The Global Hidradenitis Suppurativa Atlas Methodology: Combining Global Proportions in a Pooled Analysis.

INTRODUCTION: Data concerning the global burden of hidradenitis suppurativa (HS) are limited. Reported prevalence estimates vary between 0.0003% and 4.1%, and data from various geographical regions are still to be collected. Previously reported prevalences have been limited by the methodological approach and source of data. This has resulted in great heterogeneity as prevalence data from physician-diagnosed cases poorly match those of self-reported apparent HS disease. METHODS: The Global Hidradenitis Suppurativa Atlas (GHiSA) introduces an innovative approach to determine the global prevalence of HS. This approach involves using a previously validated questionnaire to screen apparently healthy adults accompanying a patient to a non-dermatological outpatient clinic visit in a hospital or a private/family medicine clinic. The screening questionnaire (i.e., the index test) is combined with a subsequent physician-based in-person validation (i.e., the reference standard) of the participants who screen positive. Approximately ten percent of the screen-negative participants are also clinically assessed to verify the diagnostic precision of the test. The local prevalence (pi) will be estimated from each country that submits the number of patients who are HS positive according to the index test and clinical examination (n), and the corresponding total number of observations (N). CONCLUSION: The GHiSA Global Prevalence studies are currently running simultaneously in 58 countries across six continents (Africa, Europe, Australia, North America, South America, and Asia). The goal of the combined global proportion is the generation of a single summary (i.e., proportional meta-analysis), which will be done after a logit transformation and synthesized using a random-effects model. The novel standardization of the Global Prevalence Studies conducted through GHiSA enables direct international comparisons, which were previously not possible due to substantial heterogeneity in past HS prevalence studies.

Swiss (German) Version of the Actinic Keratosis Quality of Life questionnaire.

BACKGROUND: Actinic keratosis (AK) is a sun-induced skin lesion that may progress to invasive squamous cell carcinoma of the skin. Recently, the Actinic Keratosis Quality of Life questionnaire (AKQoL) was designed for patients with AK in Denmark as a specific quality of life instrument for AK patients. OBJECTIVE: The objective of this study was to adapt the AKQoL for the German language region of Switzerland and to evaluate its psychometric properties (validity, reliability). METHODS: Translation and cultural adaptation of the questionnaire were assessed by using the technique of cognitive interviewing. During the translation process, 34 patients with AK from the Department of Dermatology, University Hospital Zurich, were interviewed in 3 sessions of cognitive interviewing. The translated questionnaire was then distributed together with the Dermatology Life Quality Index (DLQI) to a second group of 113 patients for validation and reliability testing. Within this group, we measured the internal consistency by the Cronbach coefficient α and Spearman correlation coefficient between the AKQoL and the DLQI. RESULTS: The problems encountered during the translation process led to changes in 5 categories as described by Epstein: stylistic changes, change in breadth, change in actual meaning, change in frequency and time frame, change in intensity. We found a Cronbach α of 0.82, an acceptable internal consistency. The Spearman correlation coefficient between total scores of AKQoL and DLQI was 0.57. CONCLUSION: We culturally adapted and validated a Swiss (German) version of the AKQoL questionnaire applicable for the population of a university center in Switzerland to measure and monitor the quality of life in patients with AK.

Disutility in Patients with Hidradenitis Suppurativa: A Cross-sectional Study Using EuroQoL-5D.

Disutility reflects the disability caused by a disease. The EuroQoL-5D (EQ-5D) questionnaire is a measure of health-related overall utility. The questionnaire has only been applied previously to a small number of patients with hidradenitis. In this study a survey of 421 patients with hidradenitis suppurativa was conducted using the EQ-5D questionnaire. Questions regarding pain, malodour and pruritus were included to determine quantitatively whether these factors are associated with low EQ-5D index and visual analogue scale (VAS) scores. The index and VAS scores obtained were compared with reference values for the general population in Denmark. A significantly decreased utility in patients with hidradenitis suppurativa was found for all age group levels, except for 65-74-year-olds. The total index score in the cohort was 0.705 (population mean 0.887) and the VAS was 62.25 (population mean 82.6). Multivariate analysis found significant associations between loss of utility and pain, malodour and pruritus (p < 0.0001). Patients with hidradenitis suppurativa had a significantly decreased EQ-5D compared with the background population. Malodour and pruritus were found to be associated with low index values, and pain and malodour with low VAS. Patient-reported pain and discomfort had the most negative overall effect on mean index scores.

Interpretation of the skin cancer quality of life score: a validated quality of life questionnaire for non-melanoma skin cancer.

BACKGROUND: Health-related quality of life (HRQoL) instruments are used increasingly. In order to assign clinical meaning to HRQoL scores, the interpretation of instruments is essential but lagging in dermatology. OBJECTIVE: To establish a clinical interpretation of the Skin Cancer Quality of Life questionnaire (SCQoL), a newly validated HRQoL instrument for patients with non-melanoma skin cancer (NMSC), using an anchor-based method, and to test the responsiveness. METHODS: Receiver-operating characteristic analysis was used to propose clinically meaningful cut-off scores for SCQoL including 101 patients with NMSC. RESULTS: The following bands were established: score 0-3 corresponds to no impairment, 4-6 corresponds to mild impairment, 7-10 to moderate impairment and 11-27 to severe impairment of HRQoL. Testing the responsiveness shows a moderate effect size and significantly lower scores only for the domain emotion and the global item. CONCLUSION: Using proposed clinical cut-off scores for SCQoL may help clinicians in their decision-making, help monitoring clinical improvement and classify patients just as e.g. the Dermatology Life Quality Index can.

Prevalence and Patient Characteristics of Ectodermal Dysplasias in Denmark.

Importance: Ectodermal dysplasias constitute a group of rare genetic disorders of the skin and skin appendages with hypodontia, hypotrichosis, and hypohidrosis as cardinal features. There is a lack of population-based research into the epidemiology of ectodermal dysplasias. Objective: To establish a validated population-based cohort of patients with ectodermal dysplasia in Denmark and to assess the disease prevalence and patient characteristics. Design, Setting, and Participants: This nationwide cohort study used individual-level registry data recorded across the Danish universal health care system to identify patients with ectodermal dysplasias from January 1, 1995, to August 25, 2021. A 3-level search of the Danish National Patient Registry and the Danish National Child Odontology Registry was conducted to identify patients with diagnosis codes indicative of ectodermal dysplasias; patients registered in the Danish RAREDIS Database, the Danish Database of Genodermatoses, and local databases were also added. The search results underwent diagnosis validation and review of clinical data using medical records. Of 844 patient records suggestive of ectodermal dysplasias, 791 patients (93.7%) had medical records available for review. Positive predictive values of the diagnosis coding were computed, birth prevalence was estimated, and patient characteristics were identified. Data analysis was performed from May 4 to December 22, 2023. Results: The identified and validated study cohort included 396 patients (median [IQR] age at diagnosis, 13 [4-30] years, 246 females [62.1%]), of whom 319 had confirmed ectodermal dysplasias and 77 were likely cases. The combined positive predictive value (PPV) for ectodermal dysplasia-specific diagnosis codes was 67.0% (95% CI, 62.7%-71.0%). From 1995 to 2011, the estimated minimum birth prevalence per 100 000 live births was 14.5 (95% CI, 12.2-16.7) for all ectodermal dysplasias and 2.8 (95% CI, 1.8-3.8) for X-linked hypohidrotic ectodermal dysplasias. A molecular genetic diagnosis was available for 241 patients (61%), including EDA (n = 100), IKBKG (n = 55), WNT10A (n = 21), TRPS1 (n = 18), EDAR (n = 10), P63 (n = 9), GJB6 (n = 9), PORCN (n = 7), and other rare genetic variants. Conclusions and Relevance: The findings of this nationwide cohort study indicate that the prevalence of ectodermal dysplasias was lower than previously reported. Furthermore, PPVs of the search algorithms emphasized the importance of diagnosis validation. The establishment of a large nationwide cohort of patients with ectodermal dysplasias, including detailed clinical and molecular data, is a unique resource for future research in ectodermal dysplasias.

Quality of life in non-melanoma skin cancer--the skin cancer quality of life (SCQoL) questionnaire.

BACKGROUND: Disease-specific quality of life (QoL) questionnaires are increasingly used to provide patient-reported out-come measures in both malignant and non-malignant disease. OBJECTIVE: To create, validate and test the psychometrics of the Skin Cancer Quality of Life (SCQoL), which was designed to measure health-related QoL in patients with non-melanoma skin cancer affecting any area and undergoing any therapy. METHODS AND MATERIALS: The SCQoL was developed in a stepwise approach. Three pilot studies (testing content and face validity) and psychometric testing (scale structure, reliability, domains and known-groups validity, concurrent and convergent validity) were conducted. Rasch analyses were performed on the final questionnaire. RESULTS: The initial 10-item questionnaire was reduced to 9 items following interviews and inter-item correlations. The nine item scale was confirmed by Item Response Theory (IRT) and internal consistency. Differential Item Functioning (DIF) was found for a single item, but the effect was small. CONCLUSION: The final 9-item SCQoL is unidimensional and consists of 3 domains covering function, emotions and control. Furthermore there is one single global item. The total score range from 0 to 27. Higher score denote a greater impairment of the QoL.

Darier's Disease Flare following COVID-19 Vaccine.

Darier's disease is a rare genodermatosis characterized clinically by dyskeratotic papules in the seborrheic and intertriginous areas and nail abnormalities. Dyskeratosis and acantholysis are typical histological findings. Darier's disease is not known to be inflammatory by nature as inflammation occurs primarily due to local infections, and it may therefore differ from inflammatory dermatoses such as psoriasis and cutaneous lupus in response to antigen stimulation. Known triggers of Darier's disease primarily include exogenous factors such as sun exposure, friction, or infection. We present a case of a 47-year-old white female with a flare of Darier's disease 2 days following her first vaccination with COVID-19 vaccine (ChAdOx1-s [recombinant]) (Vaxzevria® [previously known as COVID-19 vaccine AstraZeneca]). In this case report, we discuss possible mechanisms linking the vaccination and the flare of Darier's disease. We consider inflammatory mechanisms as well as a random co-occurrence. Due to the close time-related association between the disease flare and the COVID-19 vaccination, we find an urge to make other clinicians aware of a possible association.

Development of HiSQOL: A Hidradenitis Suppurativa-Specific Quality of Life Instrument.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic and painful skin disease. In addition, HS lesions may be associated with pus and odour, potentially leading to significant stigma and, consequently, greatly affected quality of life (QOL). QOL is a multidimensional construct, which can be measured in various ways. However, generic or dermatologic QOL measures may not capture changes in QOL particularly affected in HS. Accordingly, patients and experts included in the HIdradenitis SuppuraTiva cORe outcomes set International Collaboration (HISTORIC) agreed that future clinical HS trials should measure HS-specific QOL. OBJECTIVES: To develop an HS-specific QOL instrument (HiSQOL, Hidradenitis Suppurativa Quality of life). METHOD: The initial phases of the questionnaire development, described in this study, included item generation by patient interviews, development of a pilot questionnaire, questionnaire refinement, and pilot testing. RESULTS: For item generation, 21 patients were interviewed individually or in focus groups. Analysis of the interviews identified 105 candidate items and, next, a pilot questionnaire was developed. Finally, item reduction and two rounds of pilot testing resulted in a 23-item questionnaire representing physical, psychological, and social QOL dimensions. CONCLUSIONS: We have comprehensively explored on HS's possible effect on the QOL of the affected individuals and identified a 23-item HS-specific QOL questionnaire. The questionnaire proved to be feasible, acceptable, and comprehensible in the second round of pilot testing. With HiSQOL, researchers can measure HS-specific QOL in future clinical trials, potentially enabling them to discover more effective treatment options. It is envisaged, that after thorough validation in a trial setting, a streamlined version of HISQOL may also become available for clinical use in daily practice.

Hidradenitis Suppurativa is Associated with Higher Heart Rate but Not Atrial Fibrillation: A Comparative Cross-sectional Study of 462 Individuals with Hidradenitis Suppurativa in Denmark.

Hidradenitis suppurativa (HS) is a chronic inflammatory dermatological disease with inflammatory mechanisms overlapping those of psoriasis, and both diseases have been associated with cardiovascular risk factors i.e. smoking and metabolic syndrome. Two studies have recently linked psoriasis with Atrial Fibrillation (AF). AF is the most frequently occurring cardiac arrhythmia in the general population and is typically accompanied by increased heart rate. Both AF and heart rate are linked with inflammation. The aim of the study was to investigate a potential association between HS and increased heart rate as well as AF. We performed a comparative cross-sectional study using digital measurements of heart rate and resting 12-lead electrocardiography (ECG) in combination with self-reported information when diagnosing AF. Our study comprised 32 individuals with HS from the hospital (the hospital HS group), 430 from the general population HS group (the population HS group), and 20,780 controls. Age and sex adjusted analysis demonstrated a significantly higher heart rate in the HS groups vs. controls (15% (range: 8-23%) higher for the hospital HS group and 4% (2-5%) higher for the population HS group). We found no association between HS and AF (P=0.1670).

Differences in Disease-specific Quality of Life in Patients with Actinic Keratosis in Australia and Denmark.

Actinic keratosis (AK) negatively influences patient quality of life as measured by the disease-specific Actinic Keratosis Quality of Life (AKQoL) questionnaire. The quality of life in Australian patients was significantly less affected than in Danish patients. We hypothesize that general factors such as public awareness and cultural connotations of AK, may influence the impact of AK on quality of life (QoL).

The Danish Nonmelanoma Skin Cancer Dermatology Database.

AIM OF DATABASE: The Danish Nonmelanoma Skin Cancer Dermatology Database was established in 2008. The aim of this database was to collect data on nonmelanoma skin cancer (NMSC) treatment and improve its treatment in Denmark. NMSC is the most common malignancy in the western countries and represents a significant challenge in terms of public health management and health care costs. However, high-quality epidemiological and treatment data on NMSC are sparse. STUDY POPULATION: The NMSC database includes patients with the following skin tumors: basal cell carcinoma (BCC), squamous cell carcinoma, Bowen's disease, and keratoacanthoma diagnosed by the participating office-based dermatologists in Denmark. MAIN VARIABLES: Clinical and histological diagnoses, BCC subtype, localization, size, skin cancer history, skin phototype, and evidence of metastases and treatment modality are the main variables in the NMSC database. Information on recurrence, cosmetic results, and complications are registered at two follow-up visits at 3 months (between 0 and 6 months) and 12 months (between 6 and 15 months) after treatment. DESCRIPTIVE DATA: In 2014, 11,522 patients with 17,575 tumors were registered in the database. Of tumors with a histological diagnosis, 13,571 were BCCs, 840 squamous cell carcinomas, 504 Bowen's disease, and 173 keratoakanthomas. CONCLUSION: The NMSC database encompasses detailed information on the type of tumor, a variety of prognostic factors, treatment modalities, and outcomes after treatment. The database has revealed that overall, the quality of care of NMSC in Danish dermatological clinics is high, and the database provides the necessary data for continuous quality assurance.

A systematic literature review to compare quality of life in psoriasis with other chronic diseases using EQ-5D-derived utility values.

BACKGROUND: Psoriasis is a chronic, immune-mediated dermatological disease associated with substantial economic, clinical, and humanistic burden. OBJECTIVE: The aim of this study was to understand the disutility of patients with psoriasis vulgaris, using mean baseline EuroQoL five dimensions (EQ-5D) index scores reported in the published literature, and to compare this to patients with other chronic diseases. METHODS: Two systematic literature searches were conducted according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines. Searches were conducted in ten databases including Embase, MEDLINE, and the Cochrane Central Register of Controlled Trials (CENTRAL). The first search (December 2013) used search terms psoria* AND (EQ5D OR EQ OR EUROQoL). Only publications of original research, which reported baseline EQ-5D scores for mild/moderate/severe psoriasis, were included. The second search (March 2014) used the terms (systematic review) AND (EQ5D OR EQ 5D OR EuroQoL). Titles were screened by two independent reviewers. Four independent reviewers reviewed titles and full-length papers. EQ-5D scores for psoriasis patients were qualitatively compared with scores from patients with other chronic diseases identified through the literature search. RESULTS: Of 133 publications on psoriasis, 12 met the inclusion criteria. The mean EQ-5D index scores for psoriasis (all severities) ranged from 0.52 (standard deviation: 0.39) to 0.9 (standard deviation: 0.1). Of the 48 results from the second search, six met the inclusion criteria. The reported EQ-5D lower limit for other diseases ranged from 0.20 (Type 2 diabetes mellitus) to 0.66 (liver diseases). The highest EQ-5D estimates for other diseases ranged from 0.79 (liver diseases) to 0.93 (cancer patients). Both lower and upper EQ-5D estimates in psoriasis patients were within the range of those reported for other chronic diseases. CONCLUSION: Comparative studies of morbidity are relevant in health care studies and patient advocacy. This systematic review demonstrates that the ranges of disutility among psoriasis patients are within the ranges of other chronic diseases (cardiovascular diseases, diabetes, end-stage renal diseases, liver diseases, cancer, and visual disorders).

Time Spent per delta PASI (TSdP) among psoriasis patients undergoing UVB-therapy - A pilot study.

BACKGROUND: Psoriasis often poses a significant challenge to treat. Ultraviolet light band (UVB) treatment is widely used and well recognized. However, the frequent visits to the dermatologist may indirectly present an impediment to many of the patients' careers and every day life due to the vast time burden with subsequent lost work earnings. We investigated the cost-effectiveness of the psoriasis UVB treatment from a patient's point of view using time as a surrogate measure. METHOD: Twenty-five patients participated (17 males and 8 females) in the study. We registered the number of UVB treatments per patient as well as the patients PASI (psoriasis area and severity index) score at each treatment session. The time spent in relation to each treatment session was reported in a questionnaire given to the patient. Time Spent per delta PASI (TSdP) was defined as number of minutes to reduce the PASI score by one. RESULTS: Seventeen (68%) of the participants needed less than 300 min to lower the PASI with one score. The rest (n = 7 (32%)) used more than 300 min to lower the PASI score by one. Thus, the TSdP distribution displayed two peaks; ><300 min/dPASI. Log-transformed linear regression model showed that the expected TSdP would decrease with 42% for each time the start PASI was doubled, i.e. the higher the PASI level at the beginning of the treatment, the lower the TSdP. CONCLUSION: The level of reduction in psoriasis severity compared with the amount of lost work time/earnings may pose a much higher impact for patients and society than what is currently recognized.

Association of metabolic syndrome and hidradenitis suppurativa.

IMPORTANCE An association between the metabolic syndrome (MetS) and chronic inflammatory diseases, such as psoriasis or rheumatoid arthritis, has been suggested.Hidradenitis suppurativa (HS), a more localized chronic inflammation of the skin, has been speculated to have a similar association. Hidradenitis suppurativa is a substantial burden for the individual and a socioeconomic burden globally. Information about the burden of possible comorbidities is scarce.OBJECTIVE To investigate the possibility of an association between HS and MetS.DESIGN, SETTING, AND PARTICIPANTS Cross-sectional population- and hospital-based study of HS and MetS.We identified 32 patients with physician-verified HS from the outpatient clinic at the Department of Dermatology, Roskilde Hospital, and 326 patients with HS and 14 851 individuals without HS from the general population. Individuals with HS were younger,predominantly female, and more often smokers compared with the non-HS group.EXPOSURE Hidradenitis suppurativa.MAIN OUTCOMES AND MEASURES Metabolic syndrome and its components of diabetes mellitus, hypertension, dyslipidemia, and obesity.RESULTS When compared with the non-HS group, the odds ratios (ORs) for the hospital HS and population HS groups were 3.89 (95%CI, 1.90-7.98) and 2.08 (95%CI, 1.61-2.69),respectively, for MetS; 5.74 (95%CI, 1.91-17.24) and 2.44 (95%CI, 1.55-3.83), respectively, for diabetes mellitus; 6.38 (95%CI, 2.99-13.62) and 2.56 (95%CI, 2.00-3.28), respectively, for general obesity; and 3.62 (95%CI, 1.73-7.60) and 2.24 (95%CI, 1.78-2.82), respectively, for abdominal obesity. With regard to dyslipidemia, significant results were found for decreased levels of high-density lipoprotein cholesterol, with ORs of 2.97 (95%CI, 1.45-6.08) and 1.94(95%CI, 1.52-2.48) for the hospital HS and general population HS groups, respectively, when compared with the non-HS group. With regard to increased triglyceride levels, only the result for the population HS group compared with the non-HS group was significant, with an OR of1.49 (95%CI, 1.18-1.87). The OR for hypertension, which was only significant for the hospital HS group compared with the non-HS group, was 2.14 (95%CI, 1.01-4.53). Obesity and inflammation acted as possible confounders. The ORs were higher for the hospital HS group compared with the population HS group. The association between HS and MetS was not influenced by the degree of HS severity.CONCLUSIONS AND RELEVANCE As with more systemic inflammatory diseases, HS appears to be associated with MetS, indicating substantial comorbidities. Because this study is cross-sectional, causality remains to be explored.