BACKGROUND:Value-based healthcare emphasizes outcomes that matter to patients, and patient-centred outcomes sets are vital to its success. For psoriasis, initial work proposed a patient-centred outcomes set in Belgium, but it requires further validation to ensure international applicability. OBJECTIVES:This scoping study aimed to refine the outcomes set in collaboration with patient representatives and dermatologists, in preparation for international validation through a Delphi consensus process. METHODS:An international Working Group of patient representatives and dermatologists was established through the International Federation for Psoriasis Associations (IFPA) and the International Psoriasis Council (IPC). Experts participated in three discussion meetings and subsequent surveys to discuss and recommend outcomes important to people living with psoriasis, their measurement and case-mix variables. The systematic review of patient-relevant outcomes was updated as well and outcome measurement instruments were selected corresponding to the COSMIN criteria. RESULTS:The Working Group included 35 experts (12 patient representatives and 23 dermatologists) from 22 countries. A total of three discussion meetings and two subsequent surveys informed the refinement of the outcomes set. 'Acceptable costs of care for society' was excluded as an outcome, four outcomes were merged into 'psoriasis clearance' and 'social activity', and 'communication' and 'confidence in care' were reclassified as patient experiences. 'Feelings of stigmatization' and 'number of flare-ups' were added based on patient recommendations. These changes resulted in a revised set of 18 patient-relevant outcomes and 2 patient experiences. After selecting outcome measurement instruments, a heatmap was compiled to assess overlap, quality and feasibility. Finally, 50 case-mix variables were proposed based on the literature and expert opinions. CONCLUSIONS:This scoping study convened an international Working Group of patient representatives and dermatologists to establish the fundamentals for a patient-centred outcomes set. The subsequent Delphi process will finalize consensus, advancing value-based psoriasis management worldwide.

While international studies have assessed the economic burden of alopecia areata (AA), its societal costs have not been quantified in a Nordic context. We conducted a cross-sectional survey among adults with self-reported AA in Norway and Sweden, recruited via a patient organization and social media. A total of 329 respondents (263 from Norway, 66 from Sweden) provided information on demographics, disease characteristics, healthcare utilization, out-of-pocket expenses, productivity losses and treatment satisfaction. Costs were estimated from a societal perspective, combining direct medical, direct non-medical and indirect costs from reduced productivity. The annual mean total cost of AA was €7,677 in Norway and €12,582 in Sweden, with indirect costs (61–64% of the total) as the largest component, primarily driven by presenteeism and long-term sick leave. A notable finding is the significant out-of-pocket costs. In Norway, individuals paid about 65% of direct costs themselves, in Sweden about 50%. Dissatisfaction with treatment and healthcare support was widespread. Only a minority received systemic therapies, and treatment frequency with Janus kinase inhibitors was low, likely due to lack of reimbursement. AA imposes a considerable societal and individual economic burden in Norway and Sweden, underscoring the need for better therapies, healthcare support and policy recognition of its impact.

Hard-to-heal arterial leg ulcers in older adults are a challenging and complex condition. In this quasi-experimental study, three treatment approaches were compared. The purpose was to investigate (1) the healing time of arterial leg ulcers in older adults (≥ 70 years) who underwent photobiomodulation, revascularisation, or conservative treatment; (2) the importance of factors associated with impaired healing; and (3) ulcer recurrence after healing with photobiomodulation. Participants who received photobiomodulation (n = 51) were frail older adults recruited from municipal home healthcare and matched with participants who received revascularisation (n = 71) or conservative treatment (n = 153). The latter two groups were retrieved from the Swedish Quality Registry RiksSår for ulcer treatment. Photobiomodulation was performed at wavelengths of 635 and 904 nm twice weekly. The results showed that the photobiomodulation group had a significantly shorter healing time (p < 0.001) and a higher proportion of healed ulcers; photobiomodulation 66.7%, revascularized 50.7% and conservatively treated group 41.2%. The median healing times for the photobiomodulation group were 135 days (confidence interval 95-175), compared to 252 (confidence interval 181-323) and 316 (confidence interval 192-440) in revascularized and conservatively treated groups, respectively. Neither ulcer duration nor other pretreatment factors exerted clinically relevant effects on healing time. In this study, recurrence within 24 months of healing with photobiomodulation was < 12%. In conclusion, photobiomodulation has the potential to heal hard-to-heal arterial ulcers markedly faster than revascularisation or conservative treatment. It could be a suitable treatment alternative for frail older adults, including those with previous substantial ulcer duration.

PurposePatient-reported experience measures represent the patient's voice and offer a way to evaluate continuity of care. We investigated the impact of having an assigned care provider on experienced involvement, information, and emotional support.MethodsData from a national survey sent to patients recently diagnosed with cancer. Answers were grouped and compared using Pearson's chi-square test.ResultsA total of 89.1% of respondents reported having an assigned care provider. These individuals reported higher levels of involvement, information, and emotional support compared to individuals who did not have an assigned care provider. The profession of the care provider had little impact.ConclusionAn assigned care provider is important for maintaining continuity of care. This should be encouraged in cancer care as it leads to better patient experience across all investigated domains. The widely spread use of contact nurses in cancer care in Sweden provides a solid ground for such continuity. The continuous use of patient-reported experience measures promotes more people-centered healthcare practices.

BACKGROUND:Real-world data on health-related quality of life (HRQoL) in generalized pustular psoriasis (GPP) are scarce and studies have been restricted in terms of instruments used for assessments. OBJECTIVE:To assess generic and dermatology-specific HRQoL of patients with GPP compared with patients with plaque psoriasis using real-world data from the Swedish National Register for Systemic Treatment of Psoriasis. METHODS:Cross-sectional data from 2006 to 2021 including 7041 individuals with plaque psoriasis without GPP and 80 patients with GPP, of which 19% also had plaque psoriasis. Total scores for the EuroQol-5 Dimensions (EQ-5D) and Dermatology Life Quality Index (DLQI), as well as degree of severity within the instruments' dimensions/questions, were compared between patient groups. RESULTS:EQ-5D scores were significantly (p < .01) lower (worse) in patients with GPP (mean [standard deviation (SD)] 0.613 [0.346]) vs. patients with plaque psoriasis (mean [SD] 0.715 [0.274]), indicating lower generic HRQoL of patients with GPP. Significantly (p < .01) higher (worse) total DLQI scores were observed for patients with GPP (mean [SD] 10.6 [8.9]) compared with patients with plaque psoriasis (mean [SD] 7.7 [7.1]), with proportionally more patients with GPP having severe (20% vs. 16%) and very severe (17% vs. 8%) problems. The worsened scores for GPP vs. plaque psoriasis were consistent across EQ-5D dimensions and DLQI questions. CONCLUSIONS:Individuals with GPP have a considerable impairment in both generic and dermatology-specific HRQoL. The HRQoL was significantly worse in individuals with GPP compared to individuals with plaque psoriasis. The significant HRQoL impairment of GPP shows the potential value of better healthcare interventions for this multisystem disease.

BACKGROUND:Real-world data on health-related quality of life (HRQoL) in palmoplantar pustulosis (PPP) are scarce and few studies have analysed the generic HRQoL. OBJECTIVES:To assess HRQoL using the generic EQ-5D instrument and the Dermatology Life Quality Index (DLQI) instrument in PPP compared to plaque psoriasis. METHODS:Cross-sectional data from PsoReg, the Swedish National Registry for Systemic Treatment of Psoriasis (2006-2021), were examined. The study included 306 patients with PPP, out of which 22% had concomitant plaque psoriasis (n = 68), and 7041 patients with plaque psoriasis only. EQ-5D and DLQI were compared between patients with PPP and patients with plaque psoriasis, overall and stratified by sex. A subgroup analysis compared outcomes for patients with PPP vs. patients with severe plaque psoriasis (Psoriasis Area and Severity Index ≥10). Multiple regression analyses were performed to control for potential confounders (age, sex, comorbidities, lifestyle factors). RESULTS:Patients with PPP were to a larger extent female (79% vs. 37%, p < .01) and older (mean [SD] age 59.9 [11.9] vs. 50.7 [16.0] years, p < .01) than patients with plaque psoriasis. EQ-5D values were significantly lower (worse) in patients with PPP (mean [SD] 0.622 [0.309]) compared to patients with plaque psoriasis (mean [SD] 0.715 [0.274]). No significant difference was observed compared to patients with severe plaque psoriasis (p = .237). DLQI was comparable in PPP and plaque psoriasis patients (p = .117). In the regression analyses, PPP only and PPP with plaque psoriasis were associated with lower EQ-5D values of 0.065 (p < .01) and 0.061 points (p < .10) compared to plaque psoriasis patients. CONCLUSIONS:PPP had a substantial negative impact on patients' generic and dermatology-specific HRQoL. Patients with PPP were worse off in terms of generic HRQoL compared with patients with plaque psoriasis when controlling for the impact of potential confounders.

Background: Since the introduction of biologics for psoriasis, uptake has been uneven and limited. Few studies have investigated the influence of socioeconomic factors on access to biologics. Objective: To investigate how socioeconomic factors influenced access to biologics. Methods: Biologic-naïve patients in the Swedish National Register for Systemic Treatment of Psoriasis (PsoReg) for the years 2006–2014 were included. For patients who remained on nonbiologic treatments during their entire registration (n = 1851), the most recent registration was analyzed. For patients who began treatment with biologics during registration in PsoReg (n = 665), the last observation before initiation of biologics was analyzed. A logistic regression model was used to investigate whether education and income influenced the probability of a switch to biologics, whilst adjusting for demographic and individual factors such as age, sex, disease severity, and clinical characteristics. Results: The odds ratio of access to biologics was 1.8 (CI = 1.3–2.6) in the group with a high level of disposable income, compared with the middle-income group. No differences were found concerning educational levels. The odds ratios of access to biologics decreased with age. Patients with psoriatic arthritis had odds ratios of access to biologics which were more than 50 percent higher, controlling for other variables. High disease severity, in terms of physician- and patient-reported severity, increased the odds ratios of access to biologics. Conclusions: The higher-income group had better access to biologics than the middle-income group when adjusting for disease severity and lifestyle factors. This may not only be an equity problem, as a better allocation of society’s resources might have resulted in a higher overall effectiveness of biologics.

This study investigated the comorbidity burden of Swedish patients with palmoplantar pustulosis (PPP) vs. the general population and patients with psoriasis vulgaris (PV). We found a significantly higher comorbidity burden in patients with PPP compared with the general population, represented by higher odds for disease across different categories (metabolic, cerebrovascular, cardiovascular, psychiatric). Although patients with PPP and PV showed similar comorbidity burdens overall, the comorbidity profiles differed significantly. Comorbidities such as type 2 diabetes and chronic obstructive pulmonary disorder, which showed both high prevalence (> 5%) in PPP and higher odds than PV, may be of particular importance for the specific comorbidity burden of PPP.

The aim of this study was to analyse sick leave in generalized pustular psoriasis, the most severe form of pustular psoriasis. Prolonged sick leave of >14 days was analysed for 502 patients with generalized pustular psoriasis compared with controls with psoriasis vulgaris and matched controls from the general population. Using data from the Swedish National Patient Register, and the Longitudinal integrated database for health insurance and labour market studies, the study estimated the mean number of sick leave days in the year of first diagnosis of generalized pustular psoriasis (index year) and for 2 years before and after the index year. Patients with generalized pustular psoriasis were on sick leave to a larger extent than both control populations for all study years. The number of sick leave days peaked in the index year and then reduced. Compared with the control populations, sick leave in generalized pustular psoriasis was already higher prior to diagnosis, indicating delayed diagnosis and/or a comorbidity burden.

Generalized pustular psoriasis (GPP) is the most severe form of pustular psoriasis.1,2 A high comorbidity burden has been indicated,3,4 albeit not comprehensively investigated. In this nationwide study, we have studied the comorbidity profile of GPP and compared it to the general population and patients with psoriasis vulgaris (PV).

The aim of this study was to estimate the economic burden of palmoplantar pustulosis, a chronic relapsing skin condition commonly occurring in combination with psoriasis vulgaris. Using data from the Swedish National Patient Register and Swedish Prescribed Drug Register for 2015, the study estimated all-cause and palmoplantar pustulosis-specific healthcare resource use (inpatient stays, physician visits and drug use) for 14,715 patients with palmoplantar pustulosis, and compared these both with matched controls from the general population and with patients with psoriasis vulgaris (without palmoplantar pustulosis). Mean annual direct costs for a patient with palmoplantar pustulosis was higher compared with costs for the general population (3,000 vs 1,700 Euro, p < 0.001). Compared with psoriasis vulgaris, more patients with palmoplantar pustulosis had inpatient stays, but fewer had physician visits and psoriasis-related drugs; the overall costs were similar. Only a small fraction of the costs of physician visits and inpatient stays for patients with palmoplantar pustulosis were attributable to specific palmoplantar pustulosis problems, indicating a clear comorbidity burden in palmoplantar pustulosis.

Background Psoriasis severity has traditionally been categorized as mild, moderate, and severe. Commonly, cut-offs for severe disease require a body surface area (BSA) involvement of ≥10% or a Psoriasis Area Severity Index (PASI) > 10. However, clinical experience challenges these traditional measures and requirements, as patients with less extensive psoriasis may have disease that severely impacts quality of life. Objective The objective of the present study was to further explore the extent of patient burden when psoriasis affects special locations. Methods A total of 69,190 individuals living in the U.S were invited to participate in a patient advocacy survey by telephone and or web interviews over the course of 3 years (2019-2021). The survey instrument consisted of validated patient-reported outcome measures, measuring disease-specific quality of life (Dermatology Life Quality Index, DLQI), depression (Patient Health Questionnaire (PHQ)-2 and (PHQ)-9), and the ability to participate in social roles and activities (PROMIS Ability to Participate in Social Roles and Activities (SF-4a). Chi-square tests were performed to explore association between psoriasis involvement on special locations and patient outcomes and multivariate logistic regression models were then constructed, to assess impact of having psoriasis on special locations patient outcomes, controlling for potential confounding factors. Results A total of 4129 individuals completed the survey. 3594 (84.4%) of patients surveyed reported psoriasis involving special areas of the bodysuch as the scalp, face, hands, feet, or genitalia. Involvement of special areas is associated with worse quality of life and depression. 35-71% of patients with 10% or less total BSA involvement experienced a moderate-to-extremely large effect on these life function domains. When adjusting for age, sex, and body surface area, psoriasis involvement of a special location was associated with poorer patient reported outcomes, including a 46% less likelihood of reporting their skin disease as having “no or only a small effect on QoL,” a 30% less likelihood of having a “normal l ability to participate in social roles and activities,” and a 126% higher likelihood of f having depression. Conclusion Real-world data presented here demonstrate that psoriasis involving special areas is associated with adverse life consequences, including poor quality of life and depression.

Background Patient reported experiences in individuals being investigated for cancer have been recorded in a nationwide survey in Sweden, providing an opportunity to assess the impact of the Covid-19-pandemic. Material and Methods Questionnaires from 45920 patients were analyzed to assess the experience of being investigated for cancer. Data from before the Covid-19-pandemic (2018–2019) was compared to data acquired during the pandemic (2020–2021), using chi-square and Wilcoxon rank sum tests. Both, patients who were cleared from suspicion of cancer and those who were diagnosed with cancer were included. Results Fewer patients in total visited health services during the pandemic. However, patients that did seek help did so to a similar extent during as prior to the pandemic. Patient waiting time was perceived to be shorter during the pandemic and judged as neither too long nor too short by most patients. The emotional support to patients improved during the pandemic, whereas the support to next of kin declined. A majority of patients received the results from the investigation in a meeting with the physician. Although there was a preference for receiving results in a meeting with the physician, the pandemic has brought an increasing interest in receiving results by phone. Conclusion Swedish cancer healthcare has shown resilience during the Covid-19-pandemic, maintaining high patient satisfaction while working under conditions of extraordinary pressure. Patients became more open to alternatives to physical “in person” health care visits which could lead to more digital visits in the future. However, support to significant others demands special attention.

Evidence-based medicine was in the past primarily based on the (meta-)analysis of randomized clinical trials (RCTs) [...].

Background: Interleukin (IL) inhibitors have made completely cleared skin achievable for many patients with moderate to severe psoriasis in clinical trial settings. Few observational studies assess treatment response in accordance with treatment goals in guidelines. Objectives: The aim of the study was to analyze the treatment response of IL-17/IL-23 inhibitors in clinical practice and the proportions of patients that reach the treatment target of the Psoriasis Area and Severity Index (PASI) < 3 and the Dermatology Life Quality Index (DLQI) ≤5. Methods: A longitudinal, observational study based on the Swedish National Registry for Systemic Treatment of Psoriasis, PsoReg. Patients using IL-17/IL-23 inhibitors with assessments of PASI, DLQI, and EQ-5D before (maximum 6 months) and after (3–12 months) initiation of IL-17/IL-23 were included. Results: In total, 333 patients using IL-17/IL-23 inhibitors were included. Eighty percent (n = 266) received IL-17 inhibitors, and 20% (n = 67) received IL-23 inhibitors. Sixty-six percent of patients reached both PASI <3 and DLQI ≤5, 23% reached one target, and 11% reached none. The mean (SD) PASI, DLQI, and EQ-5D improvements were 6.75 (6.99), 7.14 (7.97), and 0.126 (0.296), respectively. There was no statistically significant difference in outcomes between IL-17 and IL-23 inhibitor treatment groups. Conclusions: IL-17/IL-23 inhibitors are effective in clinical practice, but there is still an unmet therapeutic need in moderate to severe psoriasis.

To the Editor: Generalized pustular psoriasis (GPP) (prevalence, 1.8-124/million people1Prinz J.C. Choon S.E. Griffiths C.E.M. et al.Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review.J Eur Acad Dermatol Venereol. 2023; 37: 256-273https://doi.org/10.1111/jdv.18720Crossref PubMed Scopus (5) Google Scholar,2Löfvendahl S. Norlin J.M. Schmitt-Egenolf M. Prevalence and incidence of generalized pustular psoriasis in Sweden: a population-based register study.Br J Dermatol. 2022; 186: 970-976https://doi.org/10.1111/bjd.20966Crossref PubMed Scopus (15) Google Scholar) is a severe form of pustular psoriasis characterized by acute flares with systemic inflammation.3Umezawa Y. Ozawa A. Kawasima T. et al.Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity.Arch Dermatol Res. 2003; 295: S43-S54https://doi.org/10.1007/s00403-002-0371-6Crossref PubMed Scopus (116) Google Scholar GPP is associated with several comorbidities,1Prinz J.C. Choon S.E. Griffiths C.E.M. et al.Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review.J Eur Acad Dermatol Venereol. 2023; 37: 256-273https://doi.org/10.1111/jdv.18720Crossref PubMed Scopus (5) Google Scholar,4Löfvendahl S. Norlin J.M. Schmitt-Egenolf M. Comorbidities in patients with generalized pustular psoriasis: a nationwide population-based register study.J Am Acad Dermatol. 2023; 88: 736-738https://doi.org/10.1016/j.jaad.2022.09.049Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar and ∼50% of patients with GPP have concomitant psoriasis vulgaris (PV).1Prinz J.C. Choon S.E. Griffiths C.E.M. et al.Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review.J Eur Acad Dermatol Venereol. 2023; 37: 256-273https://doi.org/10.1111/jdv.18720Crossref PubMed Scopus (5) Google Scholar,4Löfvendahl S. Norlin J.M. Schmitt-Egenolf M. Comorbidities in patients with generalized pustular psoriasis: a nationwide population-based register study.J Am Acad Dermatol. 2023; 88: 736-738https://doi.org/10.1016/j.jaad.2022.09.049Abstract Full Text Full Text PDF PubMed Scopus (2) Google Scholar We have assessed mortality and cause of death in GPP compared with the general population (GP) and patients with PV. From the Swedish National Patient Register, we identified 1093 physician-diagnosed patients with GPP using a criterion of 1 primary or secondary GPP diagnosis (International Classification of Diseases, 10th revision, code L40.1) in 2004-2015. These were matched (1:5) to controls from the GP and (1:3) to controls with PV (and no GPP or palmoplantar pustulosis), on the basis of sex and age (flowcharts; Supplement 1, available via Mendeley at https://data.mendeley.com/datasets/5tt3kgr9kt/1). Individuals deceased between 2004 and 2020 were identified in the Swedish Cause of Death Register. Survival was compared between groups using Kaplan-Meier curves. Age-stratified hazard ratios (HRs) for all-cause mortality were determined by Cox regression models. In subgroup analysis, we limited analyses to patients with ≥2 GPP diagnoses (a stricter GPP criterion) given at different occasions during the study period and matched controls. In the final GPP cohort (n = 1022), 44% had ≥2 GPP diagnoses and 54% had concomitant PV. The GPP cohort had relatively more deaths over the study period and ∼50% higher mortality rate compared with both control populations (Table I and Supplement 2, available via Mendeley at https://data.mendeley.com/datasets/5tt3kgr9kt/1). Kaplan-Meier survival curves for the GPP cohort (Fig 1 and Supplement 3, available via Mendeley at https://data.mendeley.com/datasets/5tt3kgr9kt/1) showed a drop in survival immediately after diagnosis, which may be due to GPP life-threatening complication, triggered by factors such as infections, drugs, or hypocalcemia.3Umezawa Y. Ozawa A. Kawasima T. et al.Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity.Arch Dermatol Res. 2003; 295: S43-S54https://doi.org/10.1007/s00403-002-0371-6Crossref PubMed Scopus (116) Google Scholar Thereafter, survival for GPP decreased steadily over the study period and was consistently lower than survival curves for the control groups. The mortality rate was higher in the GPP cohort (33/1000 person-years) compared with both GP and PV control populations (21 and 22/1000 person-years), with higher risks (HRs > 1.5) across all age groups. The highest relative risks (HRs > 2) were observed for the younger age groups. However, the absolute death risk was low in these groups (Table I).Table IBaseline characteristics and mortality outcomes (overall and by age at diagnosis) for the GPP cohort and the matched control groupsGPP cohort n = 1022Matched control groupsGeneral population∗Matched on year of birth, sex, and residential area. n = 4842Psoriasis vulgaris†Matched on year of birth, sex, and index year (ie, year of first PPP or PV diagnosis). n = 3048Women, n (%)631 (61.7)3015 (62.3)1886 (61.9)Patients with at least 2 GPP diagnoses (given at different occasions) 2004-2015, n (%)452 (44.2)N/AN/APatients with a psoriasis vulgaris diagnosis‡L40.0 or L40.9 as primary diagnosis. 2004-2015, n (%)555 (54.3)N/AN/AAge at diagnosis, mean (SD)All58.4 (16.6)58.1 (16.5)58.3 (16.5)Men58.0 (16.0)57.6 (16.0)57.8 (16.0)Women58.6 (16.9)58.3 (16.8)58.6 (16.9)Age (intervals) at diagnosis, n (%)18-44211 (20.7)1015 (21.0)633 (20.8)45-64424 (41.5)2038 (42.1)1267 (41.6)65-79284 (27.8)1331 (27.5)845 (27.7)≥80103 (10.1)458 (9.5)303 (9.9)Follow-up time in years (overall and across age at diagnosis intervals), mean (SD)Overall9.3 (4.6)10.0 (4.3)10.1 (4.1)18-4411.3 (3.9)11.2 (3.9)11.5 (3.6)45-6410.4 (4.3)11.0 (4.1)11.1 (3.8)65-798.1 (4.2)8.9 (4.1)9.0 (3.8)≥804.2 (3.5)5.8 (3.7)6.4 (3.6)No. of deaths during follow-up, n (%)Overall315 (30.8)1027 (21.2)679 (22.2)18-447 (0.7)9 (0.2)6 (0.2)45-6481 (7.9)198 (4.1)145 (4.8)65-79135 (13.2)463 (9.6)291 (9.5)≥8092 (9.0)357 (7.4)237 (7.8)Person-years at risk§The total sum of the number of years that each member of a study population has been under observation.Overall955048,23630,92618-44238611,362730245-64441522,33814,05965-79231411,8617622≥8043526751943Mortality rate (per 1000 person-years)Overall33.021.322.018-442.90.80.845-6418.38.910.365-7958.339.038.2≥80211.3133.5122.0Hazard ratios for all-cause mortality in GPP vs general population controls, (CI); (P value)Overall1.81 (1.58-2.08); (p < .001)18-443.49 (1.30-9.42); (P = .013)45-642.07 (1.58-2.70); (P < .001)65-791.61 (1.31-1.97); (P < .001)≥801.88 (1.43-2.46; (P < .001)Hazard ratios for all-cause mortality in GPP vs psoriasis vulgaris controls, (CI); (P value)Overall1.90 (1.63-2.21); (P < .001)18-443.50 (1.18-10.41); (P = .024)45-642.03 (1.52-2.71); (P < .001)65-791.66 (1.32-2.09); (P < .001)≥802.14 (1.58-2.90); (P < .001)GPP, Generalized pustular psoriasis (L40.1 as primary or secondary diagnosis); N/A, not applicable; PPP, palmoplantar pustulosis; PV, psoriasis vulgaris.∗ Matched on year of birth, sex, and residential area.† Matched on year of birth, sex, and index year (ie, year of first PPP or PV diagnosis).‡ L40.0 or L40.9 as primary diagnosis.§ The total sum of the number of years that each member of a study population has been under observation. Open table in a new tab GPP, Generalized pustular psoriasis (L40.1 as primary or secondary diagnosis); N/A, not applicable; PPP, palmoplantar pustulosis; PV, psoriasis vulgaris. The leading cause of death, grouped by International Classification of Diseases chapters, was diseases of the circulatory system. The GPP cohort had more deaths from circulatory, respiratory, and digestive diseases compared with GP controls (P < .001) and from digestive system compared with PV controls (P = .011) (Supplement 4, available via Mendeley at https://data.mendeley.com/datasets/5tt3kgr9kt/1). When limiting the analysis to patients with at least 2 codes for GPP (Supplement 5, available via Mendeley at https://data.mendeley.com/datasets/5tt3kgr9kt/1), we found a higher GPP mortality rate (overall 39.2/1000 person-years) and higher HRs (overall 2.13 compared with both control groups), suggesting that the stricter criterion resulted in selection of patients with more severe disease. The GPP mortality was in the higher range compared with small clinical studies (mortality rate range, 0-33/1000 person-years)1Prinz J.C. Choon S.E. Griffiths C.E.M. et al.Prevalence, comorbidities and mortality of generalized pustular psoriasis: a literature review.J Eur Acad Dermatol Venereol. 2023; 37: 256-273https://doi.org/10.1111/jdv.18720Crossref PubMed Scopus (5) Google Scholar and a large Japanese study (n = 1516) on hospitalized patients with GPP (4.2% died over a 10-year period).5Miyachi H. Konishi T. Kumazawa R. et al.Treatments and outcomes of generalized pustular psoriasis: a cohort of 1516 patients in a nationwide inpatient database in Japan.J Am Acad Dermatol. 2022; 86: 1266-1274https://doi.org/10.1016/j.jaad.2021.06.008Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar Limitations include potential GPP misclassification due to lack of standard case definition. Strengths of our study include the large national and representative GPP cohort, the longitudinal design, and the comparison with 2 control populations. Dr Schmitt-Egenolf is responsible for dermatology in the project management for the national guidelines for psoriasis at the Swedish Board of Health and Welfare. Drs Norlin and Löfvendahl have been involved in the health economic analyses of the national guidelines for psoriasis at the Swedish Board of Health and Welfare. Mr Gyllensvärd is an employee of Boehringer Ingelheim AB, Sweden. Drs Schmitt-Egenolf, Norlin, and Löfvendahl and Mr Gyllensvärd have no conflicts of interest to declare.

Background Generalized pustular psoriasis (GPP) is a severe form of pustular psoriasis with generalized eruption of sterile pustules, often along with systemic symptoms. There is a scarcity of population-based estimates of GPP prevalence and incidence. Objectives To estimate (i) the prevalence and incidence of GPP in the Swedish general population and (ii) the prevalence of psoriasis vulgaris within the GPP population. Methods We identified cases (2004-2015) with one ICD-10 diagnostic code (base case) for GPP within the Swedish National Patient Register, which covers inpatient and outpatient secondary care. Cases were linked to the Swedish Total Population Register, and point prevalence was estimated as on 31 December 2015. In two alternative analyses we changed case definitions to: (i) requiring two visits (strict case 1) and (ii) requiring two visits of which one was within dermatology/internal medicine (strict case 2). Results The base case point prevalence of GPP was estimated at 9.1 per 100 000 (women, 11.2; men, 7.0) and the annual prevalence in 2015 was estimated at 1.53 per 100 000. Among the GPP population, 43% also had a psoriasis vulgaris code. The incidence of GPP in 2015 was estimated at 0.82 per 100 000 (women, 0.93; men, 0.74). The criteria used had an impact on prevalence and incidence estimates: prevalence strict case 1 gave 3.8 per 100 000 and incidence strict case 1 gave 0.42 per 100 000. Conclusions Results indicate that the estimated GPP population in Sweden is within the range of previous published estimates. However, estimates were sensitive to the GPP case criteria used. The findings enhance demands for studies using validated diagnostic algorithms.

British Journal of DermatologyAccepted Articles PERSPECTIVES Severity of psoriasis – Time to disentangle severity from symptom control Kirk Geale, Kirk Geale orcid.org/0000-0001-7241-8471 Dermatology, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden Quantify Research, Stockholm, Sweden Contribution: Conceptualization (equal), Writing - original draft (equal), Writing - review & editing (equal)Search for more papers by this authorMarcus Schmitt-Egenolf, Corresponding Author Marcus Schmitt-Egenolf marcus.schmitt-egenolf@umu.se orcid.org/0000-0002-3858-8474 Dermatology, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden Correspondence Marcus Schmitt-Egenolf Email: marcus.schmitt-egenolf@umu.se Contribution: Conceptualization (equal), Writing - original draft (equal), Writing - review & editing (equal)Search for more papers by this author Kirk Geale, Kirk Geale orcid.org/0000-0001-7241-8471 Dermatology, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden Quantify Research, Stockholm, Sweden Contribution: Conceptualization (equal), Writing - original draft (equal), Writing - review & editing (equal)Search for more papers by this authorMarcus Schmitt-Egenolf, Corresponding Author Marcus Schmitt-Egenolf marcus.schmitt-egenolf@umu.se orcid.org/0000-0002-3858-8474 Dermatology, Department of Public Health and Clinical Medicine, Umeå University, Umeå, Sweden Correspondence Marcus Schmitt-Egenolf Email: marcus.schmitt-egenolf@umu.se Contribution: Conceptualization (equal), Writing - original draft (equal), Writing - review & editing (equal)Search for more papers by this author First published: 19 January 2022 https://doi.org/10.1111/bjd.21023 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi:10.1111/bjd.21023 AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future. RelatedInformation