Belgian patients of the European Registry for Hidradenitis Suppurativa (ERHS-Be): data, scores and phenotypes since 2015.

Introduction Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease, characterized by painful and recurrent lesions in apocrine gland-bearing skin areas. It is a heterogeneous disease, which makes assessment and data collection difficult. Questionnaires with detailed items, such as the Belgian European Registry for Hidradenitis Suppurativa (ERHS-Be), are useful to study HS and its associated comorbidities. The aim of this registry is to uncover new factors associated with HS, understand HS patients' clinical profiles and efficacy of treatments. Material and methods The ERHS-Be registry is based on questionnaires, with sections for socio-demographic data, medical and HS history, clinical examination and treatment plan. It allows identification of different clinical phenotypes and automatic calculation of severity scores. Results At present, 606 patients are included in the ERHS-Be (67% women, 33% men). Mean age at first visit is 38.5 years. Tobacco use is present in 72.6% of patients. A family history of HS is noted in 42% of patients. Comorbidities are documented in this cohort: depression is present in 43.8% of patients, arthritis in 27.8%, obesity in 31.5%, hypertension in 10.6%, diabetes mellitus in 6.4% and dyslipidemia in 12.4%. Moreover, 7.7% of patients suffer from IBD and 27.4% have a pilonidal sinus. History of severe acne is found in 32.1% of patients and psoriasis in 9.3%. Thirteen percent of women in our cohort suffer from polycystic ovarian syndrome. Severity of disease is quantified in 533 patients: for instance, Hurley I, II and III scores proportions are respectively 32.3%, 52.7% and 15%, while the mean IHS4 score is 5.2. This registry also enables determination of relative phenotype proportions in our cohort, according to different classifications. Conclusion The ERHS-Be questionnaires allow systematic and larger data collection, including detailed comorbidities, phenotypes and severity of disease. Analysis of this large database will contribute to a better understanding and management of HS, at a time where new therapeutic options are becoming available.

The Global Hidradenitis Suppurativa Atlas (GHiSA) 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 prevalence rates 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. 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. 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.

Outcome Measures for the Evaluation of Treatment Response in Hidradenitis Suppurativa for Clinical Practice: A HiSTORIC Consensus Statement.

Importance: Although several clinician- and patient-reported outcome measures have been developed for trials in hidradenitis suppurativa (HS), there is currently no consensus on which measures are best suited for use in clinical practice. Identifying validated and feasible measures applicable to the practice setting has the potential to optimize treatment strategies and generate generalizable evidence that may inform treatment guidelines. Objective: To establish consensus on a core set of clinician- and patient-reported outcome measures recommended for use in clinical practice and to establish the appropriate interval within which these measures should be applied. Evidence Review: Clinician- and patient-reported HS measures and studies describing their psychometric properties were identified through literature reviews. Identified measures comprised an item reduction survey and subsequent electronic Delphi (e-Delphi) consensus rounds. In each consensus round, a summary of outcome measure components and scoring methods was provided to participants. Experts were provided with feasibility characteristics of clinician measures to aid selection. Consensus was achieved if at least 67% of respondents agreed with use of a measure in clinical practice. Findings: Among HS experts, response rates for item reduction, e-Delphi round 1, and e-Delphi round 2 surveys were 76.4% (42 of 55), 90.5% (38 of 42), and 92.9% (39 of 42), respectively; among patient research partners (PRPs), response rates were 70.8% (17 of 24), 100% (17 of 17), and 82.4% (14 of 17), respectively. The majority of experts across rounds were practicing dermatologists with 18 to 19 years of clinical experience. In the final e-Delphi round, most PRPs were female (12 [85.7%] vs 2 males [11.8%]) and aged 30 to 49 years. In the final e-Delphi round, HS experts and PRPs agreed with the use of the HS Investigator Global Assessment (28 [71.8%]) and HS Quality of Life score (13 [92.9%]), respectively. The most expert-preferred assessment interval in which to apply these measures was 3 months (27 [69.2%]). Conclusions and Relevance: An international group of HS experts and PRPs achieved consensus on a core set of HS measures suitable for use in clinical practice. Consistent use of these measures may lead to more accurate assessments of HS disease activity and life outcomes, facilitating shared treatment decision-making in the practice setting.

Treatment of Acne with Isotretinoin Should Be Avoided in Patients with Hidradenitis Suppurativa "Conglobata Phenotype".

BACKGROUND: Acne conglobata (AC) and nodulocystic acne have long been confused clinically, despite the presentation and the response to treatment being different. AC and hidradenitis suppurativa (HS) resemble each other; a subtype of HS called "conglobata phenotype" has recently been reported in a large Dutch cohort. Acne vulgaris and HS are often associated. Isotretinoin is typically ineffective in treating HS and may even aggravate it, but it is often indispensable in treating acne vulgaris. OBJECTIVE: The aim of the study was to assess whether isotretinoin may be used safely in adults with both HS and acne vulgaris and when it might be contraindicated. MATERIALS AND METHODS: Belgian HS patients from the European Registry for Hidradenitis Suppurativa Registry (ERHS) reporting a history of severe acne of the face and/or the back, and who have ever used isotretinoin for their acne, were all selected. Patients whose acne worsened on isotretinoin were compared to patients whose acne did not worsen (improvement or no change). RESULTS: Among the 82 selected patients, 10 (12.2%) report that their acne was aggravated while taking isotretinoin, while 72 (87.8%) report that their acne was not aggravated on isotretinoin. Of the 10 HS patients whose acne worsened with isotretinoin, 9 (90%) were men (p = 0.04) and 8 (80%) were HS "conglobata phenotype" (p < 0.001). In contrast, 47 (65.3%) of the 72 patients whose acne did not worsen on isotretinoin belonged to the HS "regular phenotype" (p = 0.01). On multivariate analysis, the item most strongly associated with poor response to isotretinoin was the HS "conglobata phenotype," followed by body mass index (BMI) (worse response to isotretinoin if BMI >25 kg/m2). Additionally, of 26 patients who received isotretinoin while their HS had already started, only 6 (23.1%) reported isotretinoin effectiveness on their HS. CONCLUSION: Subject to confirmation by larger studies, our study suggests that isotretinoin should be avoided in the treatment of acne in HS patients with the HS "conglobata phenotype," as it may worsen the acne, likewise being male or having a BMI above 25 seems to increase this risk of a bad therapeutic outcome. Patients with an HS "regular phenotype" appear to be at a reduced risk of isotretinoin treatment worsening their acne.

Obesity and hidradenitis suppurativa: targeting meta-inflammation for therapeutic gain.

Hidradenitis suppurativa (HS) is a chronic, inflammatory condition of the pilosebaceous unit. The typical patient with HS is characterized as someone with obesity, who smokes and who has nodules, abscesses and/or draining tunnels predominantly distributed in intertriginous skin. It has been established that lifestyle and genetic factors are the main pathophysiological drivers of HS. In this critical review, we explore the interrelatedness of meta-inflammation, obesity and HS and discuss if and how this relationship may be manipulated for a therapeutic end.

Overview and comparison of the clinical scores in hidradenitis suppurativa: A real-life clinical data.

Introduction: Partly due to its clinical heterogeneity, hidradenitis suppurativa (HS) is difficult to score accurately; illustrated by the large number of disease scores. In 2016, a systematic review by Ingram et al. reported the use of about thirty scores, and since then, this number has increased further. Our aim is twofold: to provide a succinct but detailed narrative review of the scores used to date, and to compare these scores with each other for individual patients. Materials and methods: The review of the literature was done among articles in English and French, on Google, Google scholar, Pubmed, ScienceDirect and Cochrane. To illustrate the differences between scores, data from some Belgian patients included in the European Registry for HS were selected. A first series of patients compares the severity of the following scores: Hurley, Hurley Staging refined, three versions of Sartorius score (2003, 2007, 2009), Hidradenitis Suppurativa Physician Global Assessment (HS-PGA), International Hidradenitis Suppurativa Severity Scoring System (IHS4), Severity Assessment of Hidradenitis Suppurativa (SAHS), Hidradenitis Suppurativa Severity Index (HSSI), Acne Inversa Severity Index (AISI), the Static Metascore, and one score that is not specific to HS: Dermatology Life Quality Index (DLQI). A second set of patients illustrates how some scores change over time and with treatment: Hurley, Hurley Staging refined, Sartorius 2003, Sartorius 2007, HS-PGA, IHS4, SAHS, AISI, Hidradenitis Suppurativa Clinical Response (HiSCR), the very new iHS4-55, the Dynamic Metascore, and DLQI. Results: Nineteen scores are detailed in this overview. We illustrate that for some patients, the scores do not predictably and consistently correlate with each other, either in an evaluation of the severity at a time-point t, or in the evaluation of the response to a treatment. Some patients in this cohort may be considered responders according to some scores, but non-responders according to others. The clinical heterogeneity of the disease, represented by its many phenotypes, seems partly to explain this difference. Conclusion: These examples illustrate how the choice of a score can lead to different interpretations of the response to a treatment, or even potentially change the results of a randomized clinical trial.

External Validation of the IHS4-55 in a European Antibiotic-Treated Hidradenitis Suppurativa Cohort.

BACKGROUND: Previously, a new dichotomous outcome was developed, calculated as 55% reduction in the International Hidradenitis Suppurativa 4 (IHS4-55) score. It was validated in datasets of adalimumab and placebo-treated HS patients. External validation is an important aspect of clinical outcomes. OBJECTIVES: We aimed to externally validate the novel dichotomous IHS4-55 in a non-biologic treated dataset of HS patients. METHODS: Data from a previously published European-wide prospective clinical study of antibiotic treatment of HS patients were used to assess the association of IHS4-55 achievement with individual reduction in inflammatory nodules, abscesses, and draining tunnels. Moreover, the associations between IHS4-55 positivity and achievement of the minimal clinically important differences (MCIDs) for Dermatology Life Quality Index (DLQI), Numerical Rating Scale (NRS) Pain, and NRS Pruritus were analyzed. RESULTS: Data were obtained from 283 individual patients, of which 36.4% (103/283) were treated with clindamycin and rifampicin and 63.6% (180/283) with tetracyclines for 12 weeks. Achievers of the IHS4-55 demonstrated a significant reduction the counts of inflammatory nodules, abscesses, and draining tunnels (all p < 0.001). Additionally, IHS4-55 achievers had an odds ratio for achieving the MCID of DLQI, NRS Pain, and NRS Pruritus of 2.16 (95% CI 1.28-3.65, p < 0.01), 1.79 (95% CI 1.10-2.91, p < 0.05), and 1.95 (95% CI 1.18-3.22, p < 0.01), respectively. CONCLUSIONS: This study shows the external validity of the novel IHS4-55 by demonstrating a significant association between IHS4-55 achievement and a reduction in inflammatory lesion counts as well as achievement of MCIDs for DLQI, NRS Pain, and NRS Pruritus in an antibiotic-treated cohort. These findings support the use of the IHS4-55 as a novel primary outcome measure in clinical trials.

Case Report: Comorbid Hyper-IgD Syndrome and Hidradenitis Suppurativa - A New Syndromic Form of HS? A Report of Two Cases.

Hidradenitis Suppurativa (HS) is a chronic suppurative disease of the pilosebaceous unit. The current model of HS pathophysiology describes the condition as the product of hyperkeratinisation and inflammation at the hair follicular unit. Environmental factors (such as smoking and obesity), gender, genetic predisposition, and skin dysbiosis are considered the main pathogenic drivers of the disease. Autoinflammatory syndromes associated with HS are rare but may help to highlight the potential roles of autoinflammation and dysregulated innate immune system in HS. Therefore, it is of major relevance to increase the awareness about these diseases in order to improve the understanding of the disease and to optimize the management of the patients. Herein, we report for the first time, to our knowledge, two clinical cases of Hyper-IgD syndrome-associated HS. Hyper-IgD is an autoinflammatory syndrome caused by a mevalonate kinase deficiency (MKD), a key kinase in the sterol and isoprenoid production pathway. We describe the potentially shared pathophysiological mechanisms underpinning comorbid MKD-HS and propose therapeutic options for the management of these patients.

Metabolic syndrome and hidradenitis suppurativa: epidemiological, molecular, and therapeutic aspects.

Hidradenitis suppurativa (HS) is a chronic, suppurative condition of the pilosebaceous unit. Patients suffering from HS demonstrate a molecular profile in keeping with a state of systemic inflammation and are often found to fit the criteria for a diagnosis of metabolic syndrome (MetS). In this paper, we review the literature with regards to established data on the prevalence of MetS in HS patients and revise the odds ratio of comorbid disease. Furthermore, we attempt to draw parallels between inflammatory pathways in HS and MetS and evaluate how convergences may explain the risk of comorbid disease, necessitating the need for multidisciplinary care.

Unilateral Nevoid Telangiectasia in a Healthy Man.

We report the case of a healthy 26-year-old man presenting telangiectatic macules on the left thorax and arm since childhood. The main diagnostic hypothesis were unilateral nevoid telangiectasia (UNT), hereditary benign telangiectasia, atrial myxoma, segmental serpiginous angioma, circumscribed neviform angiokeratoma, and nevus vascularis mixtus. The diagnosis retained was UNT characterized by congenital or acquired telangiectasia distributed asymmetrically along the upper extremities, or the third or fourth cervical dermatomes. The congenital form is extremely rare, predominant in men, and persists in adulthood. The acquired form is most frequent, affects preferentially women, usually appears at puberty or during pregnancy and tends to disappear. Estrogen excess triggers the formation of telangiectasia. UNT is rarely associated with liver or thyroid disorder. Pulsed-dye lasers and normalization of estrogen are proposed as therapeutic options. We report a rare diagnosis of UNT in a young man with no other underlying condition. We would like to highlight that in the presence of unilateral telangiectasia, a complete clinical examination must be performed to rule out signs of hyperestrogenism in man, ocular or neurological abnormalities, a blood test to exclude pregnancy, hepatic and thyroid dysfunctions, and ultrasonography in case of suspicion of atrial myxoma.

Tuberculosis Risk Stratification of Psoriatic Patients Before Anti-TNF-α Treatment.

Psoriasis is a skin inflammatory condition for which significant progress has been made in its management by the use of targeted biological drugs. Detection of latent M. tuberculosis infection (LTBI) is mandatory before starting biotherapy that is associated with reactivation risk. Together with evaluation of TB risk factors and chest radiographs, tuberculin skin tests (TST) and/or blood interferon-γ-release assays (IGRA), like the QuantiFERON (QFT), are usually performed to diagnose M. tuberculosis infection. Using this approach, 14/49 psoriatic patients prospectively included in this study were identified as LTBI (14 TST+, induration size ≥ 10mm, 8 QFT+), and 7/14 received prophylactic anti-TB treatment, the other 7 reporting past-treatment. As the specificity and sensitivity of these tests were challenged, we evaluated the added value of an IGRA in response to a mycobacterial antigen associated with latency, the heparin-binding haemagglutinin (HBHA). All but one TST+ patient had a positive HBHA-IGRA, indicating higher sensitivity than the QFT. The HBHA-IGRA was also positive for 12/35 TST-QFT- patients. Measurement for 15 psoriatic patients (12 with HBHA-IGRA+) of 8 chemokines in addition to IFN-γ revealed a broad array of HBHA-induced chemokines for TST+QFT- and TST-QFT- patients, compared to a more restricted pattern for TST+QFT+ patients. This allowed us to define subgroups within psoriatic patients characterized by different immune responses to M. tuberculosis antigens that may be associated to different risk levels of reactivation of the infection. This approach may help in prioritizing patients who should receive prophylactic anti-TB treatment before starting biotherapies in order to reduce their number.