Self-management-competency as a new target in Hidradenitis suppurativa care.

Background: Hidradenitis suppurativa affects approximately 1% of the population.Objective: Highlighting the relevance of self-management-competency as a new therapeutic target.Method: 258 patients from the 'Epidemiology and Care in Acne inversa (EpiCAi)' project were included in the study. Disease burden was measured by patient-rated questionnaires in terms of disease activity, pain, quality of life, depression and insomnia and correlated with the domains of the health education impact questionnaire (heiQ) measuring self-management-competency.Results: 66 male (25.6%) and 192 female (74.4%) patients, with a mean age of 40.3 ± 10.24 years were included. Mean scores of pain on the numeric rating scale (NRS), Dermatology Life Quality Index (DLQI) and Hospital Anxiety and Depression Scale (HADS) were 5.11 ± 2.68, 11.35 ± 7.79 and 13.71 ± 7.57, respectively. The Insomnia severity index (ISI) showed a mean of 9.58 ± 5.76. The HADS has the highest increased total risk across all heiQ domains. With respect to the heiQ domains, the highest exposure can be attributed to improving constructive attitudes and approaches as well as decreasing emotional distress.Conclusion: There is a clear association of self-management-competency with overall disease burden, which underlines the need for psychoeducational support. This study provides ideas to develop new possible strategies of care.

Sexual impairment in patients with hidradenitis suppurativa: a systematic review.

Hidradenitis suppurativa (HS) can cause considerable impact on several aspects of quality of life. Sexuality is a central aspect of quality of life. In recent years, there has been an increase in the number of articles on HS and sexuality. To achieve our aim of synthesizing the available scientific evidence on HS and sexual health, we conducted a systematic review in February 2020. The clinical databases used included Medline and Embase. All types of epidemiological articles were included; reviews, guidelines, protocols, conference abstracts and case report articles were excluded. Eleven studies were included for review, representing 42 729 patients with HS. The most common study design was cross-sectional with or without comparison group(s), conducted in an outpatient setting or through surveys. Prevalence of sexual dysfunction ranged between 51-62%, and in the case of erectile dysfunction, a specific kind of sexual dysfunction affecting penile erection, it ranged from between 52% and 60% of patients studied using validated questionnaires. Potential risk factors for sexual dysfunction among men and women were identified, mainly related to disease activity, symptoms and partners. Mood disorders like depression and anxiety appear to be associated with sexual dysfunction. Women were more affected by sexual distress. HS patients with sexual dysfunction had a decreased overall quality of life. With respect to treatment, surgery did not improve sexual function and there is no scientific evidence regarding medical treatments. Patients stated that they would like to treat their sexual problems with healthcare professionals. In conclusion, sexual and erectile dysfunction are common in HS patients, and negatively affect their quality of life. There are clinical factors potentially associated with this which should be identified and treated by dermatologists in the comprehensive care of HS patients. Prospective studies are needed to provide more scientific evidence on this unmet need.

Psychosocial burden of Hidradenitis Suppurativa patients' partners.

BACKGROUND: Hidradenitis suppurativa is a debilitating disease related to a great psychosocial burden in affected patients and subsequently also people around them. Patients' partners as caregivers may indirectly experience wide range of devastating effects of the disease on their emotional and social life. OBJECTIVE: The purpose of this study was to determine the QoL impairment in HS patients' partners and to identify its aspects that are affected the most. Correlation between QoL burden and disease severity, duration, sex, age and smoking was also assessed. METHODS: A total of 50 HS sufferers were assessed according to disease severity and their partners' QoL was determined using the Family Dermatology Life Quality Index questionnaire. RESULTS: The mean FDLQI for patients' partners was 8.7 ± 6.8 points, indicating generally a moderate effect of HS on their life. Quality of partners' life correlated significantly with disease severity but no correlation was found according to other factors. CONCLUSION: Hidradenitis suppurativa is a highly psychologically devastating disease not only for patients but also for their partners. It occurred to diminish partners' QoL mostly by increasing daily expenditure but also other problems were often reported. Clinicians should be aware of these psychosocial implications, in order to provide optimal therapy of HS affected families by a multidisciplinary specialized management addressing both, patients and their cohabitants simultaneously.

Prevalence and clinical characteristics of itch in epidemic-like scenario of dermatophytoses in India: a cross-sectional study.

BACKGROUND: Itch is an integral part of clinical picture of superficial dermatophytoses which constitute a common and growing problem in India. OBJECTIVES: The study aimed to evaluate the prevalence, intensity and clinical characteristics of itch in superficial dermatophytosis. METHODS: The data concerning disease history and clinical type of dermatophytosis were obtained. The presence and various characteristics of itch were documented. Numerical Rating Scale (NRS) was utilized to assess the worst intensity of itch during the last 3 days and during the course of the disease. 4-Item Itch Questionnaire was utilized to assess itch extent, intensity, frequency and associated sleep impairment, while quality of life (QoL) impairment was assessed via Dermatology Life Quality Index. RESULTS: Ninety-nine patients with direct microscopic confirmation of dermatophytosis were included in the study. In 46.5% of subjects, the coexistence of tinea corporis and tinea cruris was noted, followed by tinea cruris (25.2%) and tinea corporis (13.1%). The majority of patients reported itch in the last 3 days (99%) and complained of itch limited to skin lesions (89.9%). According to NRS, the mean intensity of worst itch in the last 3 days was 6.8 ± 1.8 points. Severe and very severe itch was reported by 74.7% of patients. Itch was an isolated sensation in 34.3% of subjects, while 46.9% reported associated burning sensation. Itch was frequently exacerbated by sweating, hot temperature and wearing tight clothes. Difficulties in falling asleep and sleep awakenings were reported by 34.3% and 54.6% of subjects, respectively. Itch negatively influenced the well-being of patients and its intensity correlated with QoL impairment. CONCLUSIONS: Itch is an important symptom in superficial dermatophytoses and is associated with negative impact on sleep and carries a significant psychosocial burden. Acknowledging its presence is necessary in a holistic approach to these patients.

Profound consequences of hidradenitis suppurativa: a review.

Hidradenitis suppurativa (HS) influences patients' lives in many ways. The most troublesome symptom of HS is chronic pain, of mild-to-moderate intensity, which is reported by almost all patients. With reference to psychosocial evaluation, HS appears to be a highly debilitating disease. The major factors influencing patients' well-being are disease severity, the number of flares or affected skin areas, and the lesion location. The mean Dermatology Life Quality Index scores of 8·3-12·7 points obtained among patients with HS are typical for severe dermatoses, which have a large impact on patients' quality of life (QoL). HS not only affects skin-related QoL issues, but also has a profound impact on general QoL measures - it causes substantial deterioration of both physical and mental health. Considering the impaired QoL due to HS and HS-associated, nonpsychiatric comorbidities, it is not surprising that patients with HS experience psychological disturbances. In both observational and registry studies, depression and anxiety were significantly related to HS. The prevalences were estimated as 1·6-42·9% and 0·8-3·9%, respectively. HS was also linked to feelings of loneliness and stigmatization. Moreover, these patients have a significantly increased suicide risk (hazard ratio 2·42). HS, with its frequent involvement of the genital area, causes a pronounced impairment of sex life (66·7% of patients with HS reported sexual difficulties). Finally, HS causes significant financial burden, not only through the costs of healthcare, but also due to a substantial impact on patients' professional careers. This is a reason for absenteeism from work for approximately half of patients, and a relatively high unemployment rate.

Pilonidal sinus disease: an intergluteal localization of hidradenitis suppurativa/acne inversa: a cross-sectional study among 2465 patients.

BACKGROUND: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. OBJECTIVES: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. METHODS: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. CONCLUSIONS: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies. What's already known about this topic? The occurrence of pilonidal sinus disease has not been clearly reported among patients with hidradenitis suppurativa/acne inversa. What does this study add? This is the first study that investigated the prevalence of pilonidal sinus disease among a large cohort of patients and identified the patient characteristics. Risk factors that might help to improve the management of patients were identified.

Inter-rater agreement and reliability of outcome measurement instruments and staging systems used in hidradenitis suppurativa.

BACKGROUND: Monitoring disease activity over time is a prerequisite for clinical practice and research. Valid and reliable outcome measurement instruments (OMIs) and staging systems provide researchers and clinicians with benchmark tools to assess the primary and secondary outcomes of interventional trials and to guide treatment selection properly. OBJECTIVES: To investigate inter-rater reliability and agreement in instruments currently used in hidradenitis suppurativa (HS), with dermatologists experienced in HS as the rater population of interest. METHODS: In a prospective completely balanced design, 24 patients with HS underwent a physical examination by 12 raters (288 assessments) using nine instruments. The results were analysed using generalized linear mixed models. RESULTS: For the staging systems, the study found good inter-rater reliability for Hurley staging in the axillae and gluteal region, moderate inter-rater reliability for Hurley staging in the groin and for Physician's Global Assessment, and fair inter-rater reliability for refined Hurley staging and the International HS Severity Scoring System. For all the tested OMIs, the observed intervals for limits of agreement were very wide relative to the ranges of the scales. CONCLUSIONS: The very wide intervals for limits of agreement imply that substantial changes are needed in clinical research in order to rule out measurement error. The results illustrate a difficulty, even for experienced HS experts, to agree on the type and number of lesions when evaluating disease severity. The apparent caveats call for global efforts, such as the HIdradenitis SuppuraTiva cORe outcomes set International Collaboration (HISTORIC) to reach consensus on how best to measure physical signs of HS reliably in randomized trials. What's already known about this topic? Without valid and reliable instruments to measure outcomes, researchers and clinicians lack the necessary benchmarks to assess primary and secondary end points of interventional trials properly. Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease. Several outcome measure instruments exist for HS, but their validation is generally incomplete or of relatively low methodological quality. What does this study add? Using a prospective completely balanced design this study examined inter-rater reliability with HS-experienced dermatologists as the rater population of interest. The study did not find very good reliability for any included instrument or lesion counts. This study illustrates the difficulty in finding agreement on the type and number of HS lesions, even among experts. The results question whether physical signs are best measured by a traditional physician lesion count instrument. What are the clinical implications of this work? For staging, Hurley staging and physician global visual analogue scale proved to be acceptable instruments in terms of inter-rater reliability. For the instruments designed to measure changes in health status, our study illustrates how difficult it is, even for experts, to measure the physical signs of HS using a simple rater counting. Consequently, other assessment methods of physicals signs, such as ultrasound evaluation, require consideration.

Endoplasmic reticulum aminopeptidase 1 polymorphism Ile276Met is associated with atopic dermatitis and affects the generation of an HLA-C associated antigenic epitope in vitro.

BACKGROUND: Atopic dermatitis (AD) is a common inflammatory skin disease of complex aetiology, with interactions between susceptibility genes and environmental factors. We have previously described a protective effect of the KIR2DS1 gene encoding the natural killer cell receptor, whose ligands are HLA-C molecules. Here, we found an association of HLA-C*05:01 allele with AD. KIR-HLA-C interactions are affected by peptides presented by HLA-C. The generation of these peptides is strongly influenced by endoplasmic reticulum aminopeptidases 1 and 2 (ERAP1 and ERAP2). Expression and activity of ERAP molecules depend on the polymorphisms of their genes. OBJECTIVE: Possible associations of several single nucleotide polymorphisms (SNPs) in the ERAP1 and ERAP2 genes with susceptibility to AD. METHODS: Peripheral blood DNA isolation from 318 patients and 549 controls. PCR-SSO or PCR-SSP for HLA-C typing; TaqMan Genotyping Assay for ERAP typing. RESULTS: Only one SNP in the ERAP1 gene, rs26618T>C, causing the amino acid change Ile276Met, had an association with AD. To gain insight on the functional role of this SNP, we produced recombinant variants differing only at position 276 (Ile or Met) and tested their aminopeptidase activity against a N-terminally extended precursor LIVDRPVTLV of the HLA-C*05:01 epitope IVDRPVTLV. Both ERAP1 variants were able to efficiently generate the epitope, although the 276Ile allotype was able to do this about 50% faster. Furthermore, both variants were quite inefficient in further degradation of the mature epitope. Finally, we found that the effect of 276Met on susceptibility to AD was seen only in KIR2DS1-negative individuals, not protected by this KIR. CONCLUSION: Associations of HLA-C*05:01 allele and rs26618T>C (Ile276Met) ERAP1 polymorphism with AD, and a significant difference between these two ERAP1 variants in their ability to generate an epitope for the HLA-C*05:01 molecule was found.

Low and high body mass index in hidradenitis suppurativa patients-different subtypes?

INTRODUCTION: Overweight is a well-established risk factor for hidradenitis suppurativa (HS). In this cross-sectional study, we compare HS patients with a high body mass index (BMI) with HS patients with a low BMI to investigate differences in disease characteristics. MATERIALS AND METHOD: Patients were recruited from 17 dermatological centres from four continents. A total of 246 patients with a BMI below 25 were compared to 205 patients with a BMI of above 35. RESULTS: Patients with a high BMI suffered more severe disease (Hurley, physician global assessment, number of areas affected and patient-reported severity (PRS), P < 0.001 for all). There was no difference in smoking (P = 0.783) nor in family history (P = 0.088). In both low and high BMI patients, early onset of HS was a predictor of positive family history (P < 0.001, for each). For low BMI patients, an increase in BMI significantly increased PRS (P < 0.001). For patients with a high BMI, number of pack-years significantly increased PRS (P = 0.001). Cluster analysis of eruption patterns was location specific for low BMI patients but severity specific for high BMI patients. DISCUSSION: Patients with a low and high BMI could represent two clinically different subtypes. We suggest a non-linear relationship between BMI and impact of HS. As patients go from a low BMI patient to a high BMI patient (or from high to low), eruption patterns and risk factors may change.

Towards global consensus on core outcomes for hidradenitis suppurativa research: an update from the HISTORIC consensus meetings I and II.

BACKGROUND: A core outcomes set (COS) is an agreed minimum set of outcomes that should be measured and reported in all clinical trials for a specific condition. Hidradenitis suppurativa (HS) has no agreed-upon COS. A central aspect in the COS development process is to identify a set of candidate outcome domains from a long list of items. Our long list had been developed from patient interviews, a systematic review of the literature and a healthcare professional survey, and initial votes had been cast in two e-Delphi surveys. In this manuscript, we describe two in-person consensus meetings of Delphi participants designed to ensure an inclusive approach to generation of domains from related items. OBJECTIVES: To consider which items from a long list of candidate items to exclude and which to cluster into outcome domains. METHODS: The study used an international and multistakeholder approach, involving patients, dermatologists, surgeons, the pharmaceutical industry and medical regulators. The study format was a combination of formal presentations, small group work based on nominal group theory and a subsequent online confirmation survey. RESULTS: Forty-one individuals from 13 countries and four continents participated. Nine items were excluded and there was consensus to propose seven domains: disease course, physical signs, HS-specific quality of life, satisfaction, symptoms, pain and global assessments. CONCLUSIONS: The HISTORIC consensus meetings I and II will be followed by further e-Delphi rounds to finalize the core domain set, building on the work of the in-person consensus meetings.

Development and validation of the International Hidradenitis Suppurativa Severity Score System (IHS4), a novel dynamic scoring system to assess HS severity.

BACKGROUND: A validated tool for the dynamic severity assessment of hidradenitis suppurativa/acne inversa (HS) is lacking. OBJECTIVES: To develop and validate a novel dynamic scoring system to assess the severity of HS. METHODS: A Delphi voting procedure was conducted among the members of the European Hidradenitis Suppurativa Foundation (EHSF) to achieve consensus towards an initial HS Severity Score System (HS4). Strengths and weaknesses of HS4 were examined by a multicentre prospective study. Multivariate logistic regression, discriminant analysis and receiver operating characteristic curves, as well as examination for correlation (Spearman's rho) and agreement (Cohen's kappa) with existing scores, were engaged to recognize the variables for a new International HS4 (IHS4) that was established by a second Delphi round. RESULTS: Consensus HS4 was based on number of skin lesions, number of skin areas involved and Dermatology Life Quality Index (DLQI), and was evaluated by a sample of 236 patients from 11 centres. Subsequently, a multivariate regression model calculated adjusted odds ratios for several clinical signs. Nodules, abscesses and draining tunnels resulted as the scoring variables. Three candidate scores were presented to the second Delphi round. The resulting IHS4 score is arrived at by the number of nodules (multiplied by 1) plus the number of abscesses (multiplied by 2) plus the number of draining tunnels (multiplied by 4). A total score of 3 or less signifies mild, 4-10 signifies moderate and 11 or higher signifies severe disease. Cohen's kappa was fair (κ = 0·32) compared with Hurley classification, and moderate (κ = 0·49) compared with Expert Opinion. Correlation was good (ρ > 0·6) with Hurley classification, Expert Opinion, Physician's Global Assessment and Modified Sartorius score, and moderate for DLQI (ρ = 0·36). CONCLUSIONS: The novel IHS4 is a validated tool to dynamically assess HS severity and can be used both in real-life and the clinical trials setting.

A new laboratory device with mathematically based positioning of a frozen tissue block facilitating precise sectioning of large specimens.

Mohs micrographic surgery (MMS) is a treatment method aiming at thorough, personalized eradication of skin cancers by mean of staged excision of tissues surrounding the tumor with complete (100%) histopathological examination of their margins. In many MMS laboratories, the excised tissue is divided, shaped, frozen in a cryostat with a heat extractor and positioned manually (with the block on the object disc) in an articulated cryostat chuck during cutting. However, these activities may be difficult, time-consuming and associated with the risk of imprecise tissue sectioning. Development of a laboratory device allowing for processing of large tissue specimens, with the function of mechanical, mathematically steered positioning of the tissue block surface directly to the microtome knife cutting place, eliminating the need for manual adjustment. The prototype device was designed and manufactured. Its functioning was tested on 513 histological slides produced during 212 operations of skin cancers using MMS. The depth of the first complete sections and the diameter of sections were measured. Complete sections were obtained at an average depth of 81.60 m (min. 20 m, max. 180 m, SD = 29.15), whereas the average diameter of sections was 18.11 mm (min. 4 mm, max. 42 mm, SD = 9.10). The histological processing of large specimens with mathematically based positioning of the tissue surface in relation to the cryotome knife cutting plane is precise, fast and easy. The device can be useful in those MMS centers which continue to employ manual setting of the cryostat chuck or share the cryostat with other users, which prevents fixing the chuck position (including large hospital settings). It may also be helpful in centers using a cryostat with a fixed chuck, for the correction of minimal inaccuracies of its preset position.

European S1 guideline for the treatment of hidradenitis suppurativa/acne inversa.

Hidradenitis suppurativa/acne inversa (HS) is a chronic, inflammatory, recurrent, debilitating skin disease of the hair follicle that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing areas of the body, most commonly the axillae, inguinal and anogenital regions. A mean disease incidence of 6.0 per 100,000 person-years and an average prevalence of 1% has been reported in Europe. HS has the highest impact on patients' quality of life among all assessed dermatological diseases. HS is associated with a variety of concomitant and secondary diseases, such as obesity, metabolic syndrome, inflammatory bowel disease, e.g. Crohn's disease, spondyloarthropathy, follicular occlusion syndrome and other hyperergic diseases. The central pathogenic event in HS is believed to be the occlusion of the upper part of the hair follicle leading to a perifollicular lympho-histiocytic inflammation. A highly significant association between the prevalence of HS and current smoking (Odds ratio 12.55) and overweight (Odds ratio 1.1 for each body mass index unit) has been documented. The European S1 HS guideline suggests that the disease should be treated based on its individual subjective impact and objective severity. Locally recurring lesions can be treated by classical surgery or LASER techniques, whereas medical treatment either as monotherapy or in combination with radical surgery is more appropriate for widely spread lesions. Medical therapy may include antibiotics (clindamycin plus rifampicine, tetracyclines), acitretin and biologics (adalimumab, infliximab). A Hurley severity grade-relevant treatment of HS is recommended by the expert group following a treatment algorithm. Adjuvant measurements, such as pain management, treatment of superinfections, weight loss and tobacco abstinence have to be considered.

Acitretin treatment for hidradenitis suppurativa: a prospective series of 17 patients.

BACKGROUND: The management of hidradenitis suppurativa (HS) is still challenging, and new treatment methods are necessary. Acitretin seems to be a promising agent in HS management; however, literature data are limited. OBJECTIVES: To investigate the clinical efficacy of acitretin monotherapy in 17 patients with long-standing and recalcitrant HS. Simultaneously, an assessment of quality of life was carried out. METHODS: The study was conducted with a group of 17 patients with HS. Patients were treated with acitretin for up to 9 months and examined at baseline, after 1 month, and then every 3 months from baseline. The clinical extent of disease severity was measured with the HS Severity Index (HSSI), Hurley scoring system, Physician's Global Assessment and Dermatology Life Quality Index (DLQI). RESULTS: Nine patients (53%) finished the whole 9 months of acitretin treatment. The mean acitretin dose was 0·56 ± 0·08 mg kg(-1) daily. A significant improvement of clinical manifestation (HSSI, DLQI) was observed after only 1 month of therapy, and further improvement was recorded during the next few months. Overall, eight subjects (47%) fulfilled the criteria for response (HSSI ≥ 50% reduction from baseline). The dropout rate was 47%, due mostly to drug ineffectiveness and adverse events. Discontinuation of treatment resulted in deterioration or relapse of HS 2-8 months after acitretin cessation, in all but one patient. CONCLUSIONS: HS treatment with acitretin seems to be a promising method of disease management. However, due to the high daily dosage, its usage may be limited.