Inter‐rater and intrarater agreement and reliability in clinical staging of hidradenitis suppurativa/acne inversa

Zouboulis, Christos C.; Matusiak, Łukasz; Jemec, Gregor B. E.; Szepietowski, Jacek C.; Álvarez-Chinchilla, Pedro; Asoskova, Anastasiia V.; Bonnekoh, Hanna; Brattoli, G.; Çetinarslan, Tubanur; Dawicka, Joanna; Dente, V.; Gallyamova, Yu. A.; Giovanardi, Giulia; Glasenhardt, K.; A, Ionescu; Janushaj, E.; Lakhssassi, M.K.; Lőrincz, Kende; Ludew, D.; Makmatov-Rys, Mikhail; Malickova, M.; Mengesha, S.W.; Mintoff, Dillon; Otlewska, Agnieszka; Papakou, M.; Pîrvan, Ana Maria; Provvidenziale, Luca; Quadrana, Florencia; Savickaja, N.; Tamulyté, G.; Trifu, A.; Vīgante, A.; Włodarek, Katarzyna; Zouboulis, V.A.

doi:10.1111/bjd.17982

Cited by 15 publications

(22 citation statements)

References 7 publications

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“…Patients suffering from HS experience recurrent inflammatory nodules, draining abscesses, and tunnels mainly in, but not limited to intertriginous skin (2). HS is characterized by significant clinical heterogeneity which complicates disease severity stratification and phenotype classification (3). Several HS severity scoring systems exist, of which Hurley staging is the most widely used static score in clinical practice (4).…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2022

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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.

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Section: Introductionmentioning

confidence: 99%

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

et al. 2022

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“…Patients with HS exhibit extensive phenotypic heterogeneity making genotype-phenotype correlations difficult to establish ( Kent, 2009 ). The task is further complicated by poor interrater reliability of HS phenotypes ( van Straalen et al, 2018 ), differences in severity scoring ( Zouboulis et al, 2019 ) as well as evidence of pleiotropism ( Gratton et al, 2020 ). Notwithstanding, some general patterns are emerging, and encouraging results are emerging even from relatively low-powered studies ( Marzano et al, 2022 ).…”

Section: Discussionmentioning

confidence: 99%

The Genomic Architecture of Hidradenitis Suppurativa—A Systematic Review

2022

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Hidradenitis suppurativa is a chronic, suppurative condition of the pilosebaceous unit manifesting as painful nodules, abscesses, and sinus tracts mostly in, but not limited to, intertriginous skin. Great strides have been made at elucidating the pathophysiology of hidradenitis suppurativa, which appears to be the product of hyperkeratinization and inflammation brought about by environmental factors and a genetic predisposition. The identification of familial hidradenitis suppurativa has sparked research aimed at identifying underlying pathogenic variants in patients who harbor them. The objective of this review is to provide a broad overview of the role of genetics in various aspects of hidradenitis suppurativa, specifically the pathophysiology, diagnosis, and clinical application.

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“…These novel associations not only allow us to further understand the pathogenesis of HS but also translate to the clinical and research setting. Clinically, providers can use these findings to objectively evaluate and monitor disease progression, limiting potential interobserver variability in HS scoring 50 . This can be further extended to allow for a robust assessment of HS disease status in trials and other research studies.…”

Section: Discussionmentioning

confidence: 99%

“…Clinically, providers can use these findings to objectively evaluate and monitor disease progression, limiting potential interobserver variability in HS scoring. 50 This can be further extended to allow for a robust assessment of HS disease status in trials and other research studies.…”

Section: Discussionmentioning

confidence: 99%

Serum inflammatory markers and leukocyte profiles accurately describe hidradenitis suppurativa disease severity

et al. 2022

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Background Inflammatory markers and leukocyte profiles have not been longitudinally evaluated as objective signs of hidradenitis suppurativa (HS) severity. We sought to assess C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), interleukin-6 (IL-6), and leukocyte profiles as reliable indicators of HS severity.Methods Retrospective cohort study of 404 patients seen at the Einstein/Montefiore HS Center, Bronx, New York, between March 2019 and November 2020. Associations of disease severity (HS-Physician Global Assessment) with inflammatory markers and leukocyte profiles were assessed by odds ratios (OR) and 95% confidence intervals (95% CI) incorporating up to four visits per patient, adjusting for baseline gender, age, BMI, and smoking status.Results Patients with severe disease had elevated CRP (OR 1.87; 95% CI 1.49, 2.34),

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Inter‐rater and intrarater agreement and reliability in clinical staging of hidradenitis suppurativa/acne inversa

Cited by 15 publications

References 7 publications

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

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

The Genomic Architecture of Hidradenitis Suppurativa—A Systematic Review

Serum inflammatory markers and leukocyte profiles accurately describe hidradenitis suppurativa disease severity

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