Current status of generalized pustular psoriasis: Findings from a multicenter hospital-based survey of 127 Chinese patients

Abstract

Background:

Generalized pustular psoriasis (GPP), a rare and recurrent autoinflammatory disease, imposes a substantial burden on patients and society. Awareness of GPP in China remains limited.

Methods:

This cross-sectional survey, conducted between September 2021 and May 2023 across 14 hospitals in China, included GPP patients of all ages and disease phases. Data collected encompassed demographics, clinical characteristics, economic impact, disease severity, quality of life, and treatment-related complications. Risk factors for GPP recurrence were analyzed.

Results:

Among 127 patients (female/male ratio = 1.35:1), the mean age of disease onset was 25 years (1st quartile [Q1]–3rd quartile [Q3]: 11–44 years); 29.2% had experienced GPP for more than 10 years. Recurrence occurred in 75.6% of patients, and nearly half reported no identifiable triggers. Younger age at disease onset (P = 0.021) and transitioning to plaque psoriasis (P = 0.022) were associated with higher recurrence rates. The median diagnostic delay was 8 months (Q1–Q3: 2–41 months), and 32.3% of patients reported misdiagnoses. Comorbidities were present in 53.5% of patients, whereas 51.1% experienced systemic complications during treatment. Depression and anxiety affected 84.5% and 95.6% of patients, respectively. During GPP flares, the median Dermatology Life Quality Index score was 19.0 (Q1–Q3: 13.0–23.5). This score showed significant differences between patients with and without systemic symptoms; it demonstrated correlations with both depression and anxiety scores. Treatment costs caused financial hardship in 55.9% of patients, underscoring the burden associated with GPP.

Conclusions:

The substantial disease and economic burdens among Chinese GPP patients warrant increased attention. Patients with early onset disease and those transitioning to plaque psoriasis require targeted interventions to mitigate the high recurrence risk.

Introduction

Generalized pustular psoriasis (GPP) is a rare, chronic inflammatory systemic disease with potentially life-threatening complications. It is genetically and phenotypically distinct from plaque psoriasis.^[1] The prevalence of GPP varies across countries. In Korea, the estimated prevalence is 88–124 cases per million persons per year, considerably higher than the 1.76 cases per million persons reported in a French study.^{[2, 3]} The mortality rate associated with GPP ranges from 0 to 3.3 deaths per 100 patient-years.^[4] The diverse symptoms, comorbidities, and complications of GPP contribute to its substantial disease burden and management challenges.^[5,6,7,8] Questionnaire surveys on GPP conducted in four other countries have explored demographics, disease characteristics, symptoms, quality of life, treatment approaches, epidemiology, and unmet medical needs.^{[3,9,10,11,12,13,14]} However, no similar survey has been performed in China; thus, there is a gap in understanding the burden of GPP and the experiences of Chinese patients. The present study aimed to address this gap by summarizing relevant information and providing a clearer understanding of GPP in Chinese population.

Methods

Ethical approval

The study was approved by the institutional review board at each participating hospital. Written informed consent was obtained from all participants (or guardians for patients aged <18 years).

Survey participants

This cross-sectional survey was conducted between September 2021 and May 2023 in collaboration with 14 tertiary hospitals across China, ensuring comprehensive provincial-level healthcare coverage. These hospitals are equipped to diagnose and treat GPP, serving patients nationwide. Before the study was initiated, all participating hospitals contributed to its design to ensure familiarity with the study protocol. Researchers from each hospital underwent detailed training on data collection using the survey questionnaires to ensure high-quality data acquisition. Additionally, researchers regularly organized and reviewed the collected data to maintain accuracy and completeness. The study included GPP patients of all ages and disease stages, diagnosed by trained dermatologists at each participating medical center [Figure 1]. Diagnoses were made in accordance with the European Rare and Severe Psoriasis Expert Network criteria: Primary sterile pustules on non-acral skin, with or without systemic inflammation, psoriasis vulgaris, and either relapse (>1 episode) or persistence (>3 months).^[15] Exclusion criteria were as follows: (a) pustules confined to psoriatic plaques and (b) conditions mimicking GPP, such as acute generalized exanthematous pustulosis and subcorneal pustular dermatosis, which were differentiated using medical history and pathological examination.

Figure 1.

Flowchart of the questionnaire survey for current status of generalized pustular psoriasis in 127 Chinese patients. Both patients and physicians provided responses based on the disease phase. DLQI: Dermatology Life Quality Index; GAD-7: Generalized Anxiety Disorder Screener; GPP: Generalized pustular psoriasis; GPPASI: Generalized Pustular Psoriasis Area and Severity Index; PHQ-9: Patient Health Questionnaire-9.

Several strategies were implemented to mitigate bias. First, the inclusion of high-level healthcare facilities that attract patients nationwide ensured a representative sample. Second, patients completed the questionnaire in person with the assistance of physicians, minimizing non-response bias. Third, physicians referred to medical records during questionnaire reviews to reduce recall bias.

Survey questionnaire design

The survey, described in detail in Supplementary Materials 1 and 2, http://links.lww.com/CM9/C320, consisted of two parts. The first part, applicable to all patients, included nine sections and 37 questions addressing demographics, clinical characteristics, disease history, and costs. Cost data were divided into total treatment expenses, drug expenses, and a self-evaluation of affordability. The second part was specific to patients experiencing new pustule onset (acute phase) and required input from both patients and physicians. Patient sections contained 38 questions addressing complications, the Dermatology Life Quality Index (DLQI), and mood. Physician sections included four parts with nine questions, focusing on disease type, severity (assessed using the Generalized Pustular Psoriasis Area and Severity Index [GPPASI] and the Japanese Dermatological Association Severity Index [JDA-SI]), treatments, and adverse events [Figure 1].

Data analysis

Survey results were subjected to thorough compilation and analysis. Physicians ensured uniformity by verifying questionnaire responses for accuracy and authenticity. Associations between clinical characteristics and GPP recurrence were assessed via the Mann–Whitney U test and Kruskal–Wallis tests. The Mann–Whitney U test was used for two-group comparisons of ordinal categorical variables; the Kruskal–Wallis test was utilized for comparisons involving three or more groups. When necessary, Dunn–Bonferroni post hoc testing was performed. Spearman ρ analysis was applied for correlation analysis. Data visualization was conducted using Microsoft Excel (Microsoft Corporation, Seattle, WA, USA), and statistical analyses were carried out with SPSS version 26.0 (IBM Corp, Armonk, USA). Statistical significance was defined as a P-value below 0.05. It is important to note that the findings presented in this report are exclusively based on survey questionnaire responses.

Results

Demographics and baseline information

The survey included 127 GPP patients (female/male ratio = 1.35:1); 45 were experiencing acute flares. The median age was 32 years (1st quartile [Q1]–3rd quartile [Q3]: 23–51 years), and more than 60.0% were younger than 40 years. Disease onset primarily occurred between 0–10 years of age and 21–30 years of age [Supplementary Figure 1, http://links.lww.com/CM9/C319], with a mean onset age of 25 years (Q1–Q3: 11–44 years). Respondents were predominantly from northern (33, 26.0%), southwestern (24, 18.9%), northeastern (21, 16.5%), and southern (18, 14.2%) regions of China. Analysis of education levels indicated that 48.0% (61/127) of respondents had a high school diploma or lower. Additionally, 74.0% (94/127) were non-smokers, and 86.6% (110/127) were non-drinkers [Table 1]. Family histories included hypertension (68, 53.5%), diabetes mellitus (48, 37.8%), psoriasis (24, 18.9%), and cancer (9, 7.1%). A history of psoriasis was reported by 39.4% (50/127) of respondents; psoriasis vulgaris (43, 33.9%), erythrodermic psoriasis (10, 7.9%), psoriatic arthritis (1, 0.8%), and palmoplantar pustulosis or acrodermatitis continua of Hallopeau (3, 2.4%) were the most common forms [Supplementary Table 1, http://links.lww.com/CM9/C319].

Table 1.

Demographics and baseline characteristics of 127 GPP patients.

Items	Results (N = 127)
Sex
Women	73 (57.5)
Men	54 (42.5)
Age at presentation (years)	32 (23–51)
Age at onset (years)	25 (11–44)
Disease course
≤5 years	69 (54.3)
5–10 years	20 (15.8)
≥10 years	38 (29.9)
Regions
Northern China	33 (26.0)
Southwestern China	24 (18.9)
Northeastern China	21 (16.5)
Southern China	18 (14.2)
Eastern China	14 (11.0)
Western China	11 (8.7)
Central China	6 (4.7)
Education
High school and below	61 (48.0)
Technical secondary school	16 (12.6)
Junior college	24 (18.9)
Bachelor	22 (17.3)
Master and above	4 (3.2)
Smoking history
Never	94 (74.0)
Former	15 (11.8)
Current	18 (14.2)
Alcohol use
No	110 (86.6)
Yes	17 (13.4)
Recurrence*
None	31 (24.4)
Mild	46 (36.2)
Moderate	22 (17.4)
Severe	28 (22.0)

Data are presented as n (%) or median (Q1–Q3). ^*The patterns of recurrence is defined as follows: none, no recurrence; mild, ≤2 relapses per year; moderate, 3–4 relapses per year; severe, ≥5 relapses per year. Q1: 1st quartile; Q3: 3rd quartile.

Clinical characteristics of GPP

Disease course, recurrence, triggering factors, and prognosis

Most respondents (69, 54.3%) had lived with GPP for fewer than 5 years, whereas 38 (29.9%) had the disease for more than 10 years. Distinct recurrence patterns were evident: 24.4% (31/127) never experienced a relapse, 36.2% (46/127) reported 1–2 flares per year, 17.4% (22/127) had 3–4 flares annually, and 22.0% (28/127) experienced more than five flares per year [Table 1].

Identified triggers included medications, infections, fatigue, emotional distress, and food [Supplementary Figure 2, http://links.lww.com/CM9/C319]. Nearly half of the respondents could not identify specific triggers. Medication-related factors included abrupt discontinuation or irregular tapering of corticosteroids (both systemic and topical) or conventional psoriasis treatments, as well as the use of vaccines, biologics, herbal medicines, antibiotics, and non-steroidal anti-inflammatory drugs. Infections, particularly in the respiratory tract and oral cavity, were significant contributors. Disease flares were more common in winter (32.3%, 41/127) and spring (25.2%, 32/127).

The most common outcomes were gradual skin normalization (76, 59.8%), development of psoriasis vulgaris (24, 18.9%), and progression to erythrodermic psoriasis (27, 21.3%). Younger age at disease onset was associated with a higher likelihood of recurring GPP flares (age with recurrence: 22 years [Q1–Q3: 7–41 years] vs. age without recurrence: 32 years [Q1–Q3: 23–54 years], P = 0.021). Recurrence patterns were significantly associated with outcomes (P = 0.015). Patients who developed psoriasis vulgaris experienced a higher frequency of GPP flares relative to those with erythrodermic psoriasis (P = 0.022) or complete remission (P = 0.034). No statistically significant associations were observed for sex, smoking, alcohol use, sleep, exercise, comorbidities, hospitalization, or treatment modalities [Table 2].

Table 2.

Analysis of association between clinical characteristics and recurrence of GPP.

Variables	n (%)	Recurrence				P-value
		Never	Mild	Moderate	Severe
Gender	127 (100.0)					0.175*
Male	54 (42.5)	16	20	8	10
Female	73 (57.5)	15	26	14	18
Smoking history	127 (100.0)					0.390†
Never	94 (74.0)	22	35	18	19
Current	18 (14.2)	5	4	1	8
Former	15 (11.8)	4	7	3	1
Alcohol use	127 (100.0)					0.688*
No	110 (86.6)	25	42	19	24
Yes	17 (13.4)	6	4	3	4
Sleep hours	127 (100.0)					0.171†
<5.0 h	7 (5.5)	2	4	0	1
5.0–7.5 h	68 (53.5)	11	26	13	18
>7.5 h	52 (40.9)	18	16	9	9
Exercise	127 (100.0)					0.096†
Never	56 (44.1)	12	18	11	15
Sometimes	15 (11.8)	2	7	2	4
Often	56 (44.1)	17	21	9	9
Comorbid psoriasis	127 (100.0)					0.744*
No	77 (60.6)	18	27	16	16
Yes	50 (39.4)	13	19	6	12
Other comorbidities	127 (100.0)					0.350*
No	83 (65.4)	21	32	14	16
Yes	44 (34.6)	10	14	8	12
Hospitalization	45 (100.0)					0.611*
No	34 (75.6)	8	16	7	3
Yes	11 (24.4)	4	4	2	1
Systemic therapy	45 (100.0)					0.825†
Single traditional agent	25 (55.6)	5	13	5	2
Single biologic	7 (15.6)	3	2	1	1
Combination	13 (28.9)	4	5	3	1
Prognosis	127 (100.0)					0.015†
Complete remission	76 (59.8)	19	30	14	13
Plaque psoriasis	24 (18.9)	4	4	5	11
Erythrodermic psoriasis	27 (21.3)	8	12	3	4

^*The intra-group comparison was performed using Mann–Whitney test. ^†The intra-group comparison was performed using Kruskal–Wallis test. GPP: Generalized pustular psoriasis.

Clinical manifestations and disease severity of GPP

Among the respondents, 91.1% (41/45) presented with von Zumbusch GPP, 6.7% (3/45) with annular GPP, and 2.2% (1/45) with localized GPP. During flares, 59.8% (76/127) of respondents exhibited pustules over their entire body; the extremities (33.9%, 43/127), trunk (28.3%, 36/127), hands or feet (22.8%, 29/127), face and neck (15.0%, 19/127), scalp (15.0%, 19/127), and skin folds (13.4%, 17/127) were most commonly affected. Symptoms included moderate-to-severe pruritus and pain; 46.7% (21/45) of respondents reported systemic symptoms such as fever, fatigue, skin effusion, and lower extremity edema. Mucosal involvement occurred in 20.0% (9/45) of cases, primarily presenting as geographic tongue. Nail abnormalities were present in 51.1% (23/45) of respondents, including subungual pustules (24.4%, 11/45) and pitting (22.2%, 10/45) [Table 3].

Table 3.

Clinical manifestations during flares of 45 GPP respondents.

Items	n (%)
Skin symptoms
Pruritus
Without pruritus	10 (22.2)
With pruritus	35 (77.8)
Mild	9 (20.0)
Moderate	17 (37.8)
Severe	9 (20.0)
Pain
Without pain	7 (15.6)
With pain	38 (84.4)
Mild	9 (20.0)
Moderate	15 (33.3)
Severe	14 (31.1)
Description of pain
Sting	6 (13.3)
Causalgia	28 (62.2)
Indescribable	4 (8.9)
Systemic symptoms
Without systemic symptoms	24 (53.3)
With Systemic symptoms*	21 (46.7)
Fever	15 (33.3)
37.3–37.9 °C	1 (2.2)
38.0–38.9 °C	4 (8.9)
39.0–39.9 °C	9 (20.0)
≥40.0 °C	1 (2.2)
Fatigue	11 (24.4)
Shiver	10 (22.2)
Lower extremity edema	8 (17.8)
Skin effusion	8 (17.8)
Myalgia	6 (13.3)
Headache	4 (8.9)
Lymphadenopathy	2 (4.4)
Trichomadesis	2 (4.4)
Extra-cutaneous manifestations
Mucosal involvement
Without mucosal involvement	36 (80.0)
With mucosal involvement	9 (20.0)
Tongue	5 (11.1)
Vulvar	3 (6.7)
Eye	1 (2.2)
Nail involvement
Without Nail involvement	22 (48.9)
With Nail involvement*	23 (51.1)
Subungual pustule	11 (24.4)
Pitting	10 (22.2)
Oil-drop discoloration	4 (8.9)
Onychoptosis	2 (4.4)
Nail bed hyperkeratosis	1 (2.2)
Onycholysis	1 (2.2)

Data were presented as n (%). ^*Different clinical manifestations might coexist in the same respondent. GPP: Generalized pustular psoriasis.

Physicians assessed disease severity using the GPPASI; the mean score was 23.6 ± 13.1. Based on the GPPASI, the disease was classified as severe in 91.1% (41/45) of respondents, mild in 6.7% (3/45), and moderate in 2.2% (1/45). Severity evaluated using the JDA-SI for respondents with systemic symptoms indicated that 66.7% (14/21) experienced severe episodes, whereas 33.3% (7/21) had moderate episodes.^[16]

GPP comorbidities and complications during treatment

In this study, 53.5% (68/127) of respondents reported comorbidities; overweight or obesity was most common (34.6%, 44/127), followed by hypertension, diabetes, hyperlipidemia, and others. Regarding psychological distress, 84.5% (38/45) of respondents indicated feelings of depression, and 95.6% (43/45) experienced anxiety [Supplementary Table 1, http://links.lww.com/CM9/C319].

During GPP treatment, 28.9% (13/45) of respondents reported discomfort, primarily mucocutaneous dryness. Systemic complications affected 51.1% (23/45) of patients, and hypoalbuminemia was most common (33.3%, 15/45). Other complications included infections (26.7%, 12/45), electrolyte imbalances (17.8%, 8/45), elevated liver enzyme levels (13.3%, 6/45), and conditions such as chronic heart failure [Supplementary Table 1, http://links.lww.com/CM9/C319].

GPP disease burdens

Diagnostic challenges and impacts on the quality of life

A considerable diagnostic delay was observed, such that 52.8% (67/127) of respondents experienced an average delay of 8 months (Q1–Q3: 2–41 months); the longest delay was 35 years [Supplementary Figure 3, http://links.lww.com/CM9/C319]. Nearly half of the respondents (45.7%, 58/127) required visits to two or more hospitals for diagnosis, and 20.5% (16/127) sought treatment in more than three hospitals because of unsatisfactory experiences. Before receiving a GPP diagnosis, 32.3% (41/127) of patients were misdiagnosed, most frequently with eczema or atopic dermatitis (41.5%, 12/41), fungal infections (17.1%, 7/41), drug eruptions (9.8%, 4/41), or impetigo (7.3%, 3/41).

Quality of life during GPP flares was severely affected according to the DLQI score; 86.7% (40/45) of respondents reported substantial disruption of daily activities. The median DLQI score was 19.0 (Q1–Q3: 13.0–23.5). Patients with systemic symptoms had significantly higher DLQI scores than those without (with systemic symptoms: 22.0 [Q1–Q3: 17.5–25.0] vs. without systemic symptoms: 15.5 [Q1–Q3: 10.0–20.0], P = 0.016). DLQI scores were positively correlated with anxiety (r = 0.440, P = 0.003) and depression scores (r = 0.586, P <0.001). During flares, only 31.1% (14/45) of respondents were able to care for themselves. The majority relied on family members (e.g., parents [31.1%, 14/45], spouses [28.9%, 13/45], or descendants [8.9%, 4/45]) for assistance.

Hospitalization and drug utilization among GPP patients

During the survey, 75.6% (34/45) of respondents with acute GPP required hospitalization; 33.3% (15/45) were hospitalized for longer than 2 weeks (mean stay: 13.0 ± 4.3 days). 75.6% (96/127) of patients were actively receiving treatment. For GPP patients during acute flares, common systemic treatments included acitretin (55.5%, 25/45), biologics (42.2%, 19/45), cyclosporin (26.7%, 12/45), and traditional Chinese medicine (11.1%, 5/45). 55.5% (25/45) selected non-biologic monotherapy, biologic monotherapy was used by 15.6% (7/45) of patients, and 28.9% of patients (13/45) received combination systemic therapy. For biologics, interleukin (IL)-17 inhibitors were the most common used (31.1%, 14/45). Tumor necrosis factor inhibitors and IL-23 inhibitors were prescribed to 8.9% (4/45) and 2.2% (1/45) of patients, respectively. Topical treatments included corticosteroids (64.4%, 29/45), emollients (55.6%, 25/45), antibiotics (13.3%, 6/45), vitamin D3 analogs (13.3%, 6/45), astringents (13.3%, 6/45), and calcineurin inhibitors (8.9%, 4/45). Treatment discontinuation was reported by 18.1% (23/127) of respondents. Among them, 60.9% (14/23) had recovered, 17.4% (4/23) stopped due to concerns about side effects or treatment efficacy, and 13.0% (3/23) discontinued treatment because of financial constraints, 8.7% (2/23) did not clarify the reasons for drug discontinuation.

Economic burden of GPP

The currency used in this study was Chinese yuan (CNY), with an exchange rate of approximately US$1 = 7.20 CNY as of February 2024. Among the respondents, 81.1% (103/127) reported a monthly family income of ≤10,000 CNY. All-cause costs over the past 12 months considerably varied: 26.0% (33/127) of patients spent <10,000 CNY, 36.2% (46/127) spent 10,000–30,000 CNY, 15.0% (19/127) spent 30,000–50,000 CNY, 10.2% (13/127) spent 50,000–70,000 CNY, and 12.6% (16/127) spent >70,000 CNY. Drug expenses as a proportion of total costs also varied: among 119 respondents who answered this question, 5% (6/119) spent <20%, 25.2% (30/119) spent <50%, 40.3% (48/119) spent 50–80%, and 29.4% (35/119) spent >80%. Respondents rated their economic burden on a scale of 1–10 (a rating of 1 indicates a high burden and a rating of 10 indicates a low burden); 55.9% (71/127) rated their burden between 1 and 5. These findings indicate that GPP treatment imposes substantial financial strain on more than half of the respondents.

Discussion

This study investigated the demographic and clinical characteristics of GPP—a chronic systemic inflammatory disease affecting individuals of all ages—in the Chinese population. The higher prevalence among girls/women, with a female-to-male ratio of 1.35:1, is consistent with previous studies that showed ratios between 1.03:1 and 2:1.^{[5, 17, 18]} The age of onset distribution highlights the extensive impacts on newborns, young children (0–10 years), and young adults (20–40 years), potentially influencing both physical and mental development.^[19]

This study provides valuable insights into the medical and economic burdens of GPP in China, with three key findings. First, our diagnostic findings were consistent with previous data indicating that affected patients frequently experience misdiagnoses due to the diverse clinical presentations of GPP.^[10] The disease has been classified into six subtypes based on clinical manifestations.^{[15,18,20,21,22]} Among our respondents, acute von Zumbusch GPP, the most severe subtype, was the most common (91.1%). More than half of the respondents (52.8%) experienced delays in GPP diagnosis, ranging from days to years. Contributing factors included misdiagnosis (reported by 32.3% of respondents), difficulties in obtaining an accurate diagnosis at certain healthcare facilities, and patient-related postponement of medical attention until symptoms become severe. These findings emphasize the lack of GPP awareness in China. Additionally, the historical absence of an international consensus regarding diagnostic criteria, combined with the rarity of the disease, has led to further diagnostic delays.^{[3, 23]} Despite the high disease burden, 45.7% of respondents required visits to multiple healthcare facilities to obtain a correct diagnosis, exacerbating the medical challenges.^{[6, 24]}

Second, our results concerning GPP flares were aligned with prior findings that GPP is a chronic disease characterized by intermittent, relapsing flares.^[25] In the present study, three-quarters of respondents reported multiple recurrences. Triggers substantially varied among individuals and even within the same individual across flares. Approximately, half of the respondents were unable to identify specific triggers, which constitutes a challenge for GPP prevention. Among respondents who identified triggers, drug-related factors (e.g., abrupt discontinuation of medications such as corticosteroids and cyclosporine) were frequently reported. This finding is consistent with previous studies.^[26,27,28] Coronavirus disease 2019 vaccine administration also emerged as a potential trigger, possibly linked to the activation of type I interferons and plasmacytoid dendritic cells.^{[29, 30]} Emotional distress was another commonly reported trigger, consistent with the results of Reisner et al.^[10] Fatigue was identified as a potential trigger by our patients; in previous research, it has been more commonly described as an accompanying symptom.^{[10, 31]} Potential relationships between fatigue and GPP episodes warrant further exploration. Dietary factors also constituted possible triggers. However, dietary triggers might have been subjectively reported, and varying foods were implicated among participants. The precise role of dietary factors in triggering GPP requires additional investigation. We also examined the association between GPP flares and clinical characteristics. Patients with early-onset GPP and those with a prognosis of psoriasis vulgaris experienced more frequent relapses, providing insights for clinical management. Early-onset GPP has been associated with a higher frequency of IL36RN gene mutations, which may increase the risk of flares.^{[32, 33]} Nevertheless, further high-quality evidence regarding GPP flare risk factors is needed; the relationship between prognosis and recurrence identified in our relatively small patient population requires validation in future studies.

Third, we characterized the substantial disease burdens associated with GPP.^{[5, 8, 25]} Previous studies have shown that GPP affects multiple organ systems and is associated with a range of systemic symptoms, comorbidities, and complications. In our study, 46.7% of respondents reported systemic symptoms during flares, consistent with prior research.^{[10, 17, 34]} Other researchers have documented infectious, metabolic, cardiac, hepatic, respiratory, and neurological complications, which matched our findings.^{[35, 36]} Mental health was substantially impacted, such that 95.6% of respondents experienced anxiety and 84.5% exhibited depression; these rates were higher than the rates in a prior survey within the United States.^[10] Our findings underscore the need for comprehensive care addressing both physical and mental health in the Chinese population.

Moreover, GPP profoundly affects quality of life. Self-evaluations using the DLQI revealed a substantial impact on daily activities during flares, particularly in individuals with systemic symptoms. Higher DLQI scores have been correlated with disease severity.^{[12, 17]} In the present study, DLQI scores were positively correlated with anxiety and depression scores, emphasizing the interplay among disease severity, mental health, and life quality. Many patients, particularly those with severe disease, relied on social support from family members for daily activities.

It has been reported that GPP results in a high hospitalization burden.^{[6, 24]} In the present study, hospitalization rates among GPP patients were high, such that 75.6% of respondents required hospital care; these rates reflect the substantial challenges involved in managing GPP. Three-quarters of respondents were undergoing treatment, but the absence of standardized treatment regimens likely hinders consistent disease control and flare prevention.^[9] Treatment discontinuation was driven by concerns about efficacy, costs, and adverse events. These findings highlight the need for more effective and targeted therapies, such as IL-36RN antagonists, to improve disease management and reduce healthcare resource utilization.^{[37, 38]} Considering the financial burden related to GPP, evaluations of treatment cost-effectiveness are essential for optimizing care among affected patients.

Additionally, GPP imposes a considerable economic burden on affected patients. In this study, more than half of respondents reported financial difficulties, primarily due to low family incomes and the high costs associated with treatment regimens. For nearly 70% of respondents, medication expenses comprised >50% of their total GPP-related costs; the use of biological agents substantially increased this burden.^[39] Financial constraints led to a 13.0% treatment discontinuation rate, emphasizing the need for affordable and accessible therapeutic options. Efforts to address the economic impact of GPP are essential for enhancing patient compliance and overall quality of life.^{[6, 7, 25]}

This study had some limitations. First, the relatively small sample size may limit the generalizability of the findings. The study was conducted during the pandemic, which restricted access to participating hospitals and may have affected the representativeness of the GPP patient population in the survey. Second, although efforts were made to reduce bias, the potential for selection and recall bias remains. Third, the study did not investigate treatment sequences, treatment effects, or the relationship between treatment and prognosis; further research is needed in these areas. Finally, reliance on self-reported recurrence data may not accurately reflect real-world disease patterns due to non-standardized recurrence criteria, potentially leading to an overestimation of GPP burden.^[9]

In conclusion, the current understanding of GPP remains inadequate. There is a need to standardize clinical diagnostic criteria, enhance physician training and patient education, and increase diagnostic accuracy and timeliness. GPP is a severe and complex disease characterized by frequent recurrence, clinical heterogeneity, comorbidities, and substantial systemic symptoms during flares. The disease imposes a considerable economic burden on patients. Early onset and the transition to plaque psoriasis appear to increase the risk of recurrence. Standardized treatment protocols and effective therapies to prevent relapse are urgently needed to address the inadequacies of current management strategies.

Funding

This study was supported by Beijing Natural Science Foundation (No. 7242109), CAMS Innovation Fund for Medical Sciences (No. 2021-I2M-1-059), the National High Level Hospital Clinical Research Funding (No. 2022-PUMCH-B-092), and the Beijing Key Clinical Specialty Construction Project.

Conflicts of interest

None.