boehringer Ingelheim.
Ethical Approval: Reviewed and approved by Ethical and Independent (E & I) Review Services Institutional Review Board (IRB, 21111-01; now Salus IRB), Advarra IRB (Pro00058362), Shiba Palace Clinic Ethics Review Committee (147960_r n-31116), Shanghai Ethics Committee for Clinical Research (SECCR2021-181-01), CEIm del Parc Tauli (2021/5114), and HRA and Health and Care Research Wales (HCRW; 22/LO/0792). The study was carried out in compliance with the protocol, the principles laid down in the Declaration of Helsinki, and Guidelines for Good Pharmacoepidemiology Practice. All recruitment procedures were compliant with country-specific regulations and were approved by the institutional review board/ethics committee.
Patient Consent: Informed consent was obtained by writing from all eligible potential participants for participation in the web survey and optional telephone interviews.
Author contributions: Mark Lebwohl, Hideki Fujita, Kilian Eyerich, Andrew E. Pink, Min Zheng, Iris Chen-Yin Lai, David Trigos, Ana C. Hernandez Daly, and Tadashi Terui: conceptualization. Anne M. Skalicky and Julia R. Correll: data curation, resources, software. Anne M. Skalicky, Julia R. Correll, and Ana C. Hernandez Daly: formal analyses, project administration. Andrew E. Pink, Anne M. Skalicky, and Julia R. Correll: investigation. Ana C. Hernandez Daly: supervision. Mark Lebwohl, Hideki Fujita, Kilian Eyerich, Andrew E. Pink, Min Zheng, Iris Chen-Yin Lai, David Trigos, Anne M. Skalicky, Julia R. Correll, Ana C. Hernandez Daly, and Tadashi Terui: methodology, validation, visualization, writing (original draft preparation), writing (reviewing and editing). All authors interpreted the data, provided critical feedback on the manuscript, approved the final manuscript for submission, and were accountable for the accuracy and integrity of the manuscript.
Funding: The authors disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: this study was supported and funded by Boehringer Ingelheim International. Boehringer Ingelheim International designed the trial, analyzed the data, and paid for professional writing assistance.
The authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Mark Lebwohl is an employee at Mount Sinai; receives research funds from AbbVie, Arcutis, Avotres, Boehringer Ingelheim, Cara Therapeutics, Clexio, Dermavant Sciences, Eli Lilly, Incyte, Inozyme, Janssen, Pfizer, Sanofi-Regeneron Pharmaceuticals Inc., and UCB; is a consultant for Aikium, Almirall, AltruBio, Amgen, Apogee, Arcutis, AstraZeneca, Atomwise, Avotres Therapeutics, Boehringer Ingelheim, Bristol Myers Squibb, Castle Biosciences, Celltrion, CorEvitas, Dermavant Sciences, Dermsquared, Evommune, Facilitation of International Dermatology Education, Forte Biosciences, Galderma, Genentech, GoodRx-Mayne, Incyte, LEO Pharma, Meiji Seika Pharma, Mindera, Mirum Pharmaceuticals, Oruka, Pfizer, Revolo, Sanofi-Regeneron Pharmaceuticals Inc., Seanergy, Strata, Sunpharma, Takeda, Trevi, and Verrica. Hideki Fujita received research grants or honoraria for speaker and/or consultancy from AbbVie, Amgen, Boehringer Ingelheim, Bristol Myers Squibb, Daiichi Sankyo, Eisai, Eli Lilly, Janssen, JMEC, KAKEN Pharmaceutical, Kyowa Kirin, LEO Pharma, Maruho, Mitsubishi Tanabe, Novartis, Otsuka, Sanofi, Sato, Sun Pharma, Taiho, Takeda, Torii, UCB, and Ushio. Kilian Eyerich has received speakers’ fees and/or advisory board member for AbbVie, Almirall, Apogee, BMS, Boehringer Ingelheim, Janssen, LEO, Lilly, MoonLake, Novartis, Pfizer, Sanofi, Sitryx, and UCB. Andrew E. Pink has acted as an advisor, speaker, and investigator and has received educational support from or received research funding from AbbVie, Almirall, Amgen, BMS, Boehringer Ingelheim, Celgene, Janssen, La Roche-Posay, LEO Pharma A/S, Lilly, Novartis, Pfizer, Sanofi, and UCB. Min Zheng has participated in advisory boards and/or as an investigator and/or speaker and received grants and/or honoraria from AbbVie, Boehringer Ingelheim, Janssen-Cilag, LEO Pharma China, Novartis, Pfizer, and Xian Janssen. Iris Chen-Yin Lai is a co-investigator for several vaccine trials carried out at National Taiwan University Children’s Hospital. David Trigos is responsible for EUPATI Spain, a non-profit organization that receives funding from AbbVie, Alexion, Amgen, Ascendis Pharma, Astellas, Boehringer Ingelheim, Bristol Myers Squibb, Johnson & Johnson, Novartis, Novo Nordisk, Pfizer, and Takeda for the fulfilment of the goals of the organization. Anne M. Skalicky and Julia R. Correll are employees of Evidera, which received payment from the study sponsor, Boehringer Ingelheim, for work on this study. Ana C. Hernandez Daly is an employee of Boehringer Ingelheim. Tadashi Terui received research funds from Maruho Co., Ltd., and honoraria for a speaker, consultancy, and advisory board member from AbbVie, Amgen, Boehringer Ingelheim, Bristol Myers Squibb, Eli Lilly, Janssen Pharmaceuticals, Kyowa Kirin Pharma, Mitsubishi Tanabe Pharma, Novartis, Sanofi, Taiho Pharmaceutical, and UCB Japan.
Supplemental Material: Supplemental material for this article is available online.
ORCID iD
Mark Lebwohl https://orcid.org/0000-0002-4705-5303
Data Availability Statement
To ensure independent interpretation of clinical study results and enable authors to fulfill their role and obligations under the ICMJE criteria, Boehringer Ingelheim grants all external authors access to clinical study data pertinent to the development of the publication. In adherence with the Boehringer Ingelheim Policy on Transparency and Publication of Clinical Study Data, scientific and medical researchers can request access to clinical study data when it becomes available on Vivli - Center for Global Clinical Research Data, and earliest after publication of the primary manuscript in a peer-reviewed journal, regulatory activities are complete, and other criteria are met. Please visit Medical & Clinical Trials | Clinical Research | MyStudyWindow for further information.*
References
- 1.Choon SE, van de Kerkhof P, Gudjonsson JE, et al. International Consensus Definition and Diagnostic Criteria for generalized pustular psoriasis from the International Psoriasis Council. JAMA Dermatol. 2024;160(7):758-768. [DOI] [PubMed] [Google Scholar]
- 2.Puig L, Choon SE, Gottlieb AB, et al. Generalized pustular psoriasis: a global Delphi consensus on clinical course, diagnosis, treatment goals and disease management. J Eur Acad Dermatol Venereol. 2023;37(4):737-752. [DOI] [PubMed] [Google Scholar]
- 3.Reisner DV, Johnsson FD, Kotowsky N, Brunette S, Valdecantos W, Eyerich K. 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. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Bhutani T, Farberg AS. Clinical and disease burden of patients with generalized pustular psoriasis: a review of real-world evidence. Dermatol Ther. 2024;14(2):341-360. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5.Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expet Rev Clin Immunol. 2019;15(9):907-919. [DOI] [PubMed] [Google Scholar]
- 6.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(2):256-273. [DOI] [PubMed] [Google Scholar]
- 7.Fujita H, Gooderham M, Romiti R. Diagnosis of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):31-38. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Hayama K, Tian Y, Iwasaki R, Fujita H. Patient journey of generalized pustular psoriasis: a real-world study using data-mining methods and Japanese claims data. Acta Derm Venereol. 2024;104:adv11946. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Reynolds KA, Pithadia DJ, Lee EB, Clarey D, Liao W, Wu JJ. Generalized pustular psoriasis: a review of the pathophysiology, clinical manifestations, diagnosis, and treatment. Cutis. 2022;110(Suppl 2):19-25. [DOI] [PubMed] [Google Scholar]
- 10.Kodali N, Blanchard I, Kunamneni S, Lebwohl MG. Current management of generalized pustular psoriasis. Exp Dermatol. 2023;32(8):1204-1218. [DOI] [PubMed] [Google Scholar]
- 11.Armstrong AW, Elston CA, Elewski BE, et al. Generalized pustular psoriasis: a consensus statement from the National Psoriasis Foundation. J Am Acad Dermatol. 2024;90(4):727-730. [DOI] [PubMed] [Google Scholar]
- 12.Tada Y, Guan J, Iwasaki R, Morita A. Treatment patterns and drug survival for generalized pustular psoriasis: a patient journey study using a Japanese claims database. J Dermatol. 2024;51(3):391-402. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 13.Choon SE, De La Cruz C, Wolf P, et al. Health-related quality of life in patients with generalized pustular psoriasis: a systematic literature review. J Eur Acad Dermatol Venereol. 2024;38(2):265-280. [DOI] [PubMed] [Google Scholar]
- 14.Navarini AA, Burden AD, Capon F, et al. European consensus statement on phenotypes of pustular psoriasis. J Eur Acad Dermatol Venereol. 2017;31(11):1792-1799. [DOI] [PubMed] [Google Scholar]
- 15.Mockenhaupt M, Strober B, Lebwohl M, et al. The psychological burden of generalised pustular psoriasis: claims-based evidence from the USA and Germany (abstract 3746). In: Abstract Presented at: European Academy of Dermatology and Venerology Congress, Amsterdam, The Netherlands, September 25-28, 2024. Accessed July 29, 2025.https://mr-congress.com/de/kongress-liste/eadv-european-academy-dermatology-venerology-3/ [Google Scholar]
- 16.Kole L, Tada Y, Gerdes S, et al. Living with generalised pustular psoriasis: understanding chronic symptoms, impact on quality of life, and support and educational needs (abstract 3784). In: Abstract Presented at: European Academy of Dermatology and Venerology Congress, Amsterdam, The Netherlands, September 25-28, 2024. Accessed July 29, 2025.https://mr-congress.com/de/kongress-liste/eadv-european-academy-dermatology-venerology-3/ [Google Scholar]
- 17.Barker JN, Casanova E, Choon SE, et al. Global Delphi consensus on treatment goals for generalized pustular psoriasis. Br J Dermatol. 2025;192(4):706-716. [DOI] [PubMed] [Google Scholar]
- 18.Hayama K, Fujita H, Iwatsuki K, Terui T. Improved quality of life of patients with generalized pustular psoriasis in Japan: a cross-sectional survey. J Dermatol. 2021;48(2):203-206. [DOI] [PMC free article] [PubMed] [Google Scholar]
- 19.Norlin JM, Löfvendahl S, Schmitt-Egenolf M. Health-related quality of life in patients with generalized pustular psoriasis – a Swedish register study. Ann Med. 2024;56(1):2341252. [DOI] [PMC free article] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Supplemental Material for Patient Experiences on the Diagnosis, Management, and Burden of Generalized Pustular Psoriasis: An International Web Survey and Qualitative Interview Study by Mark Lebwohl, Hideki Fujita, Kilian Eyerich, Andrew E. Pink, Min Zheng, Iris Chen-Yin Lai, David Trigos, Anne M. Skalicky, Julia R. Correll, Ana C. Hernandez Daly, and Tadashi Terui in Journal of Psoriasis and Psoriatic Arthritis®
Data Availability Statement
To ensure independent interpretation of clinical study results and enable authors to fulfill their role and obligations under the ICMJE criteria, Boehringer Ingelheim grants all external authors access to clinical study data pertinent to the development of the publication. In adherence with the Boehringer Ingelheim Policy on Transparency and Publication of Clinical Study Data, scientific and medical researchers can request access to clinical study data when it becomes available on Vivli - Center for Global Clinical Research Data, and earliest after publication of the primary manuscript in a peer-reviewed journal, regulatory activities are complete, and other criteria are met. Please visit Medical & Clinical Trials | Clinical Research | MyStudyWindow for further information.*