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Hidradenitis suppurativa (HS) is a chronic inflammatory disorder characterized by painful nodules, sinus tracts, and scars occurring predominantly in intertriginous regions. The prevalence of HS is currently 0.053-4%, with a predominance in African-American women and has been linked to low socioeconomic status. The majority of the reported literature is  retrospective, population based, epidemiologic studies. In this regard, there is a need to establish a repository of biospecimens, which represent appropriate gender and racial demographics amongst HS patients. These efforts will diminish knowledge gaps in understanding the disease pathophysiology. Hence, we sought to outline a step-by-step protocol detailing how we established our HS biobank to facilitate the formation of other HS tissue banks. Equipping researchers with carefully detailed processes for collection of HS specimens would accelerate the accumulation of well-organized human biological material. Over time, the scientific community will have access to a broad range of HS tissue biospecimens, ultimately leading to more rigorous basic and translational research. Moreover, an improved understanding of the pathophysiology is necessary for the discovery of novel therapies for this debilitating disease. We aim to provide high impact translational research methodology for cutaneous biology research and foster multidisciplinary collaboration and advancement of our understanding of cutaneous diseases.

Competing Interests: Conflicts of interest The authors declare no conflicts of interest.

Hidradenitis suppurativa (HS) is a complex, chronic skin disease characterised by painful inflammatory nodules, abscesses, dermal tunnels, sinus tracts and fistulae with a predilection for intertriginous skin. HS carries a substantial disease burden due to its prevalence, associated comorbidities and quality of life impacts and is associated with high healthcare resource utilisation. Clarity regarding the prevalence and pathogenesis of HS has led to improved therapies and more patients seeking care in both outpatient and acute care settings, including the emergency department. Emergency medicine providers play a critical role in HS diagnosis, management of acute flares and connection of HS patients with long-term dermatologic care, which can in turn help manage utilisation of acute care resources., Competing Interests: Competing interests: HN has received grant support from AbbVie; consulting fees from 23andme, AbbVie, Aristea Therapeutics, Nimbus Therapeutics, Medscape, Sonoma Biotherapeutics, DAVA Oncology, Boehringer Ingelheim, Union Chimique Belge and Novartis; investigator fees from Pfizer; and holds shares in Radera. She is also an associate editor for JAMA Dermatology and a board member of the Hidradenitis Suppurativa Foundation., (© Author(s) (or their employer(s)) 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Hidradenitis Suppurativa (HS) is a relatively common and highly morbid inflammatory skin disease. Due to our relatively limited understanding of HS's pathogenesis, there are currently insufficient treatment options available, and many patients' medical needs are not being met. This is partly due to a scarcity of ex vivo human assays and animal models that accurately recapitulate the disease. To address this deficit, we have developed a whole-tissue explant model of HS to examine its pathogenic mechanisms and the efficacy of potential treatments within intact human tissue. We measured cytokine protein and RNA within whole tissue maintained in an agar-media solution, finding that IL-6 and IL-8 concentrations trended upwards in both HS explants and healthy controls, while IL-17A, IL-1β, and TNF-α exhibited increases in HS tissue alone. We also show that the explants were responsive to treatment with both dexamethasone and IL-2. Not only do our results show that this model effectively delivers treatments throughout the explants, but they also elucidate which cytokines are related to the explant process regardless of tissue state and which are related to HS tissue specifically, laying the groundwork for future implementations of this model.

Methods for describing and reporting the clinical and histologic characteristics of cutaneous tissue samples from patients with hidradenitis suppurativa (HS) are not currently standardized, limiting clinicians' and scientists' ability to uniformly record, report, and communicate about the characteristics of tissue used in translational experiments. A recently published consensus statement outlined morphological definitions of typical HS lesions, but no consensus has been reached regarding clinical characterization and examination of HS tissue samples. In this study, we aimed to establish a protocol for reporting histopathologic and clinical characteristics of HS tissue specimens. This study was conducted from May 2023 to August 2023. Experts in clinical care, dermatopathology, and translational research were recruited, and a modified Delphi technique was used to develop a protocol for histologic reporting and clinical characterization of submitted tissue specimens from patients with HS. A total of 27 experts participated (14 dermatologists, 3 fellowship-trained dermatopathologists, 3 plastic surgeons, 3 general surgeons, and 4 research scientists) in creating and reviewing protocols for the clinical and histopathological examination of HS tissue specimens. The protocols were formatted as a synoptic report and will help to consistently classify specimens in biobanks on the basis of histologic features and more accurately report and select samples used in translational research projects., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Several registries for hidradenitis suppurativa (HS) already exist in Europe and the USA. There is currently no global consensus on a core dataset (CDS) for these registries. Creating a global HS registry is challenging, owing to logistical and regulatory constraints, which could limit opportunities for global collaboration as a result of differences in the dataset collected. The solution is to encourage all HS registries to collect the same CDS of information, allowing registries to collaborate., Objectives: To establish a core set of items to be collected by all HS registries globally. The core set will cover demographic details, comorbidities, clinical examination findings, patient-reported outcome measures and treatments., Methods: Beginning in September 2022, 20 participants - including both clinicians with expertise in HS and patient advocates - from eight countries across three continents participated in a Delphi process consisting of four rounds of voting, with all participants completing each round. A list of potential items for inclusion in the core set was generated from the relevant published literature, including systematic reviews of comorbidities in HS, clinical and examination findings, and epidemiology. For disease severity and progression items, the Hidradenitis SuppuraTiva Core outcome set International Collaboration (HiSTORIC) core set and other relevant instruments were considered for inclusion. This resulted in 47 initial items. Participants were invited to suggest additional items to include during the first round. Anonymous feedback was provided to inform each subsequent round of voting to encourage consensus., Results: The eDelphi process established a CDS of 48 items recommended for inclusion in all HS registries globally., Conclusions: The routine adoption of this CDS in current and future HS registries should allow registries in different parts of the world to collaborate, enabling research requiring large numbers of participants., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Hidradenitis suppurativa (HS) is a chronic skin condition affecting approximately 1% of the US population. HS skin lesions are highly inflammatory and characterized by a large immune infiltrate. While B cells and plasma cells comprise a major component of this immune milieu, the biology and the contribution of these cells in HS pathogenesis are unclear. We aimed to investigate the dynamics and microenvironmental interactions of B cells within cutaneous HS lesions. Combining histological analysis, single-cell RNA sequencing, and spatial transcriptomics profiling of HS lesions, we defined the tissue microenvironment relative to B cell activity within this disease. Our findings identified tertiary lymphoid structures (TLSs) within HS lesions and described organized interactions among T cells, B cells, antigen-presenting cells, and skin stroma. We found evidence that B cells within HS TLSs actively underwent maturation, including participation in germinal center reactions and class switch recombination. Moreover, skin stroma and accumulating T cells were primed to support the formation of TLSs and facilitate B cell recruitment during HS. Our data definitively demonstrated the presence of TLSs in lesional HS skin and point to ongoing cutaneous B cell maturation through class switch recombination and affinity maturation during disease progression in this inflamed nonlymphoid tissue.

Competing Interests: Conflicts of interest The authors declare no conflicts of interest.

Background: Hidradenitis suppurativa (HS) is an understudied disease, and current HS studies have focused on participants already connected to dermatologic care., Objective: We surveyed participants in online HS support communities to gain a comprehensive understanding of how provider type impacts HS disease management and the issues individuals with HS face when accessing care., Methods: From June 13 to June 30, 2021, we administered an anonymous cross-sectional online survey to HS Facebook support group participants who had a self-confirmed diagnosis of HS. Survey items assessed respondent demographics, primary HS provider, and barriers to HS care and pain management. Descriptive analyses are presented., Results: The survey was viewed 5,168 times and 1,040 surveys met eligibility criteria (20.1%). Survey participants were 97% female and 72% White. Seventy-two percentage resided in the United States and 22% in Europe. Forty-seven percentage reported having a dermatologist as their primary HS provider, 38% reported a nondermatologist, and 15% reported no HS provider. We found that Asian race, full-time employment, private health insurance, and urban setting were each associated with higher rates of having a dermatologist as a primary HS provider. However, 43.7% of those with a dermatologist reported biologic use, as compared with 14.5% with nondermatologist HS providers. Our cohort was notably more severely impacted by comorbid diseases; 55.9% of our cohort had anxiety, 53.6% had depression, and 50.7% had obesity. Overall, 74.2% of our cohort reported experiencing stigma while accessing care for their HS., Limitations: Participant recruitment via social media platform facilitates recruitment of individuals across the spectrum of healthcare access, but may introduce selection bias and favor well-resourced areas. Self-reported data may be subject to recall bias., Conclusion: Our study provides unique insights into the characteristics and experiences of individuals with HS across the spectrum of health care access., Competing Interests: H.B.N. has received grant support from AbbVie; consulting fees from 23andme, AbbVie, Aristea Therapeutics, Nimbus Therapeutics, Medscape and DAVA Oncology, Boehringer Ingelheim, UCB, and Novartis; investigator fees from Pfizer; and has shares in Radera, Inc. She is also an Associate Editor for JAMA Dermatology and a Board Member of the Hidradenitis Suppurativa Foundation. The other author has no conflicts of interest to disclose., (Copyright © 2024 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of Women’s Dermatologic Society.)

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 prevalences 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 or a private/family medicine clinic. 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. Approximately 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., (© 2024 S. Karger AG, Basel.)

Competing Interests: H.B.N. has received grant support from AbbVie; consulting fees from 23andme, AbbVie, Aristea Therapeutics, Nimbus Therapeutics, Medscape, Sonoma Biotherapeutics, DAVA Oncology, Boehringer Ingelheim, Union Chimique Belge’s (UCB) and Novartis; investigator fees from Pfizer; and holds shares in Radera, Inc. She is also an Associate Editor for JAMA Dermatology and a board member of the Hidradenitis Suppurativa Foundation. The other author has no conflicts of interest to disclose.

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.

Competing Interests: Conflicts of interest Appendix S1 (see Supporting Information).

Chronic inflammatory skin disorders are known to affect sleep quality; however, the relationship between hidradenitis suppurativa (HS) and sleep is not well understood. We performed a systematic review of HS and sleep disorders and sleep quality in HS patients. We identified seven studies comprising 343,870 subjects. We found that HS patients have a higher likelihood of having a sleep disorder such as obstructive sleep apnea, as well as other non-sleep apnea. Several studies showed that patients reported worse sleep quality due to symptoms of HS such as pruritus and pain. HS patients may be at risk for additional cardiovascular comorbidities and poorer quality of life secondary to these sleep disorders and poor sleep quality. Further high-quality research evaluating these associations is warranted., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Competing Interests: Conflicts of interest A list of conflicts of interest can be found in Appendix S1 (see Supporting Information).

Importance: Patient-perceived barriers to hidradenitis suppurativa (HS) care are poorly understood. Understanding health care barriers is a critical first step toward improving care for this population., Objective: To characterize the health care experiences of people living with HS, including perceived barriers and facilitators to health care access, and to elucidate potential associations among these barriers and facilitators, health care access, and disease activity., Design, Setting, and Participants: In this qualitative study, an inductive thematic analysis was conducted on 45 in-depth, 60- to 90-minute semistructured interviews of 45 people with HS from diverse sociodemographic backgrounds that took place between March and April 2020. Individuals were eligible if they could speak English, were 18 years or older, and were diagnosed with HS. A diagnosis of HS was confirmed through physician diagnosis or through self-reported, affirmative response to the validated screening question, "Do you experience boils in your armpits or groin that recur at least every six months?", Main Outcomes and Measures: Interviews were audio recorded and transcribed verbatim. A modified grounded theory approach was used to develop the codebook, which investigators used for inductive thematic analysis., Results: Among the 45 participants included, the median (IQR) age was 37 (16) years, 33 (73%) were female, and 22 (49%) were White. There were 6 interrelated themes associated with participant-perceived barriers to accessing HS care: (1) bidirectional associations of disease activity and employment, (2) association of employment with health care coverage, (3) association of health care coverage with costs and perceived access to care, (4) association of costs with access to patient-centered care, (5) health care professional attitudes and knowledge influence patient-centered care and perceived access to care and disease activity, and (6) health system characteristics influence patient-centered care and associated costs, perceived access to care, and disease activity., Conclusions and Relevance: This qualitative study highlights themes that generate a conceptual model for understanding barriers that may act synergistically to limit health care access and influence disease activity. The disease activity of HS may be reduced when cycle elements are optimized. This study also highlights areas for future investigations and potential systems-level changes to improve access to patient-centered HS care.

Competing Interests: Conflicts of interest Dr Naik has received grant support from AbbVie and the National Psoriasis Foundation, consulting fees from 23andme, AbbVie and DAVA Oncology, Novartis, and Boehringer Ingelheim, all related to hidradenitis suppurativa, has shares in Radera Inc, and is a board member of the Hidradenitis Suppurativa Foundation. Dr Cowen has no conflicts of interest to report.

Introduction: Hidradenitis suppurativa (HS) is a chronic, debilitating, and painful inflammatory skin disease that significantly and negatively impacts patients' quality of life. The prevalence of HS in the USA is estimated to be 0.10%, with worldwide reports suggesting a prevalence closer to 1%. There is limited real-world evidence available on the care of patients with HS. We aimed to evaluate the trends in clinical care and treatment in the patient population with HS in the USA in a real-world setting., Methods: A cohort study was conducted using claims data from IBM MarketScan Databases, including the US Commercial Claims and Encounters with Medicare Supplemental and Coordination of Benefits (CCAE+MDCR) database and IBM US Medicaid database., Results: The annual prevalence of HS increased from 0.06% (2008) to 0.14% (2017), and from 0.17% (2008) to 0.31% (2017) among CCAE+MDCR and Medicaid patients, respectively. Dermatologist visits increased from 31.9% (2008) to 47.8% (2019) in CCAE+MDCR patients, and decreased from 10.9% (2008) to 8.5% (2018) in Medicaid patients. Opioid use decreased from 45.4% (2008) to 25.5% (2019) among CCAE+MDCR patients, and from 71.3% (2008) to 48.1% (2018) among Medicaid patients. Only 8.4% of CCAE+MDCR patients and 5.8% of Medicaid patients were exposed to any biologic in 2018., Conclusions: Improved care and treatment of HS over the last decade, including the emergence of new treatments, have been accompanied by an increase in awareness and reported prevalence of the disease. However, there are still gaps in access to dermatologic care and low utilization of biologic therapies among patients with HS. INFOGRAPHIC., (© 2022. The Author(s).)

Hidradenitis suppurativa is a chronic, progressive inflammatory disease of the skin with many systemic implications. Hidradenitis suppurativa is frequently underdiagnosed or misdiagnosed, particularly because of heterogeneity in presentation and low disease recognition. Patients can see multiple types of health care providers, including primary care providers, along their journey to an accurate diagnosis. This review provides a comprehensive overview of the clinical presentation, associated comorbidities, and life impact associated with hidradenitis suppurativa. Disease features described here can facilitate earlier identification of hidradenitis suppurativa, differentiation from common mimickers, and timely referrals for multidisciplinary management when needed. Engagement of the medical community will also support comprehensive care strategies necessary in hidradenitis suppurativa., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Conflicts of interest HBN has received grant support from AbbVie, consulting fees from 23andMe, AbbVie, and DAVA Oncology, and advisory board fees from Boehringer Ingelheim, all related to hidradenitis suppurativa. DMS and HBN have received grant support from the National Psoriasis Foundation. RGM has received grant support from SOBI, Novartis, Regeneron, and Lilly under US Federal Government Cooperative Research and Development Agreements. DCP, MO, MM, JT, HNS, SMS, SM, AADJ, THM, AD, RKR, ZD, NNM and EWC have no conflicts of interest to report.

Importance: Hidradenitis suppurativa (HS) is associated with considerable diagnostic delay. Although most patients report adolescent onset, existing HS diagnostic criteria may not adequately capture disease in pediatric populations., Objectives: To determine the proportion of physician-diagnosed pediatric patients with HS who met diagnostic criteria, and describe demographics, disease characteristics, and diagnostic patterns among pediatric patients with HS., Design, Setting, and Participants: In this retrospective, cross-sectional study, electronic medical records from 2 sites of a single academic tertiary care center were included. Eligible patients were those born after January 1, 1993, and assigned International Classification of Diseases, Ninth and Tenth Revisions (ICD-9/10) codes for HS (ICD-9 705.83/ICD-10 L73.2) between January 1, 2012, and July 1, 2021. Patients were excluded if they were older than 18 years at diagnosis, had inaccessible diagnostic visit notes, or were unintentionally assigned an HS ICD code., Exposures: Pediatric patients with HS., Main Outcomes and Measures: Fulfillment of diagnostic criteria in pediatric patients with HS., Results: A total of 297 adolescents with HS were included in the study; 123 patients were female (78.1%), 78 self-identified as Black (26.3%), and 116 self-identified as Hispanic (39.1%). The median (IQR) age at diagnosis was 14.0 (13.0-16.0) years. Documentation from the diagnostic visit demonstrated that 127 (42.8%) patients did not meet all 3 major HS diagnostic criteria. Of these patients, 122 (96.1%) did not meet the recurrence interval criterion (≥2 lesions within 6 months). Overall, 96 patients who did not meet the recurrence interval criterion had documentation from additional visits in the health system; 59 (61.5%) had documentation of 1 or more additional lesions consistent with HS. Review of these additional records demonstrated that 26 of these 59 (44.1%) patients met the recurrence interval criterion after diagnosis, and 44 (74.6%) had recurrent lesions within a 1-year interval (median, 6.5 months; interquartile range, 3.5-12.2 months). Medical chart review was conducted from November 22, 2021, to January 12, 2022. Analysis was conducted from January 12, 2022, to January 15, 2022., Conclusions and Relevance: Overall, 118 (40%) of 297 pediatric patients with HS in this retrospective cross-sectional study did not meet all major diagnostic criteria at the time of diagnosis, largely due to failure to fulfill the 6-month recurrence interval criterion. Future studies are needed to determine the appropriate recurrence interval to facilitate timely diagnosis and promote clinical trial eligibility for pediatric patients with HS.

Introduction: Hidradenitis suppurativa (HS) patients may be at increased risk of COVID-19 infection and complications from their medications and comorbidities. There is a lack of expert consensus on recommendations for the COVID-19 vaccine for HS patients. Herein, we aim to provide expert-driven consensus recommendations regarding COVID-19 vaccinations in HS patients., Methods: A modified Delphi consensus survey developed by a core committee of 7 dermatologist HS experts consisting of 4 demographic questions and 12 practice statements was distributed to the US HS Foundation-sponsored provider listserv. Participants were attending physician HS experts. Survey results were to be reviewed by the core group and revised and resubmitted until consensus (≥70% agreement) was achieved., Results: Among the 33 survey participants, there were 30 (87%) dermatologists, 1 general surgeon, 1 plastic surgeon, and 1 rheumatologist. Consensus for all 12 statements on vaccine counseling and HS treatment counseling was achieved after the first round., Discussion/conclusion: For now, this consensus can serve as a resource for clinicians discussing COVID-19 vaccination with their HS patients. These recommendations will need to be updated as new evidence on COVID-19 emerges., Competing Interests: J.L.H. is on the board of directors for the HS Foundation (HSF) and is a consultant for Novartis and speaker for AbbVie. A.A. is consultant for Abbvie, BI, InflaRX, Janssen, Novartis, and UCB and investigator for BI and Processa. C.J.S. is on the board of directors of the HSF, has received research funding from Abbvie, Novartis, Incyte, InflaRx, Chemocentryx, and UCB, and has received honoraria for consulting and/or speaking from Abbvie, UCB, Novartis, and InflaRx. H.B.N. has received grant support from AbbVie, consulting fees from 23andme, Abbvie, and DAVA Oncology, advisory board fees from Boehringer Ingelheim, and is an investigator for Pfizer. She is also an Associate Editor for JAMA Dermatology and an unpaid board member of the US HSF. J.W.F. has conducted advisory work for Janssen, Boehringer Ingelheim, Pfizer, Kyowa Kirin, LEO Pharma, Regeneron, Chemocentryx, Abbvie, and UCB, participated in trials for Pfizer, UCB, Boehringer Ingelheim, Eli Lilly, CSL, Janssen, and received research support from Ortho Dermatologics and Sun Pharma. J.S.K. is consultant for Abbvie, Bayer, Incyte, InflaRX, Janssen, Novartis, and UCB; on the speaker bureau for AbbVie; and received research funding from Incyte. V.Y.S. is on the board of directors for the HSF, is a stock shareholder of Learn Health, and has served as an advisory board member, investigator, speaker, and/or received research funding from Sanofi Genzyme, Regeneron, AbbVie, Eli Lilly, Novartis, SUN Pharma, LEO Pharma, Pfizer, Incyte, Boehringer Ingelheim, Menlo Therapeutics, Dermira, Burt's Bees, Galderma, Kiniksa, UCB, TARGET-DERM, Altus Lab, MYOR, Polyfin, GpSkin, and Skin Actives Scientific., (The Author(s). Published by S. Karger AG, Basel.)

Background: Hidradenitis suppurativa (HS) is associated with comorbidities that contribute to poor health, impaired life quality, and mortality risk., Objective: To provide evidence-based screening recommendations for comorbidities linked to HS., Methods: Systematic reviews were performed to summarize evidence on the prevalence and incidence of 30 comorbidities in patients with HS relative to the general population. The screening recommendation for each comorbidity was informed by the consistency and quality of existing studies, disease prevalence, and magnitude of association, as well as benefits, harms, and feasibility of screening. The level of evidence and strength of corresponding screening recommendation were graded by using the Strength of Recommendation Taxonomy (SORT) criteria., Results: Screening is recommended for the following comorbidities: acne, dissecting cellulitis of the scalp, pilonidal disease, pyoderma gangrenosum, depression, generalized anxiety disorder, suicide, smoking, substance use disorder, polycystic ovary syndrome, obesity, dyslipidemia, diabetes mellitus, metabolic syndrome, hypertension, cardiovascular disease, inflammatory bowel disease, spondyloarthritis, and sexual dysfunction. It is also recommended to screen patients with Down syndrome for HS. The decision to screen for specific comorbidities may vary with patient risk factors. The role of the dermatologist in screening varies according to comorbidity., Limitations: Screening recommendations represent one component of a comprehensive care strategy., Conclusions: Dermatologists should support screening efforts to identify comorbid conditions in HS., Competing Interests: Conflicts of interest Dr Garg has served as a consultant for AbbVie, Amgen, Boehringer Ingelheim, Incyte, Janssen, Novartis, Pfizer, UCB, and Viela Bio; has received honoraria from AbbVie, Amgen, Boehringer Ingelheim, Incyte, Janssen, Novartis, Pfizer, UCB, and Viela Bio; and has received grants: from AbbVie and the National Psoriasis Foundation. Dr Alavi has served as a consultant for AbbVie, Boehringer Ingelheim, Incyte, Janssen, Pfizer, UCB, Actelion, Celgene, Galderma, GlaxoSmithKline, Leo Pharma, Novartis, Sanofi-Genzyme, Kymera, Eli Lily, InflaRx, Sanofi, and Bausch; has received honoraria from AbbVie, Boehringer Ingelheim, Incyte, Janssen, Pfizer, UCB, Actelion, Celgene, Galderma, GlaxoSmithKline, Leo Pharma, Novartis, Genzyme, Kymera, Eli Lily, Sanofi, and Bausch; has received grants from AbbVie; and has received donations of medical equipment from Galderma and Swift. Dr Alhusayen has served as a consultant for AbbVie, Janssen, Leo Pharma, Hidramed Solutions and has received honoraria from AbbVie, and Eli Lilly. Dr Daveluy has served as a consultant for AbbVie; has received honoraria from AbbVie; has served on the speakers bureau for AbbVie; and has received grants from AbbVie, InflaRx, Regeneron, and Pfizer. Dr Delorme has served as a consultant for AbbVie, Celgene, Eli-Lilly, Janssen, Novartis, and Sanofi-Genzyme; has received honoraria from AbbVie, Amgen, Avene, Celgene, Eli Lilly, Janssen, Novartis, and UCB Pharma; has served on the speakers bureau for AbbVie, Bausch Health, Celgene, Eli Lilly, Janssen, Novartis, and Sanofi Genzyme; and has received grants from AbbVie, InflaRx, Regeneron, and Pfizer. Dr Gulliver has served as a consultant for AbbVie, Amgen, Bausch Health, Celgene, Cipher, Eli Lilly, Janssen, LEO Pharma, Novartis, PeerVoice, Pfizer, Sanofi-Genzyme, UCB, and Valeant; has received honoraria from AbbVie, Amgen, Bausch Health, Celgene, Cipher, Eli Lilly, Janssen, LEO Pharma, Novartis, PeerVoice, Pfizer, Sanofi-Genzyme, UCB, and Valeant; and has received grants from AbbVie, Amgen, Eli Lilly, Novartis, and Pfizer. Dr Hamzavi has served as a consultant for Incyte, UCB, and Pfizer and has received grants from Lenicura and Boehringer Ingelheim. Dr Jaleel has served as a consultant for Eli Lilly and ChemoCentryx/IQVIA and has received grants from the Dermatology Foundation and Skin of Color Society. Dr Kimball has served as a consultant for AbbVie, Janssen, Lilly, Novartis, Pfizer, UCB, and Kymera; has received honoraria from AbbVie, Janssen, Lilly, Novartis, Pfizer, UCB, and Kymera; and has received royalties from Pfizer, Trifecta, and UCB. Dr Kirby has served as a consultant for AbbVie, ChemoCentryx, Incyte, Novartis, and UCB; has received honoraria from AbbVie, Incyte, and Viela Bio; and has served on the speakers bureau for AbbVie. Dr Kirchhof has served as a consultant for AbbVie, Eli Lilly, Janssen, Novartis, Pfizer, UCB, Sanofi-Genzyme; has served on the speakers bureau for AbbVie, Janssen, Novartis, Pfizer, UCB, and Sanofi-Genzyme; and has received grants from the Canadian Dermatology Foundation. Dr Lev-Tov has served as a consultant for Pfizer, Mölnlycke, and NextScience; has received honoraria from Essity; has received grants from NextScience and Essity; and has received donations of medical equipment from Essity, Mölnlycke, and NextScience. Dr Lowes has served as a consultant for AbbVie, Janssen, Viela Bio, Almirall, BSN, Incyte, Janssen, Kymera, and XBiotech. Dr Naik has served as a consultant for Boehringer Ingelheim, Janssen, and 23andme and has received grants from AbbVie, Hidradenitis Suppurativa Foundation. Dr Orenstein has received a Hidradenitis Suppurativa Foundation Danby Research Grant. Dr Piguet has served as a consultant for Pfizer, AbbVie, Janssen, UCB, Novartis, Almirall, and Celgene; has received honoraria from Kyowa Kirin Co Ltd, AbbVie, and Novartis; has received grants from AbbVie, Bausch Health, Celgene, Janssen, LEO Pharma, Lilly, NAOS, Novartis, Pfizer, Pierre-Fabre, and Sanofi; and has received a donation of medical equipment from La Roche-Posay. Dr Sayed has served as a consultant for UCB, InflaRx, and AbbVie; has received honoraria from UCB and AbbVie; and has served on the speakers bureau for AbbVie and Novartis. Dr Tan has served as a consultant for Almirall, Bausch, Boots/Walgreens, Botanix, Cipher, Galderma, Incyte, L’Oréal, Novartis, Pfizer, Promius, Sun, and UCB; has received honoraria from Almirall, Bausch, Boots/Walgreens, Botanix, Galderma, L’Oréal, Novartis, Pfizer, Promius, and Sun; has served on the speakers bureau for Galderma and L’Oréal; and has received grants from Incyte and UCB. Drs Alikhan and Goldfarb, Author Lester, Drs Malviya and Micheletti, and Authors Strunk and Wright have no conflicts of interest to declare., (Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Importance: The identification and validation of biomarkers in hidradenitis suppurativa (HS) has potential to improve the understanding and management of this chronic, burdensome disease., Objective: To systematically identify all known HS biomarkers, categorize them by biomarker type, and critically evaluate their validity according to established criteria., Evidence Review: Eligibility criteria for this review (PROSPERO Registration 230830) included randomized clinical trials, uncontrolled clinical trials, cohort studies, case-control studies, and other observational studies with no restrictions of patient age, sex, race or ethnicity, or language of publication up until December 31, 2020. All articles were categorized into biomarker type, defined using the US Food and Drug Administration Biomarkers, Endpoints, and other Tools (BEST) glossary. Assessment of each identified biomarker was undertaken in line with the US Food and Drug Administration and European Medicines Agency guidelines for the validation of proposed biomarkers. Assessment of the strength of overall data regarding individual biomarkers was undertaken using the Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) approach., Findings: A total of 3953 nonduplicate articles were screened, of which 1429 articles were retrieved based on the include/exclusion criteria applied. After full-text screen and data extraction, 106 articles were included in this review. The evidence of strength of 6 categories of biomarkers (susceptibility/risk, diagnostic, monitoring, predictive, prognostic, and pharmacodynamic/response biomarkers) was assessed using GRADE criteria. A total of 48 biomarkers were identified with a minimum GRADE rating of moderate. Only 1 diagnostic (serum IL-2R), 1 monitoring (dermal Doppler vascularity), and 2 predictive biomarkers (epithelialized tunnels and positive family history of HS) achieved a GRADE rating of high. None of the identified biomarkers had sufficient clinical validity to be recommended for routine use in the clinical setting., Conclusions and Relevance: Major barriers to the identification, validation, and introduction of routine biomarkers in the management of HS include lack of independent biomarker validation studies (especially assumption-free "omics"-based techniques); insufficient assessment of collinearity between identified or proposed biomarkers; and a lack of routine integration of biomarkers into the structure of clinical trials. International consensus among researchers, clinicians, and pharmaceutical stakeholders is required to standardize goals and methods and encourage biomarker integration into future HS clinical trials. This systematic review presents a number of priorities for near-term future research to overcome such barriers and limitations of biomarkers in HS.

Hidradenitis suppurativa (HS) is a debilitating chronic inflammatory skin disease that presents as exquisitely tender abscesses, draining fistulae, and sinus tracts. HS can lead to significant impairments in patients' quality of life, especially for children and adolescents who face challenges related to self-esteem and physical and emotional development. Severe long-term physical sequelae of inadequately treated HS include extensive scarring, urogenital strictures, immobility, and squamous cell carcinoma; emotional sequelae include depression, anxiety, and suicidal ideation. Many of the devastating long-term sequelae associated with HS can be prevented with early recognition and proper collaborative management. This article reviews strategies to aid pediatricians in early diagnosis of HS and provides clinical pearls for management and prevention of disease flares. [ Pediatr Ann . 2022;51(3):e123-e127.] .

Hidradenitis suppurativa (HS) is a severe chronic relapsing inflammatory disorder of the hair follicle unit that can cause painful abscesses, nodules, tunnels, and tracts in intertriginous parts of the body. The disease can often result in disfigurement and adversely impact patient quality of life. The management of HS has expanded significantly over the past decade to include multiple modalities, including topical therapies, systemic therapies (non-biologics and biologics), surgical therapies, lifestyle changes, and management of comorbidities. Management can often be clinically challenging and may involve the combination of medical and surgical approaches for optimal results. The purpose of this review is to present an update on non-biologic and non-interventional modalities published in 2019-2021 in the clinical management of HS. With emerging therapies, ongoing clinical trials, and heightened awareness about HS, there is hope that new treatment options will revolutionize the management of patients suffering from HS., (© 2022. The Author(s), under exclusive licence to Springer Nature Switzerland AG.)

Competing Interests: Conflicts of interest Dr Naik has received grant support from AbbVie, consulting fees from 23andme, advisory board fees from Invitrogen Biovitrum; has served as an investigator for Pfizer; and is a board member of the US Hidradenitis Suppurativa Foundation. Dr Alhusayen has received fees for participating in advisory boards for AbbVie and Janssen, has received consulting fees from Eli Lilly and Hidramed solutions, and is the President of the Canadian Hidradenitis Suppurativa Foundation. Author Ingram has received fees for participating in advisory boards for Viela Bio and Kymera Therapeutics; consulting fees from UCB Pharma, Novartis, ChemoCentryx, and Boehringer Ingelheim; editorial honorarium as BJD Editor-in-Chief; royalties as chapter author for UpToDate; and co-copyright holder for the HiSQOL, HS Patient Global Assessment, HS Investigator Global Assessment. He received travel expenses and a speaker's honorarium from UCB Pharma. Dr Lowes has received fees for participating in advisory boards for AbbVie, Janssen, Viela Bio and consulting fees from Incyte, BSN, XBiotech, Kymera, and Almirall and is the Vice President of the US Hidradenitis Suppurativa Foundation. Author Guilbault has received compensation for patient advisory board from Boehringer Ingelheim and is a board member of the US HS Foundation and Hope for HS. Author Vilumsen has received compensation for patient advisory board from Boehringer Ingelheim and consulting fee for UCB and is the President of Patientforeningen HS Danmark. Drs Frew and Marzano and Authors Paul and Yannuzzi have no conflicts of interest to declare.

Importance: Generalized pustular psoriasis (GPP) is a chronic, orphan disease with limited epidemiological data., Objective: To describe the clinical characteristics, treatments, longitudinal disease course, and disease-specific health care utilization among patients with GPP across the United States., Design, Setting, and Participants: A retrospective longitudinal case series involving 95 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for GPP and were treated at 20 US academic dermatology practices between January 1, 2007, and December 31, 2018., Main Outcomes and Measures: The primary outcome is to describe the patient characteristics, associated medical comorbidities, treatment patterns complications, and GPP-specific health care utilization., Results: Sixty-seven of 95 patients (70.5%) were women (mean age, 50.3 years [SD, 16.1 years]). In the initial encounter, 35 patients (36.8%) were hospitalized and 64 (67.4%) were treated with systemic therapies. In total, more than 20 different systemic therapies were tried. During the follow-up period, 19 patients (35.8%) reported hospitalizations at a median rate of 0.5 hospitalizations per year (IQR, 0.4-1.6). Women had a decreased risk of an emergency department or hospital encounter (odds ratio, 0.19; 95% CI, 0.04-0.83)., Conclusions and Relevance: Generalized pustular psoriasis is a rare, chronic disease without standard treatment and is associated with continued health care utilization over time.

Importance: Palmoplantar pustulosis (PPP) is a is a chronic, orphan disease with limited epidemiological data., Objective: To describe the clinical characteristics, treatments, longitudinal disease course, and health care utilization in adults with PPP across the US., Design, Setting, and Participants: This retrospective, longitudinal case series from 20 academic dermatology practices in the US included a consecutive sample of 197 adults who met the European Rare and Severe Psoriasis Expert Network consensus definition for PPP between January 1, 2007, and December 31, 2018. Data analysis was performed June 2020 to December 2020., Main Outcomes and Measures: The primary outcome was to describe the patient characteristics, associated medical comorbidities, treatment patterns, complications, and PPP-specific health care utilization., Results: Of 197 patients, 145 (73.6%) were female, and the mean (SD) age at presentation was 53.0 (12.6) years, with a mean (SD) follow-up time of 22.1 (28.0) months. On initial presentation, 95 (48.2%) patients reported skin pain, and 39 (19.8%) reported difficulty using hands and/or feet. Seventy patients (35.5%) were treated with systemic treatments, and use of more than 20 different systemic therapies was reported. In patients with at least 6 months of follow-up (n = 128), a median (IQR) of 3.7 (4-10) dermatology visits per year were reported; 24 (18.8%) patients had 5 or more visits during the study period., Conclusions and Relevance: In this case series, PPP was associated with persistent symptoms, continued health care utilization, and a lack of consensus regarding effective treatments, emphasizing the unmet medical need in this population. Additional research is necessary to understand treatment response in these patients.

During the COVID-19 pandemic, rapid, real-world evidence is essential for the development of knowledge and subsequent public health response. In dermatology, provider-facing and patient-facing registries focused on COVID-19 have been important sources of research and new information aimed at guiding optimal patient care. The 7 dermatology registries included in this update now include more than 8000 case reports sourced from physicians and patients from countries all over the world., Competing Interests: Disclosure Dr L.E. French is president of the ILDS. Dr E.E. Freeman is principal investigator of the AAD/ILDS COVID-19 Dermatology Registry. The ILDS provides financial support for the AAD/ILDS registry. Dr E.E. Freeman is an author for UpToDate. Drs S.R. Feldman, C.E.M. Griffiths, S.K. Mahil, H.B. Naik, and C.H. Smith have received funding or support from and Drs L.E. French, A.D. Irvine, and P.I. Spuls have been consultants for AbbVie; Dr M.A. Lowes has served on the advisory board for AbbVie. Drs S.R. Feldman, L.E. French, C.E.M. Griffiths, S.K. Mahil, R. Sinclair, and C.H. Smith have received funding or support from Novartis. Drs S.R. Feldman, C.E.M. Griffiths, R. Sinclair, C.H. Smith, and D. Wall have received funding from Pfizer. Drs S.R. Feldman, S.K. Mahil, and C.H. Smith have received funding from and Drs A.D. Irvine and P.I. Spuls have been consultants for Sanofi. Drs S.R. Feldman, C.E.M. Griffiths, A.D. Irvine, S.K. Mahil, R. Sinclair, and D. Wall have received funding or honoraria from Eli Lily; Drs S.R. Feldman, L.E. French, C.E.M. Griffiths, S.K. Mahil, R. Sinclair, and D. Wall have received funding or honoraria from Janssen. Drs S.R. Feldman and C.E. M. Griffiths have received funding or support from BMS, Almirall, Amgen, Galderma, LEO Pharma, Stiefel GSK, and Sun Pharmaceuticals. Drs C.E.M. Griffiths and S.K. Mahil have received funding or support from Celgene and UCB Pharma. Dr C.E.M. Griffiths has received honoraria and/or research grants from MSD and Sandoz. Dr S.R. Feldman received research, speaking and/or consulting support from Alvotech, Boehringer Ingelheim, Mylan, Ortho Dermatology, Samsung, Menlo, Helsinn, Arena, Forte, Merck, Regeneron, Novan, Qurient, National Biological Corporation, Caremark, Advance Medical, Suncare Research, Informa, UpToDate, and National Psoriasis Foundation. Dr S.R. Feldman is founder and majority owner of www.DrScore.com and is founder and part owner of Causa Research, a company dedicated to enhancing patients’ adherence to treatment. Dr L.E. French received speaking and/or consulting support from Galderma, Amgen, Leo Pharma, Biotest, and Pincell Srl. Dr H.B. Naik has received consulting fees from 23andme, Abbvie, and DAVA Oncology and advisory board fees from Boehringer Ingelheim and is an investigator for Pfizer. She also is an associate editor for JAMA Dermatology. Dr M.A. Lowes has served on the advisory boards for Abbvie, InflaRx, Janssen, and Viela Bio, and consulted for Almirall, BSN medical, Incyte, Janssen, Kymera, and XBiotech. Dr C.H. Smith is an investigator on Medical Research Council and Horizon 2020–funded consortia with industry partners (see psort.org.uk and biomap-imi.eu). Drs H.B. Naik (board member) and M.A. Lowes (vice president) are unpaid members of the US Hidradenitis Suppurativa Foundation. Drs L. Castelo-Soccio, K.M. Cordoro and I. Lara-Corrales are part of the PeDRA COVID-19 Response Task Force, a collaboration between the SPD and the PeDRA. Dr C. Flohr is President of the British Society for Paediatric Dermatology and Chief Investigator of the UK-Irish Atopic Eczema Systemic Therapy Register (A-STAR) (ISRCTN11210918). Dr C. Flohr and the patient-facing part of the SECURE-AD registry are supported by the National Institute for Health Research Biomedical Research Centre at Guy’s and St Thomas’ NHS Foundation Trust, London, UK. Dr A.D. Irvine is the unpaid Chairman of National and International Skin Registry Solutions (NISR) Ltd, a not-for-profit charity dedicated to developing registries for patients with skin disease. NISR supports the Secure-AD registry. Dr A.D. Irvine is a co–principal investigator on the A-STAR and has been a consultant or speaker for Regeneron and LEO Pharma outside of the scope of the current work. Dr P.I. Spuls has received departmental independent research grants from a few pharmaceutical companies for the TREAT NL registry, is involved in performing clinical trials with many pharmaceutical industries that manufacture drugs used for the treatment of conditions like psoriasis and atopic dermatitis, for which financial compensation is paid to the department/hospital, and is chief investigator of a systemic and phototherapy atopic eczema registry (TREAT NL) for adults and children and one of the main investigators of the SECURE-AD registry. Drs D. Wall and R. Sinclair colead and are members of the steering committee, along with Dr N. Meah, of the SECURE-Alopecia registry. Drs R. Sinclair, D. Wall, and N. Meah are leading the development of the Global Registry of Alopecia Areata Disease Severity and Treatment Safety. Dr D. Wall is an employee of the charity NISR. Dr R. Sinclair also reports serving as a consultant or paid speaker for or participating in clinical trials sponsored by LEO Pharma, Amgen, Merck & Co, Celgene Corporation, Coherus BioSciences, Regeneron Pharmaceuticals, MedImmune, GlaxoSmithKline, Cutanea, Samson Clinical, Boehringer Ingelheim, Merck Sharpe & Dohme, Oncobiologics, F. Hoffman–La Roche, and Bayer AG and serves as the current president of the Australasian Hair and Wool Research Society. G.C. Chamberlin and D.E. McMahon and Drs E.A. Balogh and A.V. Marzano have no conflicts of interest to declare., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: The impact of the COVID-19 pandemic on routine medical care may result in altered healthcare resource use in patients with immune-mediated conditions. The aim of this study was to determine the impact of treatment interruptions in patients with and without COVID-19 infections who were treated with targeted immunomodulators (TIMs) in the USA., Methods: Data from the IBM ® MarketScan ® Research Databases were analyzed in patients with immune-mediated conditions from January 1, 2018, through December 31, 2020. Healthcare resource use (HCRU) including hospitalizations, emergency department (ED) visits, in-person outpatient visits, and respiratory outcomes was assessed in a cohort of patients without COVID-19 who had uninterrupted versus interrupted TIM use. The impact of treatment interruption on HCRU and respiratory outcomes was also evaluated in a cohort of patients with COVID-19. Results from adjusted logistic regression were reported as adjusted odds ratios (aORs) with 95% confidence intervals., Results: Approximately 25% of patients in both the COVID-19 (N = 787) and non-COVID-19 cohorts (N = 77,178) experienced interruptions in TIM therapy. In the non-COVID-19 cohort, the likelihood of being hospitalized was 20% less in patients with uninterrupted versus interrupted TIM use (aOR = 0.80, 95% CI 0.71-0.90). Patients with uninterrupted TIM use had a similar likelihood of an ED visit (aOR = 0.99, 95% CI 0.91-1.08) and respiratory outcome (aOR = 0.97, 95% CI 0.71-1.31) versus patients with interrupted TIM use. The likelihood of having an in-person outpatient visit was 87% greater in patients with uninterrupted versus interrupted TIM use (aOR = 1.87, 95% CI 1.81-1.94). Similar findings were observed in the COVID-19 cohort., Conclusion: This analysis of real-world claims data showed that uninterrupted TIM use was not associated with an increased likelihood of hospitalizations, ED visits, or negative respiratory outcomes compared to interrupted TIM use among patients with immune-mediated conditions, regardless of COVID-19 diagnosis., (© 2021. The Author(s).)

High-quality dermatology patient registries often require considerable time to develop and produce meaningful data. Development time is influenced by registry complexity and regulatory hurdles that vary significantly nationally and institutionally. The rapid emergence of the coronavirus disease 2019 (COVID-19) global pandemic has challenged health services in an unprecedented manner. Mobilization of the dermatology community in response has included rapid development and deployment of multiple, partially harmonized, international patient registries, reinventing established patient registry timelines. Partnership with patient organizations has demonstrated the critical nature of inclusive patient involvement. This global effort has demonstrated the value, capacity, and necessity for the dermatology community to adopt a more cohesive approach to patient registry development and data sharing that can lead to myriad benefits. These include improved utilization of limited resources, increased data interoperability, improved ability to rapidly collect meaningful data, and shortened response times to generate real-world evidence. We call on the global dermatology community to support the development of an international federation of patient registries to consolidate and operationalize the lessons learned during this pandemic. This will provide an enduring means of applying this knowledge to the maintenance and development of sustainable, coherent, and impactful patient registries of benefit now and in the future., Competing Interests: Conflict of interest C.F. is Chief Investigator of the UK-Irish Atopic eczema Systemic TherApy Register (A-STAR). C.F., A.I., and P.S. co-lead the SECURE-AD register, which studies the impact of COVID-19 infection episodes on atopic dermatitis. D.W. and R.S. co-lead and are members of the Steering Committee, along with L.B., N.M., D.J.T., and K.Y., of the SECURE-Alopecia registry, which studies the impact of COVID-19 infection on patients with all forms of hair loss. R.S., D.W., N.M., K.Y., and L.B. are leading the development of Global Registry of Alopecia areata disease Severity and treatment Safety (GRASS). C.F., P.S., and C.A. are members of the international TREatment of Atopic eczema Taskforce (TREAT) Executive Committee. C.E.M.G. is Chief Investigator of the British Association of Dermatologists Biologics and Immunomodulators Register (BADBIR) and an Executive Member of the PsoProtect and Psoprotectme Registries. C.H.S. is Research Chair of BADBIR, and joint Principle Investigator (PI) of PsoProtect and PsoProtectMe Registries. S.K.M. is joint Principle Investigator of PsoProtect and PsoProtectMe Registries. I.L.C. is part of the Pediatric Dermatology Research Alliance COVID-19 Response Task Force, a collaboration between the Society for Pediatric Dermatology (SPD) and the Pediatric Dermatology Research Alliance (PeDRA). B.W.M.A. is a patient representative for the SECURE-AD patient register, and the Dutch TREAT NL and BioDAY registers. P.S. is member of the PsoProtect International Scientific Advisory Board. H.B.N is a board member of the Hidradenitis Suppurativa Foundation. R.A., J.R.I., and H.B.N. are members of the Steering Committee of the Global Hidradenitis Suppurativa COVID-19 Registry., (Copyright © 2021. Published by Elsevier Inc.)

Importance: Hidradenitis suppurativa (HS) in pediatric patients has been understudied. Increased awareness and recognition of HS prevalence in children demand efforts to better understand this condition., Objective: To describe the demographics, clinical features, treatment, associated comorbidities, and outcomes in a large cohort of pediatric patients with HS., Design, Setting, and Participants: International, multicenter, retrospective medical record review of pediatric patients (aged 1-18 years) with a clinical diagnosis of HS carried out in 10 dermatology clinics across the US, Canada, Israel, Australia, and Italy from January 1996 to January 2017., Main Outcomes and Measures: Patient demographics, clinical features, severity, associated comorbidities, and treatments in pediatric patients with HS., Results: This cross-sectional study included 481 patients diagnosed with HS. Overall, 386 (80%) were girls. The mean (SD) age of disease onset was 12.5 (2.9) years, and the mean (SD) age at diagnosis was 14.4 (3.5) years. Family history of HS was present in 111 of 271 (41%) patients. First signs/symptoms reported at disease onset were cyst/abscess in 229 of 481 (48%), pain/tenderness in 118 of 481 (25%), and papules/pustules in 117 of 481 (24%). At initial dermatologic assessment, 233 of 481 (48%) patients already had evidence of skin scarring. Disease severity (Hurley staging) was documented in 288 of 481 (60%) patients (47% stage 1, 45% stage 2 and 8% stage 3). Comorbid conditions were reported in 406 of 481 (85%) patients, the most common being obesity (263/406 [65%]) and acne vulgaris (118/406 [29%]). Complications occurred in 378 of 481 (79%) patients, the most common of which were scars or contractures (301/378 [80%])., Conclusions and Relevance: The findings of this study indicate that there is a gap in recognizing and diagnosing pediatric HS. Pediatric patients with HS are likely to present with other comorbidities. Prospective observational and interventional studies are needed to better understand clinical course and optimal treatments for pediatric HS.

Hidradenitis suppurativa is a chronic inflammatory disease that disproportionately affects women of childbearing age. Hidradenitis suppurativa is characterized by painful nodules, abscesses, draining dermal tunnels, and scarring with a predilection for intertriginous sites, such as the axilla, groin, and breast regions. Delay in diagnosis and treatment of hidradenitis suppurativa often results in long-term sequelae leading to significant morbidity, and rarely mortality, in these patients. This clinical opinion suggests that obstetrician-gynecologists are uniquely poised to recognize early signs of hidradenitis suppurativa during routine well-woman examinations and initiate treatment or referral to dermatology. Herein, we provide clinical pearls for obstetrician-gynecologists caring for female patients with hidradenitis suppurativa, including strategies for comprehensive management and recommendations to improve the comfort of patients with hidradenitis suppurativa during examinations., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Hidradenitis suppurativa (HS) is a highly prevalent, morbid inflammatory skin disease with limited treatment options. The major cell types and inflammatory pathways in skin of patients with HS are poorly understood, and which patients will respond to TNF-α blockade is currently unknown. We discovered that clinically and histologically healthy appearing skin (i.e., nonlesional skin) is dysfunctional in patients with HS with a relative loss of immune regulatory pathways. HS skin lesions were characterized by quantitative and qualitative dysfunction of type 2 conventional dendritic cells, relatively reduced regulatory T cells, an influx of memory B cells, and a plasma cell/plasmablast infiltrate predominantly in end-stage fibrotic skin. At the molecular level, there was a relative bias toward the IL-1 pathway and type 1 T cell responses when compared with both healthy skin and psoriatic patient skin. Anti-TNF-α therapy markedly attenuated B cell activation with minimal effect on other inflammatory pathways. Finally, we identified an immune activation signature in skin before anti-TNF-α treatment that correlated with subsequent lack of response to this modality. Our results reveal the fundamental immunopathogenesis of HS and provide a molecular foundation for future studies focused on stratifying patients based on likelihood of clinical response to TNF-α blockade.