Summary
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.
What's already known about this topic?
Hidradenitis suppurativa (HS) causes considerable morbidity.
Smoking and obesity are suggested exogenous risk factors, and inheritance has an influence on development of HS.
Some patients with HS experience remission.
What does this study add?
Nonsmoking and nonobesity are linked to a better chance of remission from HS.
In this cohort 39% of patients experienced remission.
New long‐term follow‐up data describing the clinical course of HS are presented.
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.
Summary
Background
Quantification of disease severity supports the development of evidence‐based treatments. Assessments to capture clinical improvement in hidradenitis suppurativa (HS) can be ...improved.
Objectives
This study aimed to validate the Hidradenitis Suppurativa Clinical Response (HiSCR), which is defined as a ≥ 50% reduction in inflammatory lesion count (sum of abscesses and inflammatory nodules, AN), and no increase in abscesses or draining fistulas in HS when compared with baseline as a meaningful clinical endpoint for HS treatment.
Methods
Patients with ≥ 3 ANs at baseline in a Phase II adalimumab trial for HS were included for analysis. HiSCR achievers vs. nonachievers were assessed at week 16 and week 52. Criteria measures included physician‐rated assessments Hurley stage, modified Sartorius score (MSS), and HS Physician's Global Assessment and patient‐reported outcomes (PROs: visual analogue pain scale, Dermatology Life Quality Index, and Work Productivity and Activity Impairment questionnaire). Test–retest reliability, convergent validity, responsiveness and predictive validity of HiSCR, and its meaningfulness to patients were assessed.
Results
Among 138 eligible study participants, the majority were female (69·6%) with a mean age of 36·7 years. The mean (median) MSS was 125·2 (85·5) at baseline. Test–retest reliability of the AN count was 0·91. HiSCR was significantly correlated with improvements in all physician‐rated and PRO measures (Spearman's rho between −0·61 and −0·27, all P < 0·001). Improvements of all PROs in HiSCR achievers exceeded the respective meaningful improvement thresholds.
Conclusions
In patients with HS with ≥ 3 ANs, HiSCR achievers had significant improvements in physician‐rated and patient‐reported HS disease severity and impact. HiSCR is a valid and meaningful endpoint for assessing HS treatment effectiveness in controlling inflammatory manifestations in this population.
What's already known about this topic?
Hurley stage, modified Sartorius score and Physician's Global Assessment have been used in clinical studies to assess severity of hidradenitis suppurativa.
However, these clinical measures assessing treatment effectiveness may not have optimal sensitivity, measurement consistency and ease of use.
What does this study add?
The Hidradenitis Suppurativa Clinical Response (HiSCR) is a valid, responsive and meaningful clinical endpoint of the inflammatory manifestations of HS that can be adapted to clinical research and daily practice.
Hidradenitis suppurativa is a painful, chronic inflammatory skin disease. In two double-blind, placebo-controlled trials, treatment with adalimumab resulted in significantly increased rates of ...clinical response at week 12. Rates of serious adverse events were similar between groups.
Hidradenitis suppurativa, also known as acne inversa, is a painful, chronic inflammatory skin disease
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characterized by multifocal, recurrent nodules, abscesses, and fistulas, predominantly affecting the axillary, inguinal, breast-fold, and anogenital regions.
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The prevalence of self-reported disease is 1% in Western Europe.
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The average interval from the onset of symptoms to diagnosis is 7.2 years.
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Women are affected 2 to 5 times as frequently as men, and the disease may be more common in blacks than in whites.
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Disease severity ranges from mild (localized lesions) to severe (multiple areas of widely dispersed lesions, including interconnected sinus tracts and . . .
Summary
Hidradenitis suppurativa (HS) is a chronic, inflammatory, debilitating skin disease. The aim of the study was to systematically review the literature and critically answer the question: In ...patients with HS, do cardiovascular risk factors appear at a significantly higher rate compared with controls? The main search was conducted in Medline, Embase and the Cochrane Central Register. Studies eligible for inclusion were of case–control, cross‐sectional and cohort design, and included comparison of any cardiovascular risk factor(s) in patients with HS with those of control groups. An I2 value > 50% was considered to show substantial heterogeneity. In this case, DerSimonian and Laird random‐effect models were considered to compute pooled odds ratios (OR). Otherwise, a fixed‐effects model was suitable. Nine studies, with 6174 patients with HS and 24 993 controls, were included. Significant association of HS with obesity OR 3·45, 95% confidence interval (CI) 2·20–5·38, P < 0·001, central obesity (OR 2·97, 95% CI 1·41–6·25, P = 0·004), active smoking (OR 4·34, 95% CI 2·48–7·60, P < 0·001), history of smoking (OR 6·34, 95% CI 2·41–16·68, P < 0·001), hypertriglyceridemia (OR 1·67, 95% CI 1·14–2·47, P = 0·009), low high‐density lipoprotein (HDL) (OR 2·48, 95% CI 1·49–4·16, P < 0·001), diabetes (OR 2·85, 95% CI 1·34–6·08, P = 0·007) and metabolic syndrome (OR 2·22, 95% CI 1·62–3·06, P < 0·001) was detected. Associations were significant both in population and hospital patients with HS, with hospital HS groups having uniformly higher ORs than the population HS groups. Causality could not be assessed. Heterogeneity was substantial in all analyses. This systematic review indicated that cardiovascular risk factors appear at a significantly higher rate in patients with HS compared with controls. The need for screening of patients with HS for modifiable cardiovascular risks is emphasized.
What's already known about this topic?
Patients with hidradenitis suppurativa (HS) present several cardiovascular risk factors more often and to a higher degree than healthy controls.
Results of epidemiological studies vary depending on the populations studied.
What does this study add?
A significant association of HS with obesity, active smoking, hypertriglyceridemia, low high‐density lipoprotein, diabetes and metabolic syndrome was detected.
Hospital HS groups had uniformly higher association with comorbidity than the population HS groups
Linked Comment: Simpson, Br J Dermatol 2015; 173: 1118–19.
Summary
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.
What's already known about this topic?
Outcome measure instruments used for hidradenitis suppurativa (HS) are markedly heterogeneous with 30 instruments recently found in 12 randomized trials.
Lack of consensus regarding outcome measure instruments limits evidence synthesis and increases the risk of outcome reporting bias.
A core domain set is an agreed minimum set of what to measure that should be reported in all clinical trials of a specific condition.
What does this study add?
Our study provides global multistakeholder consensus on core outcome domains for HS.
The final core domain set includes 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 healthcare professionals; it is recommended by the steering committee as an additional core domain, in the context of being a patient‐reported domain.
What are the clinical implications of this work?
The routine adoption of this core outcome set in future HS trials should ensure that outcome domains of importance to both patients and HCPs are included and reported.
Linked Editorial: Kottner & Schmitt. Br J Dermatol 2018; 179:549–550.
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IMPORTANCE: Surgery is a mainstay in the management of hidradenitis suppurativa (HS). Adalimumab is the first drug approved for HS. OBJECTIVE: To investigate the efficacy and safety of adalimumab in ...combination with wide-excision surgery followed by secondary intention healing. DESIGN, SETTING, AND PARTICIPANTS: The Safety and Efficacy of Adalimumab for Hidradenitis Suppurativa Peri-Surgically (SHARPS) trial was a phase 4, randomized, double-blind, placebo-controlled study of adalimumab in conjunction with surgery. Patients were enrolled in 45 sites across 20 countries from July 18, 2016, to February 2, 2019, with the last patient visit on October 16, 2019. Eligible patients (aged 18-65 years) had moderate to severe HS that required radical surgery in an axillary or inguinal region and had 2 other anatomical regions affected, with 1 or more regions at Hurley stage II or III. Analysis was conducted in November 2019. INTERVENTIONS: Patients were randomized 1:1 to receive continuous adalimumab, 40 mg, or placebo during presurgery (12 weeks), perioperative (2 weeks), and postoperative (10 weeks) periods. MAIN OUTCOMES AND MEASURES: The primary end point was the proportion of patients achieving HS clinical response across all body regions at week 12. RESULTS: Overall, 103 patients were randomized to adalimumab and 103 to matching placebo. Among all patients, 51% (n = 106) were women, 94% (n = 193) were White, and the mean (SD) age was 37.6 (11.3) years. At week 12, significantly more patients receiving adalimumab (49 of 103 48%) vs placebo (35 of 103 34%; P = .049) achieved HS clinical response across all body regions (treatment difference, 14% 95% CI, 0%-27%). Treatment-emergent adverse events were reported in 74 of 103 patients (72%) and 69 of 103 patients (67%) in the adalimumab and placebo groups, respectively. No increased risk of postoperative wound infection, complication, or hemorrhage was observed with adalimumab vs placebo. Two deaths occurred in the adalimumab group; neither was considered as having a reasonable possibility of relationship to study drug. CONCLUSIONS AND RELEVANCE: Adalimumab was efficacious in conjunction with wide-excision surgery followed by secondary intention healing, with no need to interrupt treatment prior to surgery. These data support further investigation of adalimumab as an adjuvant therapy to surgery in patients with moderate to severe HS. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT02808975
Summary
Background
Hidradenitis suppurativa (HS) is a chronic inflammatory disease involving inverse recurrent suppuration (IRS). The epidemiology of the disease is not well described, with previous ...studies reporting prevalence estimates from 0·00033% to 4%.
Objective
To determine the prevalence of IRS in a large population‐based survey.
Methods
Data were obtained from a general cross‐sectional population study in Denmark. A validated self‐administered questionnaire was used to identify IRS. Persons with the combination of outbreaks of ‘boils’ during the last 6 months in predefined areas and a minimum of two boils were identified.
Results
A total of 16 404 adults aged 30–89 years provided data and the overall prevalence of IRS was found to be 2·10% 95% confidence interval (CI) 1·88–2·32; in men it was 1·58% (95% CI 1·29–1·86) and in women 2·56% (95% CI 2·21–2·86). No data for adults aged 20–29 years were included. The prevalence declined with age. The majority of cases (72·9%) had a body mass index of ≥ 25 kg m−2 and 77·7% were current or ex‐smokers. The mean number of inflamed lesions was 6·5 (range 2–67) during the last 6 months. The lesions affected mostly the genitalia (43·4%) and groin (39·8%) for women and ‘other places’ (not specified) (78·8%) for men.
Conclusions
Data on IRS suggest that HS may be more prevalent than previously suggested, and the prevalence may be comparable to other major dermatoses, e.g. psoriasis. However, as no physician examination was done, the risk of recall and information bias is a major limitation.
What's already known about this topic?
Hidradenitis suppurativa is a chronic inflammatory disease.
The previous estimates of hidradenitis suppurativa prevalence rates range from 0·00033% to 4%.
What does this study add?
Prevalence data for inverse recurrent suppuration were compatible with the diagnosis of hidradenitis suppurativa in a large population‐based survey sample.
Prevalence data were derived from a validated questionnaire.
To cite this article: Jungersted JM, Scheer H, Mempel M, Baurecht H, Cifuentes L, Høgh JK, Hellgren LI, Jemec GBE, Agner T, Weidinger S. Stratum corneum lipids, skin barrier function and filaggrin ...mutations in patients with atopic eczema. Allergy 2010; 65: 911-918. Prior to the discovery of filaggrin (FLG) mutations, evidence for an impaired skin barrier in atopic dermatitis (AD) has been documented, and changes in ceramide profile, altered skin pH and increased trans-epidermal water loss (TEWL) in patients with AD have been reported. Until now, no studies have analysed stratum corneum (SC) lipids combined with skin barrier parameters in subjects of known FLG genotype. A cohort of 49 German individuals genotyped for the most common FLG mutations (R501X, 2282del4) had SC samples taken for lipid analysis by high-performance thin layer chromatography. In addition, TEWL, erythema, skin hydration and pH were measured. In 27 of the 49 individuals, a 24-h irritation patch test with sodium lauryl sulphate was performed. For the analysis, both the AD group and the control group were stratified by FLG mutation status (FLGmut/FLGwt). In the FLGmut AD group, significantly lower levels of ceramide 4 and significantly higher levels of ceramide 7 were observed when compared to both healthy control groups. However, ceramide 7 levels also significantly differed between FLGwt AD and FLGwt controls, as did ceramide 1 levels. No significant differences were observed for ceramide 2, 3, 5 and 6. FLGmut individuals had significantly higher skin pH values than individuals not carrying FLG mutations. Patients with AD with FLG mutations had significantly higher erythema compared to patients with AD without FLG mutations. Our results confirm previous observations of altered ceramide levels in AD, which however appear to show no clear relationship with FLG mutations.
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