Validation of global item for assessing impact on quality of life of patients with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory disease. The HS core outcome set calls for a patient global assessment (PtGA). OBJECTIVES: To assess the validity, reliability and responsiveness of a candidate single-item PtGA for HS-specific health-related quality of life (HRQoL). METHODS: Cognitive debriefing interviews were conducted with patients with HS in Denmark and the USA. A cross-sectional observational study was done with adults with HS in the USA and Denmark. Candidate PtGA item, demographic items and multiple patient-reported scales - the Hidradenitis Suppurativa Quality of Life (HiSQOL), Dermatology Life Quality Index (DLQI) and numerical rating scale (NRS) for pain - were concurrently administered to evaluate convergent and known-groups validity. Scales with a single-item assessment of change were readministered 24-72 h later, to evaluate reliability and responsiveness. RESULTS: After cognitive debriefing, the candidate PtGA for HS-specific HRQoL was finalized with five response levels. Convergent validity of the PtGA was supported by significant correlations with HiSQOL score [r = 0·79, 95% confidence interval (CI) 0·75-0·82] and DLQI (r = 0·78, 95% CI 0·74-0·82). The PtGA displayed known-groups validity with DLQI score bands based on significance of an anova (P < 0·001). Good test-retest reliability was supported by the intraclass correlation coefficient (0·82, 95% CI 0·78-0·85) for those who reported stable HS. Responsiveness was assessed by differences in PtGA score against a patient-reported assessment of change, which showed significant differences towards improvement. CONCLUSIONS: The single-item PtGA exhibits reliability, validity and responsiveness in assessing HS-specific HRQoL in HS, making it a good provisional tool for HS clinical research.

The Hidradenitis Suppurativa Quality of Life (HiSQOL) score: development and validation of a measure for clinical trials.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic, inflammatory condition that can have a large negative impact on health-related quality of life (HRQOL). A reliable and validated measure of HS-specific HRQOL in clinical studies is needed. OBJECTIVES: To develop and validate the Hidradenitis Suppurativa Quality Of Life (HiSQOL©) scale, for clinical trial measurement of HS-specific HRQOL. METHODS: In stage 1, qualitative concept elicitation interviews were conducted with patients with HS in Denmark (n = 21) and the U.S.A. (n = 21). In stage 2, cognitive debriefing interviews were performed with U.S. (n = 30) and Danish patients with HS (n = 30). In stage 3 an observational study of 222 patients with HS in the U.S.A. was conducted for item reduction, measure validation and assessment of psychometric properties. In stage 4, an observational study of 215 patients with HS in Denmark was conducted to confirm the psychometric structure derived in stage 3. In both studies the Dermatology Life Quality Index, Hospital Anxiety and Depression Scale and numerical rating scale for pain were also included. RESULTS: In concept elicitation, 99 items were generated, which were reduced to 41 after removing duplicates. In cognitive debriefing, two items were added and one item removed. A 42-item instrument was psychometrically assessed. Based on psychometric analyses and patient input, the instrument was reduced to 17 items that had strong psychometric properties in both the U.S. and Danish samples. CONCLUSIONS: The HiSQOL is a reliable and valid instrument to measure HS-specific HRQOL in clinical trials.

Creation and pilot test results of the dermatology-specific proxy instrument: the Infants and Toddlers Dermatology Quality of Life.

BACKGROUND: Until now, there was no validated dermatology-specific health-related quality of life (HRQoL) instrument to be used in youngest patients. OBJECTIVE: To create dermatology-specific proxy instrument for HRQoL assessment in children from birth to 4 years. METHODS: International focus groups, item selection and pilot tests were utilized. In order to avoid the problem of cross-cultural inequivalence, focus group work and pilot tests were planned simultaneously in all national centres of the project. Comprehensibility, clarity, acceptance and internal consistency of new instrument were checked. RESULTS: The title 'Infants and Toddlers Dermatology Quality of Life' was chosen for our new instrument with the proposed acronym 'InToDermQoL'. Focus group work was completed in seven national centres (Croatia, Germany, Greece, Malta, Poland, Romania and Ukraine). A total of 170 families of children with different skin diseases were interviewed, and a pilot version of the instrument was created. Centres from France, Denmark and Spain have joined the project at this stage. Parents of 125 children with skin diseases filled in the pilot versions of the instrument. Good comprehensibility, clarity, acceptance and internal consistency of the InToDermQoL were confirmed. The pilot test results showed that the InToDermQoL questionnaire well differentiates severity-dependent differences. It was also checked and confirmed during the pilot test that no significant information was missed in the questionnaire. Three age-specific versions of the InToDermQoL questionnaire with 10, 12 and 15 items, respectively, were approved for field tests. CONCLUSION: The pilot test results showed that the InToDermQoL questionnaire has good comprehensibility, clarity, acceptance and internal consistency and well differentiates severity-dependent differences. Further validation of the InToDermQoL during international field test will be performed.

A core domain set for hidradenitis suppurativa trial outcomes: an international Delphi process.

BACKGROUND: There is no consensus on core outcome domains for hidradenitis suppurativa (HS). Heterogeneous outcome measure instruments in clinical trials likely leads to outcome-reporting bias and limits the ability to synthesize evidence. OBJECTIVES: To achieve global multistakeholder consensus on a core outcome set (COS) of domains regarding what to measure in clinical trials for HS. METHODS: Six stakeholder groups participated in a Delphi process that included five anonymous e-Delphi rounds and four face-to-face consensus meetings to reach consensus on the final COS. The aim was for a 1 : 1 ratio of patients to healthcare professionals (HCPs). RESULTS: A total of 41 patients and 52 HCPs from 19 countries in four continents participated in the consensus process, which yielded a final COS that included five domains: pain, physical signs, HS-specific quality of life, global assessment and progression of course. A sixth domain, symptoms, was highly supported by patients and not by HCPs but is recommended for the core domain set. CONCLUSIONS: Routine adoption of the COS in future HS trials should ensure that core outcomes of importance to both patients and HCPs are collected.

Spanish version of the Actinic Keratosis Quality of Life questionnaire.

BACKGROUND: Patient-reported outcome measures are very important outcomes. For specific diseases, health-related quality of life-instruments (HRQoL) are increasingly used to provide data on patients' overall perceptions of the course of a given disease. Actinic keratoses (AKs) are common keratotic lesions that occur on chronically sunlight-exposed skin. Only few studies regarding HRQoL in AKs have been made. OBJECTIVE: In order to be able to compare HRQoL among different countries and cultures, we aimed to translate and validate the Actinic Keratosis Quality of Life (AKQoL) questionnaire into Spanish and quantify the impairment caused by AKs in Spanish patients. METHODS: The AKQoL was translated. Then, 15 patients with AKs were interviewed to ensure cultural adaption before it was tested in one hundred patients with AK lesions at the Melanoma Unit of Hospital Clinic in Barcelona. RESULTS: Validation showed high interitem correlations, as well as a high correlation of each item and the total score. Internal consistency (Cronbach's coefficient alpha) was also high at 0.91 and an alpha value of 0.90 at retest. The test-retest correlation was 0.96, and the intraclass coefficient was 0.98. CONCLUSION: The presented data support the AKQoL Spanish version as a valid and reliable HRQoL questionnaire for the description of AK-related QoL and may provide a method for comparison of AK specific QoL between different cultures and countries.

Risk factors, clinical course and long-term prognosis in hidradenitis suppurativa: a cross-sectional study.

BACKGROUND: Hidradenitis suppurativa (HS) causes considerable morbidity. The long-term prognosis is of obvious interest to both patients and physicians. We conducted this study to determine the prognosis and risk factors in patients diagnosed with HS. OBJECTIVES: To describe the long-term prognosis and the clinical course of HS and its association to known risk factors. METHODS: A postal follow-up survey with uncomplicated factual questions was conducted. As all of the patients were well acquainted with their long-standing disease, this was thought to be sufficient for meaningful results. All cases were diagnosed by a dermatologist. Overall, 212 patients diagnosed with HS between 1981 and 2001 were studied after a median follow-up period of 22 years (range 12-32). RESULTS: The overall response rate was 71.2%, with 60.8% (129/212) valid (fully completed) questionnaires. Remission was reported by 39.4% (50/127) and improvement by 31.5% (40/127). Unchanged severity was reported by 20.5% (26/127), and 8.7% (11/127) experienced worsening disease. Tobacco smoking was reported by 92.2% (119/129). Among nonsmokers, 40% (35/88) reported remission vs. 29% (17/59) of active smokers. A higher proportion of nonobese patients (45%) reported remission than obese patients (23%). CONCLUSIONS: We found that 39.4% of the sample reported remission of HS. Suspected risk factors appeared to influence the prognosis. Smoking and obesity were significantly linked to a lower rate of self-reported remission. The notion that lifestyle factors play a role in HS appears to be supported by this survey.

Assessing the influence of actinic keratosis on patients' quality of life: the AKQoL questionnaire.

BACKGROUND: Limited knowledge is available regarding quality of life in patients with actinic keratosis (AK). OBJECTIVES: To develop and validate a disease-specific questionnaire - the Actinic Keratosis Quality of Life questionnaire (AKQoL) - to assess the quality of life of patients with AK. METHODS: Based on an extensive literature search and patient interviews, the AKQoL was developed in a stepwise approach. An initial mega-questionnaire was composed and subsequently shortened based on statistical differences between patients and controls. A test-retest was done to establish the reliability and to refine the items further. Rasch analyses were performed on the final questionnaire. RESULTS: Initially, 175 items were tested in a mega-questionnaire. The questionnaires were sent out twice and statistical analyses were made, reducing the number of questions to 18 and 10, respectively. Subsequent inter-item correlations showed that one item had only a weak correlation to the rest of the scale. This was confirmed by the Rasch model and by internal consistency as evaluated by Cronbach's coefficient alpha. Only one item was found to provide a small sex difference. A Bland-Altman plot showed excellent reliability. Items are scored on a standard 4-point Likert scale and summarized in a total score of maximum 27 points. A higher score indicates greater quality of life impairment. CONCLUSIONS: A nine-item questionnaire for patients with AK was developed. The AKQoL has three domains covering emotions, function and control and one single global item. The questionnaire's scale structure, the content and face validity, and the reliability have been established.

Depression in patients with hidradenitis suppurativa.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic recurrent inflammatory skin disease with abscess formation and scarring predominantly in the inverse areas. The disease is often difficult to treat and patients experience a decreased quality of life (QoL). It is hypothesized that depression is more common in HS patients than among other dermatological patients. OBJECTIVES: To evaluate the prevalence of depression in patients with HS. METHODS: In total 211 HS patients were included in the study and 233 were dermatological control patients. Their QoL and depression scores were assessed using the Dermatology Life Quality Index (DLQI) and the Major Depression Inventory (MDI) questionnaires. HS severity was recorded with a questionnaire and Hurley stages were extracted from the case records. RESULTS: The DLQI was significantly higher for HS patients than for the control patients, 8.4 ± 7.5 vs. 4.3 ± 5.6 (P < 0.0001) and correlated with Hurley stage severity scores. Mean MDI scores were significantly higher for HS patients, 11.0 vs. 7.2 (P < 0.0001). However, clinically defined depression rates according to the International Classification of Diseases, 10th edition (ICD-10) criteria were not significantly higher in HS patients compared to controls (9% vs. 6%). CONCLUSIONS: HS is a chronic skin disease with major impact on QoL even when compared to other dermatological diseases. MDI scores in HS patients correlate with disease severity. This correlation could indicate that the MDI represents a valid measure of disease related morbidity that may serve as an outcome measure in future studies and a relevant point of intervention for individual patients.

Questionnaire-based diagnosis of hidradenitis suppurativa: specificity, sensitivity and positive predictive value of specific diagnostic questions.

BACKGROUND: Estimates of the prevalence of hidradenitis suppurativa (HS) range from 0.33% to 4%. Further epidemiological data are therefore needed. Because of the hidden nature of the disease, physical screening may be cumbersome and questionnaire-based screening may be more appropriate. OBJECTIVES: To establish the sensitivity (SE), specificity (SP) and positive predictive value (PPV) of simple diagnostic questions used in HS. METHODS: Potential diagnostic questions regarding HS were identified and sent to 85 patients with HS and to an age- and sex-matched control group randomly selected among outpatients not being treated for HS. All respondents were recruited in the Department of Dermatology in Roskilde Hospital. RESULTS: In total, 74 of 85 patients with HS (87%) returned the questionnaire (61 women and 13 men). Of these, 72 reported repeated outbreaks of painful nodules or boils in locations typical for HS compared with 13 patients in the control group. The SE ranged from 0.92 to 0.97, the SP from 0.82 to 0.86 and the PPV from 0.85 to 0.89. Boils appeared significantly more often in patients with HS, who also reported significantly greater suffering from their lesions. CONCLUSIONS: The high diagnostic power suggests that all the questions are potentially useful. The clear symptomatology of HS may be a key factor. It is suggested that further improvement may be achieved by adding definitions of pimples, nodules or boils to future questionnaires. Similarly, adding the possibility to indicate uncommon locations, duration and quality-of-life impairment may benefit the diagnostic power.

The handicap caused by atopic dermatitis--sick leave and job avoidance.

BACKGROUND: The handicap caused by skin diseases is insufficiently described in comparison to other types of diseases (e.g. rheumatological diseases). Estimates of disease handicap provide important data for overall disease assessment, resource planning and background data for planning possible preventive intervention studies. OBJECTIVE: Primarily to describe the degree of handicap caused by atopic dermatitis (AD) in relation to the choice of education and occupation. Secondarily to describe the frequency of social pensions awarded due to AD in Denmark and the development of pensions awarded over time. METHODS: A descriptive study based on questionnaires gathered from members of the Danish Association of Atopic Eczema. Information was obtained on age, disease duration, severity, localization of eczema and Dermatology Life Quality Index (DLQI). Additional data about sick days due to eczema and patients' choice of education or employment were obtained from the Danish Ministry of Social Affairs. RESULTS: Study population 112 (24 males and 88 females), aged 15 years and older, self-assessed morbidity was mild (1/3), moderate (1/2) or severe (approximately 1/5). Mean DLQI score was 9.67. Mean loss of working days due to AD was 5.8 days/6 months (95% CI: 0.36-11.30). The mean total loss of working days due to eczema alone was 148% of the national average, and 38% of the respondents had abstained from a specific education or a job due to AD. Since 1970, the average number of pension due to AD awarded in Denmark has grown from 4.2 per year for 1970-1976 to 18.0 per year for 1999-2002. CONCLUSION: Even mild to moderate AD causes handicap as seen from increased sick leave and the number of awarded early retirement pensions. There are therefore both individual as well as societal consequences of AD.

Parent gender and assessment of infant life quality.

OBJECTIVES: The primary objective was to examine the influence of parents' gender on the assessment of quality of life among infants with atopic dermatitis. The secondary objective was to examine the psychological factors affecting the assessment. METHODS: Questionnaires were completed by 28 couples, as a basis for gender comparison. Data were gathered on disease severity, confidence of treatment, Major Depression Index (MDI), Spielberger Trait Anxiety Inventory (STAI) and the Infants' Dermatitis Quality of Life Index (IDQOL). RESULTS: The mothers' scores were higher than those of fathers for all assessments, although differences were not statistically significant. Significant correlations were found between MDI and severity assessment for mothers only and between MDI and STAI for both genders. Multiple linear regression analysis suggested gender differences in the proposed model, where only depression was appearing statistically significant (P = 0.029). CONCLUSION: Our data presented suggest that no significant gender-dependent differences occur in parents' overall severity assessment of the eczema in their children, but the influence of the different factors studied is not equal in the two sexes. Future instruments aimed at surrogate assessment of disease severity in children by their parents should take this into account and provide explicit testing of possible gender differences.