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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: The EQ VAS component of the EQ-5D questionnaire has been used to assess patients' valuation of their own health besides its use for self-reporting of overall health status. The objective of the present study was to identify patients' valuation of EQ-5D-3L health states using the EQ VAS in different patient groups over time and in comparison to the general population. Methods: Data were obtained from patients from nine National Quality Registers (n = 172,070 patients) at baseline and at 1-year follow-up and compared with data from the general population (n = 41,761 participants). The correlation between EQ VAS scores and EQ-5D-3L index based on the Swedish experience-based VAS value set was assessed. Ordinary least squares (OLS) regression models were used to determine the association between EQ-5D-3L dimensions and EQ VAS valuation. Results: EQ VAS scores showed consistency with severity of health states both at baseline and at 1-year follow-up in the nine selected EQ-5D-3L health states. The regression models showed mostly consistent decrements by severity levels in each dimension at both time points and similar to the general population. The dimension mainly associated with inconsistency was the self-care severity level three. Problems in the anxiety/depression dimension had the largest impact on overall health status in most of the patient groups and the general population. Conclusion: The study has demonstrated the important role EQ VAS can play in revealing patients' valuation of their health and showed the variation in valuation of EQ-5D-3L dimensions and levels of severity across different patient groups. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Https://doi.org/10.1093/bjd/ljad134 Dear Editor, Palmoplantar pustulosis (PPP), a chronic relapsing skin condition characterized by pustules on the palms and soles, is often seen with psoriasis vulgaris (PV).[1],[2] Smoking is a risk factor.[2] PPP has been associated with several comorbidities, including thyroid disease, metabolic disease, atopy, arthritis and depression.[3],[4] However, large population-based studies with adequate control groups are lacking. Graph: Figure 1 (a) Prevalence of selected comorbidities in patients with palmoplantar pustulosis (PPP) and matched controls from the general population and patients with psoriasis vulgaris (PV). Although patients with PPP and PV showed similar comorbidity burdens overall, the comorbidity profiles differed significantly. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

36836227 7 Kessler R., Philipp J., Wilfer J., Kostev K. Predictive Attributes for Developing Long COVID-A Study Using Machine Learning and Real-World Data from Primary Care Physicians in Germany. Evidence-based medicine was in the past primarily based on the (meta-)analysis of randomized clinical trials (RCTs). RWE data are unlike RCT data in that they are often not obtained in a protocol-driven way and can be difficult to analyze with traditional statistical methods due to their structural diversity. [Extracted from the article]

Summary: 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. [ABSTRACT FROM AUTHOR]

Background: Generalized pustular psoriasis (GPP), which can occur with or without psoriasis vulgaris (PV), is a severe form of pustular psoriasis with potentially life-threatening symptoms. GPP is also associated with several comorbidities, which further adds to the burden of disease. This study investigates the economic burden of disease in patients with GPP. Methods: All-cause and GPP-specific healthcare resource use (inpatient stays, physician visits and drug use), as well as associated costs, were compared for year 2015 between GPP patients (n = 914) and two matched control groups representing the general population (n = 4047) and patients with PV but no GPP (n = 2556). Information on resource use for 2015 was obtained from the Swedish National Patient Register and Swedish Prescribed Drug Register, respectively. Results: All-cause inpatient stays, physician visits, and use of psoriasis-related drugs were significantly more common among GPP patients compared to both control groups. This difference was reflected in total direct cost for GPP patients (5062 euros/year) which was 3.1 and 1.8 times higher (p < 0.001) compared to the general population and PV controls, respectively. For GPP patients, the share of total cost was 22% for all-cause physician outpatient visits and 40% for all-cause inpatient stays. However, only 6.3% and 11.3% of these costs, respectively, were due to GPP-specific problems. Psoriasis-related drugs constituted 27% of total costs for GPP patients of which a large fraction (86%) was represented by biologics. Conclusion: This study demonstrates a higher economic burden for GPP patients compared to both the general population and patients with PV, with inpatient visits and use of biologic drugs as major cost driving factors. Only fractions of the costs for physician visits and inpatient stays were attributable to specific GPP problems, indicating a higher economic burden of GPP-consequences and complications. [ABSTRACT FROM AUTHOR]

Purpose: Evidence is insufficient to infer whether topical calcineurin inhibitors (TCIs; tacrolimus and pimecrolimus) cause malignancy. The study objective was to estimate the long-term risk of skin cancer and lymphoma associated with topical TCI use in adults and children, separately. Patients and Methods: A cohort study in Denmark, Sweden, UK, and the Netherlands was conducted. Adjusted incidence rate ratios (IRRs) and 95% confidence intervals (CIs) were calculated for nonmelanoma skin cancer (NMSC), melanoma, cutaneous T-cell lymphoma (CTCL), non-Hodgkin lymphoma (NHL) excluding CTCL, and Hodgkin lymphoma (HL) in new users of TCIs versus users of moderate/high-potency topical corticosteroids. Results: The study included 126,908/61,841 adults and 32,605/27,961 children initiating treatment with tacrolimus/pimecrolimus, respectively. Follow-up was ≥ 10 years for 19% of adults and 32% of children. Incidence rate ratios and (95% confidence intervals) for tacrolimus versus corticosteroid users in adults were < 1 for melanoma, non-Hodgkin lymphoma, and Hodgkin lymphoma; and 1.80 (1.25– 2.58) for cutaneous T-cell lymphoma. For pimecrolimus, IRRs in adults were < 1 for non-Hodgkin lymphoma, cutaneous T-cell lymphoma, and Hodgkin's lymphoma; and 1.21 (1.03– 1.41) for melanoma; and 1.28 (1.20– 1.35) for nonmelanoma skin cancer. In children, results were inconclusive due to few events. In adults, incidence rate ratios ≥ 5 years after first topical calcineurin inhibitor exposure were not higher than in overall analyses. Conclusion: Overall, we found little evidence associating use of topical calcineurin inhibitors with skin cancer and lymphoma; confounding by indication, surveillance bias, and reverse causation may have influenced these results. Even if causal, the public health impact of these excess risks would be low and confined to the first years of exposure. [ABSTRACT FROM AUTHOR]

Studies of the effects of bariatric surgery on psoriasis are few, with conflicting results. By linking the Swedish National Register for Systemic Treatment of Psoriasis (PsoReg) with the Scandinavian Obesity Surgery Registry (SOReg), individuals with psoriasis who had undergone bariatric surgery in Sweden during 2008 to 2018 were identified, and matched with data for patients with psoriasis in PsoReg. Psoriasis Area Severity Index (PASI) and Dermatology Life Quality Index (DLQI) were compared between the groups. Altogether, 50 operated individuals (median body mass index (BMI) 38.7 kg/m2) and 91 non-operated individuals (median BMI 33.0 kg/m2) were included. Control of disease at baseline was good in both groups. Linear mixed models showed no significant difference in psoriasis disease burden, measured as changes in mean PASI (ΔPASI) (-1.2, p = 0.43) and DLQI (ΔDLQI) (-2.2, p = 0.34). In summary, this study demonstrated no significant effect of bariatric surgery on psoriasis disease burden in patients with relatively well-controlled moderate to severe psoriasis. [ABSTRACT FROM AUTHOR]

Abstract Background Hypohidrotic ectodermal dysplasia (HED) is a genetic disorder characterized by defective development of teeth, hair, nails and eccrine sweat glands. Both autosomal dominant and autosomal recessive forms of HED have previously been linked to mutations in the ectodysplasin 1 anhidrotic receptor (EDAR) protein that plays an important role during embryogenesis. Methods The coding DNA sequence of the EDAR gene was analyzed in two large Swedish three-generational families with autosomal dominant HED. Results A non-sense C to T mutation in exon 12 was identified in both families. This disease-specific mutation changes an arginine amino acid in position 358 of the EDAR protein into a stop codon (p.Arg358X), thereby truncating the protein. In addition to the causative mutation two polymorphisms, not associated with the HED disorder, were also found in the EDAR gene. Conclusion The finding of the p.Arg358X mutation in the Swedish families is the first corroboration of a previously described observation in an American family. Thus, our study strengthens the role of this particular mutation in the aetiology of autosomal dominant HED and confirms the importance of EDAR for the development of HED.

Summary: Background: Most anti‐tumour necrosis factor (anti‐TNF) agents are transferred across the placenta and may increase paediatric susceptibility to infections. Aims: To assess the risk of paediatric infections after maternal anti‐TNF treatment. Methods: Population‐based cohort study in Denmark, Finland and Sweden 2006‐2013 in which 1027 children born to women with rheumatoid arthritis, psoriasis, psoriatic arthritis, ankylosing spondylitis or inflammatory bowel disease, treated with anti‐TNF, and 9346 children to women with non‐biologic systemic treatment, were compared to 1 617 886 children of the general population. Children were followed for 3 years. Results: Adjusted by maternal age, parity, smoking, body mass index, country and calendar year, the incidence rate ratios with 95% confidence interval (CI) for hospital admissions for infection in the first year were 1.43 (1.23‐1.67) for anti‐TNF and 1.14 (1.07‐1.21) for non‐biologic systemic treatment, and 1.29 (1.11‐1.50) and 1.09 (1.02‐1.15), respectively, when additionally adjusting for adverse birth outcomes. There was a slight increase in antibiotic prescriptions in the second year for anti‐TNF, 1.19 (1.11‐1.29), and for non‐biologic systemic treatment, 1.10 (1.07‐1.13). There was no difference among anti‐TNF agents, treatment in the third trimester, or between mono/combination therapy with non‐biologic systemic treatment. Conclusions: Both anti‐TNF and non‐biologic systemic treatment were associated with an increased risk of paediatric infections. However, reassuringly, the increased risks were present regardless of treatment in the third trimester, or with combination treatment, and were not persistent during the first 3 years of life. Our findings may indicate a true risk, but could also be due to unadjusted confounding by disease severity and healthcare‐seeking behaviour. This may in turn shift the risk‐benefit equation towards continuation of treatment even in the third trimester. [ABSTRACT FROM AUTHOR]

Purpose To study the risk of preterm birth, caesarean section, and small for gestational age after anti‐tumor necrosis factor agent treatment (anti‐TNF) in pregnancy. Methods: Population‐based study including women with inflammatory bowel disease, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, and psoriasis, and their infants born 2006 to 2013 from the national health registers in Denmark, Finland, and Sweden. Women treated with anti‐TNF were compared with women with nonbiologic systemic treatment. Adalimumab, etanercept, and infliximab were compared pairwise. Continuation of treatment in early pregnancy was compared with discontinuation. Odds ratios with 95% confidence intervals were calculated in logistic regression models adjusted for country and maternal characteristics. Results: Among 1 633 909 births, 1027 infants were to women treated with anti‐TNF and 9399 to women with nonbiologic systemic treatment. Compared with non‐biologic systemic treatment, women with anti‐TNF treatment had a higher risk of preterm birth, odds ratio 1.61 (1.29‐2.02) and caesarean section, 1.57 (1.35‐1.82). The odds ratio for small for gestational age was 1.36 (0.96‐1.92). In pairwise comparisons, infliximab was associated with a higher risk of severely small for gestational age for inflammatory joint and skin diseases but not for inflammatory bowel disease. Discontinuation of anti‐TNF had opposite effects on preterm birth for inflammatory bowel disease and inflammatory joint and skin diseases. Conclusions: Anti‐TNF agents were associated with increased risks of preterm birth, caesarean section, and small for gestational age. However, the diverse findings across disease groups may indicate an association related to the underlying disease activity, rather than to agent‐specific effects. [ABSTRACT FROM AUTHOR]

Background: Assessing the impact of disease severity on generic quality of life (QOL) is a critical step in outcomes research and in the development of decision-analytic models structured around health states defined by clinical measures. While data from routine clinical practice found in healthcare registers are increasingly used for research, more attention should be paid to understanding the relationship between clinical measures of disease severity and QOL. The purpose of this work was therefore to investigate this relationship in psoriasis using a population-based dataset.Methods: Severity was measured by the Psoriasis Area and Severity Index (PASI), which combines severity of erythema, induration, and desquamation into a single value ranging from 0 to 72. The generic EQ-5D-3L utility instrument, under the UK tariff, was used to measure QOL. The association between PASI and EQ-5D-3L was estimated using a population-based dataset of 2674 patients with moderate to severe psoriasis enrolled over ten years in the Swedish psoriasis register (PsoReg). Given the repeated measurement of patients in the register data, a longitudinal fixed-effects model was employed to control for unobserved patient-level heterogeneity.Results: Marginal changes in PASI are associated with a non-linear response in EQ-5D-3L: Moving from PASI 10 to 9 (1 to 0) is associated with an increase of 0.0135 (0.0174) in EQ-5D-3L. Furthermore, unobserved patient-level heterogeneity appears to be an important source of confounding when estimating the relationship between QOL and PASI.Conclusions: Using register data to estimate the impact of disease severity on QOL while controlling for unobserved patient-level heterogeneity shows that PASI appears to have a larger impact on QOL than previously estimated. Routine collection of generic QOL data in registers should be encouraged to enable similar applications in other disease areas.Trial Registration: Not applicable. [ABSTRACT FROM AUTHOR]

Definitions of severity are important to patient wellbeing because they play a central role in framing treatment goals and determining eligibility for systemic treatment. If a patient experiences complete resolution of his or her severe symptoms, using current definitions, how should we classify this patient's psoriasis severity? An extension of the language used to describe patients' disease activity and presentation is an important step in framing psoriasis and its treatment, ultimately empowering patients, payers and society. [Extracted from the article]

Background: Clinical studies of continuous versus intermittent biologic therapy for moderate to severe psoriasis demonstrate improved efficacy with continuous treatment. Objective: To analyse Swedish real-world data of continuously and intermittently treated biologic-naïve patients after switching to a biologic agent. Methods: This is an observational study based on PsoReg, the Swedish registry for systemic psoriasis treatment. Outcome effects in biologic-naïve patients who switched to a biologic agent (n = 351) were analysed in groups of continuous, intermittent and terminated treatment. Results: Intermittently treated patients (n = 50) reported higher Psoriasis Area and Severity Index and Dermatology Life Quality Index values after switching than patients with continuous (n = 260) or terminated treatment (n = 41). Study Limitations: The reason for intermittent treatment was not recorded. The intermittently treated patients may be a heterogeneous group and a limitation is that it cannot be determined whether less than continuous use was offered to handle negative aspects. Conclusion: Patients with continuous biologic treatment tend to achieve better outcomes compared to intermittently treated patients. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

The introduction of biologics has changed treatment patterns as well as costs in patients with psoriasis. This study was performed to estimate direct and indirect costs of the psoriasis population in Sweden, and to analyse changes in costs between 2006 and 2009. The study population was identified in national registers. Direct costs included health care visits with primary psoriasis diagnoses in specialist care and drugs relevant for treating psoriasis. Productivity loss, including costs of long-term sick leave and disability pension, was estimated as the difference between psoriasis patients and matched controls from the general population. Total direct cost increased from SEK 348 million (~ €39) in 2006 to SEK 459 million (~ €51) in 2009, whereas the total productivity loss decreased from SEK 1,646 (~ €183) to 1,618 million (~ €180) between 2006 and 2009. Although direct costs, especially for biologic agents, have increased for patients with psoriasis over time, this study indicates that costs related to productivity loss are still more substantial. [ABSTRACT FROM AUTHOR]

Background Mutations in the EDAR-gene cause hypohidrotic ectodermal dysplasia, however, the oral phenotype has been described in a limited number of cases. The aim of the present study was to clinically describe individuals with the c.1072C > T mutation (p. Arg358X) in the EDAR gene with respect to dental signs and saliva secretion, symptoms from other ectodermal structures and to assess orofacial function. Methods Individuals in three families living in Sweden, where some members had a known c.1072C > T mutation in the EDAR gene with an autosomal dominant inheritance (AD), were included in a clinical investigation on oral signs and symptoms and self-reported symptoms from other ectodermal structures (n = 37). Confirmation of the c.1072C > T mutation in the EDAR gene were performed by genomic sequencing. Orofacial function was evaluated with NOT-S. Results The mutation was identified in 17 of 37 family members. The mean number of missing teeth due to agenesis was 10.3 ± 4.1, (range 4-17) in the mutation group and 0.1 ± 0.3, (range 0-1) in the non-mutation group (p < 0.01). All individuals with the mutation were missing the maxillay lateral incisors and one or more of the mandibular incisors; and 81.3% were missing all four. Stimulated saliva secretion was 0.9 ± 0.5 ml/min in the mutation group vs 1.7 ± 0.6 ml/min in the non-mutation group (p < 0.01). Reduced ability to sweat was reported by 82% in the mutation group and by 20% in the non-mutation group (p < 0.01). The mean NOT-S score was 3.0 ± 1.9 (range 0-6) in the mutation group and 1.5 ± 1.1 (range 0-5) in the nonmutation group (p < 0.01). Lisping was present in 56% of individuals in the mutation group. Conclusions Individuals with a c.1072C > T mutation in the EDAR-gene displayed a typical pattern of congenitally missing teeth in the frontal area with functional consequences. They therefore have a need for special attention in dental care, both with reference to tooth agenesis and low salivary secretion with an increased risk for caries. Sweating problems were the most frequently reported symptom from other ectodermal structures. [ABSTRACT FROM AUTHOR]

We aimed to describe and compare patients diagnosed with malignant melanoma (MM), depending on their initial contact with care and with regard to age, sex, and MM type and thickness, and to explore pathways and time intervals (lead times) between clinics from the initial contact to diagnosis and treatment. The sample from northern Sweden was identified via the Swedish melanoma register. Data regarding pathways in health care were retrieved from patient records. In our unselected population of 71 people diagnosed with skinmelanoma of SSM and NM types, 75%of patients were primarily treated by primary health-care centres (PHCs). The time interval (delay) from primary excision until registration of the histopathological assessment in the medical records was significantly longer in PHCs than in hospital-based and dermatological clinics (Derm). Thicker tumors were more common in the PHC group. Older patients waited longer times for wide excision. Most MM are excised rapidly at PHCs, but some patients may not be diagnosed and treated in time. Delay of registration of results from histopathological assessments within PHCs seems to be an important issue for future improvement. Exploring shortcomings in MM patients' clinical pathways is important to improve the quality of care and patient safety. [ABSTRACT FROM AUTHOR]

Objectives: Moderate to severe psoriasis, once regarded as merely a skin disease, is today seen as an inflammatory systemic disease. The sex ratio of the prevalence of psoriasis is balanced. In recent years several reports have documented that men receive more systemic or UV treatment than women, and different hypotheses were made. In PsoReg, the national registry for systemic treatment of psoriasis in Sweden, we have, like other European registries, observed a predominance of men (59%), especially of men treated with biologics (63%). Biologics are a relatively new group of very effective but high-priced drugs. The objective of this study was to analyse if women are discriminated by not having the same access to the high-priced biologics. Design: Population based cohort study using data from a nationwide quality register of psoriasis patients. Population: 2294 patients with moderate to severe psoriasis receiving systemic treatment from a specialist in dermatology. Main Outcome Measures: Time to initiation of biologic treatment. A multiple Cox proportional hazard’s regression was performed, with time to initiating a biologic treatment as the outcome in order to assess the independent role of the patient’s sex in initiating such therapy. The psoriasis severity was defined as a time-varying variable. Results: Men had more severe psoriasis than women according to the Psoriasis Area and Severity Index (PASI), regardless of age at enrolment, and throughout the study period. The analysis in the multiple Cox regression show that age, psoriasis severity and psoriasis arthropathy were relevant factors for initiating biologic therapy, whereas sex is not. Conclusions: Although as many women as men are believed to suffer from psoriasis, men seem to be more severely affected by psoriasis. The asymmetry in allocation of biologic therapy thereby probably reflects the differing disease activity between the sexes, and is not a discrimination against women per se. [ABSTRACT FROM AUTHOR]

Background: Although clinical studies have shown efficacy of biological agents in moderate to severe psoriasis, observational studies of real-world effectiveness are rare. Objective: To analyse the psoriasis area and severity index (PASI) and quality of life by the EQ-5D questionnaire and dermatology quality of life index (DLQI) in psoriasis patients who switched from conventional systemic treatment to biological agents in clinical practice. Furthermore, to analyse patient groups with the highest benefit of biological agents. Methods: Longitudinal, observational study based on the Swedish National Registry for Systemic Treatment of Pso-riasis, PsoReg. Outcomes of biological-naïve patients who switched to a biological agent (n = 267) were analysed before switch and at the first follow-up. Results: Patients significantly improved in EQ-5D, DLQI and PASI (p < 0.001). Patients with DLQI ≥10 and/or PASI ≥10 had the greatest benefits from biological agents in terms of EQ-5D. Conclusions: Patients with moderate to severe psoriasis benefit from biological agents in clinical practice; the patients with the highest benefits were those with high pretreatment PASI and DLQI scores. Copyright © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

With the introduction of new systemic drugs for the management of psoriasis, we felt an obligation in Sweden to establish a trusted tool to monitor their use. We formed PsoReg to create a solid, long-term database in order to analyze safety and effectiveness of different systemic psoriasis treatment regimens. PsoReg will provide information to help clinicians individualize therapy on a rational basis through evaluation of effectiveness and adverse effects in specific patient subgroups. Designed and managed by specialized health care professionals, PsoReg will enroll all psoriasis patients on systemic treatment to allow a fair comparison of old versus new-generation psoriasis treatments. PsoReg will even create benchmark data for quality assurance of the medical service. A web-based design allows real-time pharmacovigilance and enables the registry to assist clinicians in their day-to-day management of psoriasis patients. In this way PsoReg can become an integrated part of tomorrow’s dermatology. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: Hypohidrotic ectodermal dysplasia (HED) is a genetic disorder characterized by defective development of teeth, hair, nails and eccrine sweat glands. Both autosomal dominant and autosomal recessive forms of HED have previously been linked to mutations in the ectodysplasin 1 anhidrotic receptor (EDAR) protein that plays an important role during embryogenesis. Methods: The coding DNA sequence of the EDAR gene was analyzed in two large Swedish threegenerational families with autosomal dominant HED. Results: A non-sense C to T mutation in exon 12 was identified in both families. This diseasespecific mutation changes an arginine amino acid in position 358 of the EDAR protein into a stop codon (p.Arg358X), thereby truncating the protein. In addition to the causative mutation two polymorphisms, not associated with the HED disorder, were also found in the EDAR gene. Conclusion: The finding of the p.Arg358X mutation in the Swedish families is the first corroboration of a previously described observation in an American family. Thus, our study strengthens the role of this particular mutation in the aetiology of autosomal dominant HED and confirms the importance of EDAR for the development of HED. [ABSTRACT FROM AUTHOR]

Although the pathogenesis of psoriasis is still a matter of debate, there are several lines of evidence supporting the concept of this disease being immunologically mediated with T cells playing a crucial role. Because a considerable portion of the cellular infiltrate in psoriasis consists of activated T-helper cells, expression of HLA class II antigens might be of particular importance for the understanding of its pathogenesis. Therefore, we investigated the HLA type of patients with type I (early onset, positive family history) and type (late onset, no family history) psoriasis by means of serology (n= 89) and genotyping using sequence-specific oligonueleotide probes (n = 64). Serologic analysis of class I documented the association of type I psoriasis with HLA-Cw6, -B13, and -B57, whereas type II psoriasis showed a weaker correlation with HLA-Cw2 and -B27. Genotyping using SSO for class II detected the elevation of the HLA- DRB1*0701/2 allele frequency from 13% in normal population to 36% in type I, but only to 15% in type II psoriatics. Moreover, positive correlations with type I psoriasis were detected for HLA-DQA1*0201 and HLA-DQB1*0303. The HLA-DRB1*0701/2, -DQA1*0201,-DQB1*0303 extended haplotype was found exclusively in type I psoriasis. This is the first report documenting the association of distinct HLA class II alleles with type I psoriasis as detected on the DNA level, an approach both more specific and more sensitive when compared to serology. [ABSTRACT FROM AUTHOR]

In nonpustular psoriasis, principally two forms can be distinguished [Christophers E, Henseler T: Patient subgroups and the inflammatory pattern in psoriasis. Acta Dermatol Venercol 69(suppl 151):88-92, 1989): Type I frequently shows positive family history, linkage disequilibrium for human leucocyte antigens (HLAs) Cw6, B13 and Bw57 as well as an early onset. Type II manifests itself around the 5th decade, it is more frequently than normal associated with Cw2 and B27. In the light of this association with HLAs an autoimmune pathogenesis has been discussed. In order to investigate the pathogenetic function of T cells we obtained biopsies from patients with type I(n = 10) and type II (n = 10) psoriasis. Three-step peroxidase staining was performed using panel of monoclonal antibodies directed against five variable (V) regions of the β chain (Vβ5a, Vβ5b, Vβ6, Vβ8, Vβ12) and one of the α chain (Vα2) of the T cell receptor (TCR). Positive or negative selection of a particular TCR V region could not be detected in the demonstrable repertoire. Furthermore, the usage of the V regions under investigation revealed a similar pattern in the two forms of psoriasis. [ABSTRACT FROM AUTHOR]

Background: Hidradenitis suppurativa (HS) is an inflammatory and chronic-recurrent disease of the hair follicle. Its aetiopathogenesis is not completely known. Although bacterial colonization and superinfection are clinically relevant, lymph node involvement has rarely been studied. Objectives: In this pilot retrospective study, we evaluated the clinical and microbiological nodal involvement in patients with axillary hidradenitis suppurativa. Materials and methods: We retrospectively analyzed patients suffering from axillary hidradenitis suppurativa and referred to the Dermatology Unit of the Foundation Ca' Granda Ospedale Maggiore Policlinico in Milan between October 2018 and November 2019. The sampling procedure took place during the surgical excision of lesions at the Operative Unit of Plastic and Reconstructive Surgery of Policlinico San Donato Hospital. Three types of sample were sent to microbiological analysis: exudate swab, axillary lymph node, fistula. Result: In total, we recruited 10 patients. Two of them underwent bilateral axillary surgery. Nine lymph nodes were analyzed. In one patient, bacterial culture in lymph nodes, skin lesions and fistulae matched. Conclusions: Unequivocable conclusions cannot be drawn due to the low number of patients. Further studies are necessary to confirm the preliminary results of our retrospective pilot study. [ABSTRACT FROM AUTHOR]

Psoriasis is a chronic, immune-mediated inflammatory disease that predominantly affects the skin and joints. The recent therapeutic development for psoriasis has been remarkable and biologics have dramatically changed the treatment of psoriasis. In moderate-to-severe cases, systemic therapies are required to control their symptoms and biologics can provide greater efficacy when compared with other types of therapies. The coronavirus disease (COVID-19) pandemic has had a great impact on the lives of many people and has worsened substantially worldwide. During the ongoing COVID-19 pandemic, it still remains unclear whether biologics suppress the immune system and increase the risk of COVID-19. In this review, we have summarized the experience with biologics used for treating psoriasis during the COVID-19 pandemic. Biologics seem to be beneficial to COVID-19 infection. Shared decision-making that is based on updated information is highlighted in the time of COVID-19. [ABSTRACT FROM AUTHOR]

Psoriasis is an immune-mediated, chronic inflammatory disease, which mainly affects the skin, although it has systemic pathological effects. Comorbidities of psoriasis include ocular disorders, which are often nonspecific or mildly symptomatic. The aim of this study was to show the importance of ocular-disease screening in psoriatic patients using the Ocular Manifestations in Psoriasis Screening (OcMaPS) questionnaire. Patients suffering from moderate-to-severe psoriasis referring at our outpatient-clinic were consecutively enrolled. Each patient was asked to complete a screening questionnaire (OcMaPS). Patients reporting ocular symptoms were referred for an ophthalmological examination. A total of 372 patients were enrolled in the study. Ocular symptoms were detected in 39 patients (10.5%), and 37 patients were referred to ophthalmological examination which confirmed the presence of ocular manifestation in 30 patients. There were three cases (10%) of uveitis, 14 (46.6%) of dry eye and 13 (43.3%) of cataract, in progress or already treated with surgery. In the remaining seven patients, no ocular manifestations were found. Ocular manifestations in psoriatic patients are not rare. It is important to be aware of ocular symptoms in psoriatic patients, screening patients (with a consultation or OcMaPS questionnaire), which leads to earlier diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Psoriasis is a chronic, inflammatory skin disease affecting 2–4% of the general population. Accompanying subjective symptoms (pruritus or pain) may cause decreased life quality including sleep disorders (SD). Sixty psoriatic patients fulfilled the following questionnaires: Pittsburgh Sleep Quality Index (PSQI), STOP BANG for the obstructive sleep apnea syndrome (OSAS) assessment, and Restless Legs Syndrome (RLS) Severity Scale. Patients' laboratory and clinical data were also investigated. All data obtained were compared to 40 participants without dermatoses. Mean PSQI, risk of OSAS, and RLS severity of psoriatics were significantly higher than in controls (p < 0.0001, p < 0.05, p < 0.05 respectively). There was a positive correlation between the time of suffering from psoriasis and the risk of OSAS (R = 0.286, p < 0.05). We did not observe any significant relationship between PSQI, risk of OSAS, or RLS and psoriasis severity assessed with PASI (Psoriasis Area and Severity Index). We identified four possible predictors of RLS: glucose, CRP and total cholesterol concentrations, and PSQI. SD are significantly more frequent in psoriatics than in people without chronic dermatological diseases but surprisingly they are not correlated with psoriasis severity. SD decrease patients' life quality and may result in serious consequences. Therefore, establishing recommendations concerning screening for SD and their predictors should be considered. [ABSTRACT FROM AUTHOR]