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.

Baseline Characteristics of a National French E-Cohort of Hidradenitis Suppurativa in ComPaRe and Comparison with Other Large Hidradenitis Suppurativa Cohorts.

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition substantially impacting patients' quality of life; the pathogenesis remains unclear, and treatment is complex and not yet standardized. Observational data are increasingly being used to evaluate therapeutics in "real-life" interventions, and the development of e-cohorts is offering new tools for epidemiological studies at the population level. OBJECTIVE: The aim of this study was to describe the clinical characteristics and treatment history of HS participants in the Community of Patients for Research (ComPaRe) cohort and to compare these to other cohorts. METHODS: We performed a cross-sectional study of the baseline data of HS participants in ComPaRe, an e-cohort of patients with chronic diseases. Data were collected using patient-reported questionnaires about clinical-dem-ographic aspects, quality of life, and treatment history. RESULTS: A total of 396 participants (339 females, 57 males) were included (mean age 38 years); 83 (21%) had a family history of HS, 227 (57.3%) were current smokers, and 241 (60.9%) were overweight or obese. Most of the participants declared a Hurley stage II (n = 263, 66.4%) or III (n = 76, 20.3%). The breast was more frequently affected in women than men (37.5 vs. 5.3%, p < 0.0001), whereas the dorsal region was more frequently affected in men (39.5 vs. 10.9%, p < 0.0001). Increased disease stage was associated with obesity (25.9 vs. 33.8 vs. 51.3%, p = 0.02) and some HS localizations (genital [p < 0.005], pubis [p < 0.007], gluteal fold [p = 0.02], and groin [p < 0.0001]). The most frequently prescribed treatments were oral antibiotics (n = 362, 91.4%), especially amoxicillin-clavulanic acid and cyclins. Less than 10% of participants received biologics. Most of these results were consistent with previously published cohorts. CONCLUSION: Recruitment of participants by such a web platform can be a faster way to get relevant scientific data for a wide variety of patients that could be used for epidemiological studies and to evaluate therapeutics in "real-life" interventions.

What causes hidradenitis suppurativa ?-15 years after.

The 14 authors of the first review article on hidradenitis suppurativa (HS) pathogenesis published 2008 in EXPERIMENTAL DERMATOLOGY cumulating from the 1st International Hidradenitis Suppurativa Research Symposium held March 30-April 2, 2006 in Dessau, Germany with 33 participants were prophetic when they wrote "Hopefully, this heralds a welcome new tradition: to get to the molecular heart of HS pathogenesis, which can only be achieved by a renaissance of solid basic HS research, as the key to developing more effective HS therapy." (Kurzen et al. What causes hidradenitis suppurativa? Exp Dermatol 2008;17:455). Fifteen years later, there is no doubt that the desired renaissance of solid basic HS research is progressing with rapid steps and that HS has developed deep roots among inflammatory diseases in Dermatology and beyond, recognized as "the only inflammatory skin disease than can be healed". This anniversary article of 43 research-performing authors from all around the globe in the official journal of the European Hidradenitis Suppurativa Foundation e.V. (EHSF e.V.) and the Hidradenitis Suppurativa Foundation, Inc (HSF USA) summarizes the evidence of the intense HS clinical and experimental research during the last 15 years in all aspects of the disease and provides information of the developments to come in the near future.

Which Factors Determine Affected Sites in Hidradenitis Suppurativa?

BACKGROUND: The factors that determine whether an area of the body will be affected by hidradenitis suppurativa (HS) are unknown. METHODS: To address these factors, we performed multivariate regression analyses in a cohort of 1,138 patients. RESULTS: We found that the body sites affected occurred in specific combinations that were influenced by sex and body mass index. We also revealed unexpected correlations between some sites and other comorbidities such as inflammatory diseases, acne conglobata, or dissecting folliculitis of the scalp. CONCLUSION: Such correlations are crucial to unravel a disease as variable as HS and identify pathophysiological mechanisms to enable the provision of personalized management.

Hidradenitis Suppurativa Influences Tattooing Practice in Women.

BACKGROUND: The reasons or the motivations that compel people to get a tattoo or a body piercing (BP) are plentiful and personal. Patients with chronic cutaneous or systemic conditions may be interested in getting tattooed or pierced. OBJECTIVE: To determine the frequency of body art practices in hidradenitis suppurativa (HS) and to evaluate the possible impact of HS in the decision-making process. METHODS: An anonymous specific paper-based questionnaire was prospectively offered to patients (≥18 years) consecutively managed for HS from September 2016 to March 2017 in a French specialized center. RESULTS: In total, 209 patients participated in the study, of whom 77 (37%) and 50 (24%) had tattoos and BP, respectively. These proportions were higher than those reported in the French population. HS prevented the decision to get a BP or a tattoo in 2 and 5%, respectively (fear of infection or healing difficulties). Fifteen patients (7%) got tattooed to hide a surgical HS scar (n = 5, 2%), to regain control over the pain or their bodies (n = 9, 4%), or to turn others' eyes away from HS lesions or scars (n = 1, 0.5%). HS severity influenced a proportion of patients who intended to get a new tattoo. CONCLUSION: HS is associated with a significant increase in body art practices.

What should a surgeon know about hidradenitis suppurativa?

Otherwise known as Verneuil's disease, hidradenitis suppurative (HS) is a severe dermatosis of heterogeneous appearance affecting 1% of the population. Its pathophysiology is multifactorial, involving genetic predisposition, inflammatory disorder and environmental elements. Its diagnosis is based on the association of three clinical characteristics: characteristic lesions, typical localizations, and the chronic and recurrent nature of the lesions. Given its diversified aspects, diagnosis can be difficult to achieve. As its manifestations often include abscesses, it is important for the surgeon to know how to detect this pathology. The development of biologics has improved treatment of this disease, treatment that necessitates a multidisciplinary medical and surgical approach involving dermatologists and proctologists as well as surgeons. The objective of this report is to synthesize what a surgeon will need to know so as effectively treat HS patients.

Antibiotic Treatment for Hidradenitis Suppurativa in France: A Practice Survey.

Purpose: Antibiotics are used for hidradenitis suppurativa's management with limited evidence. Choice of antibiotics is based on small randomized controlled trial or open case-series. Patients and Methods: We performed a practice survey in Resoverneuil, a French network of physicians treating hidradenitis suppurativa, to identify the antibiotic strategy according to the Hurley stage. Online questionnaire was sent to all members of ResoVerneuil between January and February 2021. Results: In total, 108 physicians answered the survey: 37.6% were hospital based, 34.6% had a private practice and 27.8% a mixed practice, and 13.8% had a dedicated consultation for hidradenitis suppurativa. Sixty-three physicians reported seeing fewer than 5 patients with hidradenitis suppurativa per month; 29 seeing 5 to 15 patients per month; and 9 seeing more than 15 patients per month. More than 90% declared prescribing antibiotics for flares in Hurley 1 and 2 stages, and 83% in Hurley 3 stages, mostly amoxicillin-clavulanic acid and pristinamycin. Of these physicians, 29.7% declared prescribing a background antibiotic therapy for Hurley 1 stage with less than 4 flares per year, and more than 75% for Hurley 1 stage with more than 4 flares per year, Hurley 2 and Hurley 3 stages; mostly cyclins, combination of rifampicin and clindamycin and sulfamethoxazole-trimethoprim. Conclusion: This survey underlines the heterogeneity in antibiotic prescription for hidradenitis suppurativa in France, particularly as background therapy.

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.

How to Define Mild to Severe Hidradenitis Suppurativa? A Simple New Tool Based on Latent Class Analysis of EPIVER Data Study.

Purpose: Hidradenitis suppurativa (HS) is an inflammatory skin disease characterized by recurrent or chronic painful and suppurating lesions in the apocrine gland-bearing regions. The lack of knowledge about HS and its extremely heterogeneous clinical presentation, in terms of both lesion appearance and sites of involvement, frequently delay its diagnosis for several years. Objectives: in this study, using the latent class analysis, it was demonstrated that severity of HS could be evaluated not only with clinical or surgical characteristics but also with gender specificities. Patients and Methods: Clinical and sociodemographic data of HS patients were retrospectively analysed with the latent class method in order to create a classification tool of disease severity. Results: From the study of 1428 HS patients (544 men and 884 women), two classification models, depending on gender, were developed. Each classification model was composed of three distinct latent classes clearly identified and defined from mild-to-severe cases of HS. These classification models of HS severity were not distorted by patient ages and were coherent with Hurley stages but were more clinically precise. Conclusion: In this study, a convenient classification tool, useful for facilitating decision support in routine practice, has been developed. This tool could be used to define clinical subgroups within a study population.

Hidradenitis Suppurativa or Hidradenitis Suppurativa-Like Lesions Located on Amputation Stumps? Description of 2 Cases.

Hidradenitis suppurativa (HS) is a chronic and recurrent inflammatory skin disorder affecting mainly the areas rich in apocrine sweat glands, such as the axillae, groins and buttocks. The role of mechanical pressure and friction due to clothing in the pathogenesis of HS lesions has been previously stressed. Here, we report 2 middle-aged men who presented with HS lesions/HS-like lesions on their amputation stump and review 2 additional cases from the literature. Management was challenging as 2 patients needed tumor necrosis αinhibitor while deroofing/surgery was the option for the 2 others. These cases highlight that mechanical pressure and friction are environmental factors that can play a role in the pathogenesis of HS lesions.

Clinical characteristics of pediatric hidradenitis suppurativa: a cross-sectional multicenter study of 140 patients.

Hidradenitis suppurativa (HS) rarely affects pediatric patients. The literature on pediatric HS patients is scarce. This is a cross-sectional study based on case note review or interviews and clinical examination of 140 pediatric patients undergoing secondary or tertiary level care. Patients were predominantly female (75.5%, n = 105) with a median age of 16. 39% reported 1st-degree relative with HS. Median BMI percentile was 88, and 11% were smokers (n = 15). Median modified Sartorius score was 8.5. Notable comorbidities found were acne (32.8%, n = 45), hirsutism (19.3%, n = 27), and pilonidal cysts (16.4%, n = 23). Resorcinol (n = 27) and clindamycin (n = 25) were the most frequently used topical treatments. Patients were treated with tetracycline (n = 32), or oral clindamycin and rifampicin in combination (n = 29). Surgical excision was performed in 18 patients, deroofing in five and incision in seven patients. Obesity seemed to be prominent in the pediatric population and correlated to parent BMI, suggesting a potential for preventive measures for the family. Disease management appeared to be similar to that of adult HS, bearing in mind that the younger the patient, the milder the disease in majority of cases.

Thyroid hormone receptor expression in the obligatory paedomorphic salamander Necturus maculosus.

Amphibian metamorphosis is under the strict control of thyroid hormones (TH). These hormones induce metamorphosis by controlling gene expression through binding to thyroid hormone receptors (TRs). Necturus maculosus is considered to be an obligatory paedomorphic Amphibian since metamorphosis never occurs spontaneously and cannot be induced by pharmacological means. Since metamorphosis depends on the acquisition of response of tadpole tissues to thyroid hormone, we aimed to determine TR gene expression patterns in Necturus maculosus as well as the expression of two TH-related genes: Cytosolic Thyroid Hormone-Binding Protein (CTHBP)-M2-pyruvate kinase, a gene encoding a cytosolic TH binding protein and stromelysin 3, a direct TH target gene in Xenopus laevis. Tissue samples were obtained from specimens of Necturus maculosus. We performed in situ hybridization using non-cross-hybridizing RNA probes obtained from the cloned Necturus TRalpha and TRbeta genes. We found clear expression of Necturus TRalpha gene in several tissues including the central nervous system, epithelial cells of digestive and urinary organs, as well as myocardium and skeletal muscle. TRbeta was also expressed in the brain. In other tissues, hybridization signals were too low to draw reliable conclusions about their precise distribution. In addition, we observed that the expression of CTHBP and ST3 is largely distinct from that of TRs. The fact that we observed a clear expression of TRalpha and TRbeta which are evolutionary conserved, suggests that Necturus tissues express TRs. Our results thus indicate that, in contrast to previously held hypotheses, Necturus tissues are TH responsive.