Hidradenitis Suppurativa Research Articles

The Association Between Hidradenitis Suppurativa and Suicidal Ideation, Attempts, and Completion: A Systematic Review

The Association Between Hidradenitis Suppurativa and Suicidal Ideation, Attempts, and Completion: A Systematic Review

Comprehensive Catalog of Variants Potentially Associated with Hidradenitis Suppurativa, Including Newly Identified Variants from a Cohort of 100 Patients.

Hidradenitis suppurativa (HS) is a chronic skin disease characterized by painful, recurrent abscesses, nodules, and scarring, primarily in skin folds. The exact causes of HS are multifactorial, involving genetic, hormonal, and environmental factors. It is associated with systemic diseases such as metabolic syndrome and inflammatory bowel disease. Genetic studies have identified mutations in the γ-secretase complex that affect Notch signaling pathways critical for skin cell regulation. Despite its high heritability, most reported HS cases do not follow a simple genetic pattern. In this article, we performed whole-exome sequencing (WES) on a cohort of 100 individuals with HS, and we provide a comprehensive review of the variants known to be described or associated with HS. 91 variants were associated with the γ-secretase complex, and 78 variants were associated with other genes involved in the Notch pathway, keratinization, or immune response. Through this new genetic analysis, we have added ten new variants to the existing catalogs. All variants are available in a .vcf file and are provided as a resource for future studies.

Dermatologic Conditions in Down Syndrome: A Multi-Site Retrospective Review of International Classification of Diseases Codes.

As the population and life expectancy of people with Down syndrome increases, identifying common skin conditions throughout the lifespan will help inform clinical care and research. We sought to evaluate dermatologic conditions diagnosed in pediatric and adult patients with Down syndrome. This multicenter retrospective study analyzed demographic and outpatient visit International Classification of Diseases codes of patients with Down syndrome evaluated at outpatient dermatology clinics in the United States or Canada between 2011 and 2021. A total of 1529 patients with Down syndrome were identified from eight academic medical centers: 50.8% were children (0-12 years), 25.2% were adolescents (13-17 years), and 24% were adults (18 years and older). Eczematous dermatitis was the most common diagnosis overall (26%), followed by folliculitis (19.3%) and seborrheic dermatitis (15.6%). Other notable diagnoses included dermatophyte infections (13%), alopecia areata (11.6%), and psoriasis (6.7%). About 4.3% of visits included a code for high-risk medication use. Eczematous dermatitis, alopecia areata, and folliculitis were the most common diagnoses observed in children; folliculitis, hidradenitis suppurativa, and eczematous dermatitis in adolescents; and seborrheic dermatitis, eczematous dermatitis, and folliculitis in adults. Dermatologic conditions in patients with Down syndrome vary by age, but are most often eczematous, adnexal, and cutaneous autoimmune disorders. This multicenter retrospective review identifies skin diseases that should be prioritized for clinical care guideline development and research in the Down syndrome community.

Guidance for Circumflex Scapular Artery Flap Utilization in Pediatric Reconstruction.

The circumflex scapular artery (CSA) flap system, consisting of scapular, parascapular, and chimeric flaps, is useful for pediatric reconstruction in many anatomical locations. The objectives of this case series are to offer insights into our decision-making process for selecting the CSA flap in particular pediatric reconstructive cases and to establish a framework for choosing a scapular or parascapular skin paddle. We also aim to emphasize important technical considerations of CSA flap utilization in pediatric patients. Pediatric reconstruction with CSA flaps performed at our institution between 2006-2022 was retrospectively reviewed. Patient demographics, indications, flap characteristics, complications, and operative data were abstracted. Functional donor site morbidity was assessed through postoperative physical examinations. Unpaired t test analyzed scapular versus parascapular flap size. Eleven CSA flaps were successfully performed in 10 patients (6 scapular and 5 parascapular flaps). Patient ages ranged from 2 to 17 years. Scapular fasciocutaneous free flaps (n = 4) were performed in patients' ages 2-5 years for hand and forearm scar contractures. Two pedicled scapular flaps were performed for a single patient for bilateral axillary hidradenitis suppurativa. The 5 parascapular flaps were performed in patients' ages 2-14 years for calcaneus and forearm avulsion wounds and reconstruction after resection of hidradenitis suppurativa, nevus sebaceous, and Ewing sarcoma. In the sarcoma resection case, a chimeric flap with latissimus dorsi was employed. Average flap size was 101.6 ± 87.3 cm2 (range: 18-300 cm2). Parascapular flaps were significantly larger than scapular flaps (156.60 ± 105.84 cm2 vs 55.83 ± 26.97 cm2, P = 0.0495). Overall, 3 complications occurred (27.3% of cases) including venous congestion (n = 2) and wound dehiscence (n = 1). There were no reported cases of compromised shoulder function at 1.9 ± 2.5-year follow-up. The successful reconstruction rate for scapular, parascapular, and chimeric flaps was 100%. The CSA flap treated a wide variety of indications demonstrating the flap's attributes: large vessel caliber, wide arc of rotation, reliable vascular anatomy, minimal donor site morbidity, and ability to incorporate bone and muscle. Our cases also highlight important pediatric considerations such as vascular mismatch and limited scapular bone stock. We recommend selection of the parascapular over the scapular flap with reconstruction of larger, complex defects given its ability to be harvested with a large skin paddle.

Comprehensive Codified Algorithms to Identify the Underestimated Burden of Hidradenitis Suppurativa in the United States.

Hidradenitis suppurativa (HS) is a painful, inflammatory skin disease associated with a high disease burden and long diagnostic delay. Prevalence estimates of HS vary widely in the literature owing to differing estimation methodologies. This study aimed to apply stepwise algorithms to estimate the prevalence of possible/diagnosed cases of HS in the US. This was a retrospective cohort study in adult and pediatric patients with HS which utilized data from four US databases (MarketScan [Medicare and Medicaid] andOptum [electronichealth record (EHR) andClinformatics Data Mart (CDM)]). Patients withpossible/diagnosed HS were identified using two algorithms (termed Algorithm 1 and Algorithm 2), which assessed symptoms such as multiple skin boils in site-specific areas based on international classification of disease (ICD) codes. Patients with diagnosed HS were defined as having ≥ 2 outpatient or ≥ 1 inpatient diagnosis codes of HS. In each database, patients with continuous medical and pharmacy benefits in the 365days pre-index and 0-365days post-index periods were eligible for inclusion. Across all databases, Algorithm 2 (MarketScan Medicare [N = 309,916]; MarketScan Medicaid [N = 188,783]; Optum EHR [N = 366,158]; Optum CDM [N = 173,812]) identified more patients with possible/diagnosed HS than Algorithm 1 (MarketScan Medicare [N = 194,353]; MarketScan Medicaid [N = 99,276]; Optum EHR [N = 177,957]; Optum CDM [N = 112,244]). Based on ICD-9/10 codes, the 5-year period prevalence of HS ranged from 0.06% to 0.12% across all databases, while for Algorithm 1 and Algorithm 2, this ranged from 0.27% to 0.41% and 0.49% to 0.78%, respectively. Adults and females generally had a higher 5-year period prevalence versus pediatric patients and males, respectively. This real-world study highlights that HS diagnosis codes alone may be insufficient to estimate the prevalence of HS, demonstrating the value of employing algorithms in practice which assess for parameters such as multiple skin boils in site-specific areas. Integrating robust methods to identify the prevalence of HS may improve the diagnostic delay observed in HS and improve treatment outcomes.

Follicular Skin Disorders, Inflammatory Bowel Disease, and the Microbiome: A Systematic Review.

Follicular skin disorders, including hidradenitis suppurativa (HS), frequently coexist with systemic autoinflammatory diseases, such as inflammatory bowel disease (IBD) and its subtypes, Crohn's disease and ulcerative colitis. Previous studies suggest that dysbiosis of the human gut microbiome may serve as a pathogenic link between HS and IBD. However, the role of the microbiome (gut, skin, and blood) in the context of IBD and various follicular disorders remains underexplored. Here, we performed a systematic review to investigate the relationship between follicular skin disorders, IBD, and the microbiome. Of the sixteen included studies, four evaluated the impact of diet on the microbiome in HS patients, highlighting a possible link between gut dysbiosis and yeast-exclusion diets. Ten studies explored bacterial colonization and HS severity with specific gut and skin microbiota, including Enterococcus and Veillonella. Two studies reported on immunological or serological biomarkers in HS patients with autoinflammatory disease, including IBD, and identified common markers including elevated cytokines and T-lymphocytes. Six studies investigated HS and IBD patients concurrently. Our systematic literature review highlights the complex interplay between the human microbiome, IBD, and follicular disorders with a particular focus on HS. The results indicate that dietary modifications hold promise as a therapeutic intervention to mitigate the burden of HS and IBD. Microbiota analyses and the identification of key serological biomarkers are crucial for a deeper understanding of the impact of dysbiosis in these conditions. Future research is needed to more thoroughly delineate the causal versus associative roles of dysbiosis in patients with both follicular disorders and IBD.

Differentiating clinical characteristics of perianal inflammatory bowel disease from perianal hidradenitis suppurativa

AbstractBackgroundPerianal draining tunnels in hidradenitis suppurativa (HS) and perianal fistulizing inflammatory bowel disease (IBD) present diagnostic and management dilemmas.MethodsWe conducted a retrospective chart review of patients with perianal disease evaluated at Mayo Clinic from January 1, 1998, through July 31, 2021. Patients' demographic and clinical data were extracted, and 28 clinical features were collected. After experimenting with several machine learning techniques, random forests were used to select the 15 most important clinical features to construct the diagnostic prediction model to distinguish perianal HS from fistulizing perianal IBD.ResultsA total of 263 patients were included (98 with HS, 100 with IBD, and 65 with both IBD and HS). Patients with HS had a higher mean body mass index, a higher smoking rate, and more commonly showed cutaneous manifestations of tunnels and comedones, while fistulas, abscesses, induration, anal tags, ulcers, and anal fissures were more common in patients with IBD. In addition to having lesions in the perianal area, patients with IBD often had lesions in the buttocks and perineum, while those with HS had additional lesions in the axillae and groin. Among the statistically significant features, the 15 most important were identified by random forest: fistula, tunnel, digestive symptom, knife‐cut ulcer, perineum, body mass index, age, axilla, abscess, tags, smoking, groin, genital cutaneous edema, erythema, and bilateral/unilateral.ConclusionsThe results of this study may help differentiate perianal lesions, especially perineal HS and fistulizing perineal IBD, and provide promise for a better therapeutic outcome.

Rationale of Patient-Initiated Loss to Follow Up in Hidradenitis Suppurativa Patients

Introduction: Hidradenitis Suppurativa (HS) is a chronic inflammatory skin condition with a predilection for African American and female patients. HS consists of recurrent abscesses and sinus tracts in intertriginous areas. This condition has debilitating medical and psychosocial consequences, with treatment of severe cases remaining difficult. This may lead to patient frustration and lack of follow-up. This study aims to explore the rationale of HS patient-initiated loss to follow-up. Methods: We identified 72 patients at a single center with dermatologist-diagnosed HS seen within the last 12 months (06/30/2021-06/30/2022). Of those 72 patients, 35 were determined to be severe. Of those 35 patients, 11 were not seen within the last 6 months and thus lost to follow-up. These individuals were polled to explore the rationalization of discontinued treatment. Results: Of the 11 patients with severe HS that were lost to follow up, 4 did not respond after three attempts at contact. Of the remaining 7 patients, 3 (27. 27%) reported discontinued care due to scheduling conflicts, 1 (9.09%) had improvement of symptoms, 1 (9.09%) lost medical coverage, 1 (9.09%) cost, and 1 (9.09%) discontinued care due to a competing comorbidity. Discussion: This study determined that of the 7 patients with severe HS that were reached, the largest contributor to patient-initiated loss to follow-up were scheduling conflicts. Thus, the results reported in this study indicate a need for further research into the proactive dismantling of barriers preventing patients from receiving adequate and effective care.

Characterization of Disparities, Diagnoses and Treatment Outcomes with Pemphigus Vulgaris

Pemphigus vulgaris (PV) and foliaceus (PF) are classified as rare autoimmune blistering disorders in which IgG antibodies destroy desmosome proteins desmoglein 1 and desmoglein 3 within the stratum spinosum of the epithelial layer of the skin. These disorders once used to be a fatal condition, but the advent of steroids and immunosuppressants has made these diseases manageable. Such treatments, however, only alleviate symptoms and have not been viable in allowing patients to achieve prolonged steroid-free remission. In 2018, the US Food and Drug Administration (FDA) approved the anti-CD20 monoclonal antibody rituximab as the first-line treatment for moderate to severe cases of pemphigus. While rituximab has been generally successful in treating this disease and has led to prolonged steroid-free remission in many patients, treatment disparities and differences in patient outcomes due to social determinants of health have not yet been studied in the context of PV and PF. Such studies have already been conducted with more common dermatological disorders such as hidradenitis suppurativa (HS), which show significant evidence of outcome disparities due to social factors such as race, ethnicity and socioeconomic status. However, such studies have yet to be conducted with pemphigus likely because of how rare the disease is and because the current first-line treatment was FDA approved recently in 2018. The aim of this study is to conduct a retrospective multivariable analysis of 100 patients with pemphigus from UT Southwestern Medical Center and Parkland County Hopital to determine which social factors are the most responsible for any discovered pemphigus treatment outcome disparities. The impacts of this study are far-reaching as any discovered disparities can alert dermatologists to place a special emphasis to address any social factors that may play a role in leading to treatment outcome differences for patients with pemphigus.

Real-world data about secukinumab in hidradenitis suppurativa: tobacco pack years an underestimated factor

Real-world data about secukinumab in hidradenitis suppurativa: tobacco pack years an underestimated factor

Pathogenesis of Inflammation in Skin Disease: From Molecular Mechanisms to Pathology.

Many skin diseases begin with inflammatory changes on a molecular level. To develop a more thorough understanding of skin pathology and to identify new targets for therapeutic advancements, molecular mechanisms of inflammation in the context of skin disease should be studied. Current research efforts to better understand skin disease have focused on examining the role of molecular processes at several stages of the inflammatory response such as the dysregulation of innate immunity sensors, disruption of both transcriptional and post-transcriptional regulation, and crosstalk between immune and neuronal processes (neuro-immune crosstalk). This review seeks to summarize recent developments in our understanding of inflammatory processes in skin disease and to highlight opportunities for therapeutic advancements. With a focus on publications within the past 5 years (2019-2024), the databases PubMed and EBSCOhost were used to search for peer-reviewed papers regarding inflammatory molecular mechanisms and skin disease. Several themes of research interest regarding inflammatory processes in skin disease were determined through extensive review and were included based on their relative representation in current research and their focus on therapeutic potential. Several skin diseases such as psoriasis, atopic dermatitis, hidradenitis suppurativa, and scleroderma were described in the paper to demonstrate the widespread influence of inflammation in skin disease.

Bimekizumab for the treatment of hidradenitis suppurativa.

Hidradenitis suppurativa (HS) is a painful, inflammatory dermatosis involving recurrent abscesses, nodules and tunnels in intertriginous regions. Biologics and other immunomodulators have significantly expanded the treatment options available for HS. Bimekizumab is a monoclonal antibody targeting both interleukin-17A and interleukin-17F, key mediators of inflammation, that is already approved for psoriasis, psoriatic arthritis and axial spondylarthritis. It is currently pending FDA review for HS treatment but has already received marketing authorization for this indication in Europe. This review aims to explore drug-specific characteristics of bimekizumab including its mechanism of action, pharmacokinetics and pharmacodynamics and the current state of the literature regarding its use in HS such as safety, efficacy and dosing, while highlighting its implications in clinical practice. Recent Phase II and III trial data demonstrating positive efficacy and safety profiles in the treatment of HS will also be detailed.

Precocious Puberty Among Children and Adolescents With Hidradenitis Suppurativa

This case-control study examines the association of precocious puberty with hidradenitis suppurativa among US children and adolescents.

Trends of Metformin Use in Patients with Hidradenitis Suppurativa, 2016-2021

Introduction: Hidradenitis Suppurativa (HS) is a dermatologic condition that is comorbid with diabetes mellitus (DM) and polycystic ovarian syndrome (PCOS). Metformin has been used as a therapy in DM and PCOS but little research has been done to see if it is being used in patients with HS. Methods: Using MarketScan, information on HS, DM, and PCOS diagnoses were collected from January 1, 2016--December 31, 2021. Metformin prescription information was also collected on these patients. Results: During the study, the percentage of HS patients with a metformin prescription increased (Z = -9.6, p<0.001). Metformin prescriptions in patients with HS but without DM or PCOS diagnoses also increased (Z= -13.1, p<0.001). Discussion: Metformin offers promising therapeutic options for the treatment of patients with HS, even if they do not have comorbid conditions.

Coalescing Tender Nodules on the Anterior Chest: An Atypical Presentation of Hidradenitis Suppurativa

Hidradenitis suppurativa (HS) is an inflammatory skin condition that is characterized by inflamed nodules and abscesses that can form sinus tracts under the skin, typically in skin fold areas, that are tender and drain pus. Efficient diagnosis and early intervention is critical for this condition, as initiating treatment can keep the lesions under control and prevent worsening of nodules that can cause significant discomfort and permanent scarring. Here, we present a case of an unusual presentation of hidradenitis suppurativa in a 41-year-old male who presented to the emergency department with a chief complaint of a tender, purulent anterior chest cutaneous mass, which pathology later confirmed was consistent with an HS lesion. While HS typically occurs in regions with skin folds, this case illuminates an atypical presenting area – the anterior chest wall. Thus, clinicians should keep HS on the differential for such lesions, as the location of the affected area should not rule in or out HS as a diagnosis.

Demographic Data, Risk Factors, and Disease Burden of HS Patients in Lithuania at a Reference Center.

Hidradenitis suppurativa (HS) diagnosis often faces a global delay of 7.2 years due to factors like lack of recognition, stigma, and socioeconomic barriers. Limited effective therapies and frequent exacerbations impact patients' quality of life, posing a significant burden on healthcare systems. HS patients were assessed according to European Hidradenitis Suppurativa Foundation (EHSF) Registry questionnaire guidelines at various stages of the disease and treatment. The study included 49 patients; 57.14% (n = 28) of them were male. The average age of the subjects was 39.91 ± 13.665 years; the average BMI was 27.84 ± 7.362. A total of 59.18% (n = 29) were active or previous smokers. There were statistically more male smokers than female (p < 0.01). Average disease onset was 25.71 ± 13.743 years; the mean time to diagnosis was 5.2 ± 7.607 years. A total of 70.2% (n = 33) were previously misdiagnosed. Subjects had 6.17 ± 6.98 painful days over the preceding 4 weeks. The average intensity of pain according to the visual analogue scale (VAS) was 5.60 ± 3.36 points. The mean dermatology life quality index (DLQI) at baseline was 8.9 ± 7.436. The research revealed delayed diagnoses, especially for females. Smoking was linked to higher Hurley stages, with a prevalence among male smokers, and HS had a substantial impact on patients' quality of life.

Monoclonal antibodies for inflammatory, autoimmune and oncological skin diseases

In 1997 rituximab, agenetically engineered chimeric monoclonal antibody (mAb) targeting CD20 expressed on Bcells was approved for treatment of non-Hodgkin's lymphoma. Since then, pharmacological improvements combined with increased knowledge on the immunopathogenesis of diseases led to the development of specific mAb targeting different antigens (e.g., interleukins or transmembrane receptors). This approach reshaped the therapeutic methodology in many fields, including dermatology. Nowadays, the treatment of frequent and possibly impairing inflammatory disorders such as psoriasis, atopic dermatitis or hidradenitis suppurativa have different mAbs approved for both adult and pediatric patients. This class of drugs often shows amore favorable outcome and abetter safety profile than routine immunosuppressants, such as steroids and steroid-sparing substances. For many years mAbs also represented apillar of oncological treatment for severe diseases such as malignant melanoma or Merkel cell carcinoma. This review summarizes the current knowledge on already approved and promising new mAbs for the treatment of inflammatory and oncological skin diseases.

Microenvironmental host-microbe interactions in chronic inflammatory skin diseases.

Several microbiome studies have recently demonstrated microbial dysbiosis in various chronic inflammatory skin diseases, and it is considered an important role in the pathogenesis. Although the role of skin dysbiosis in inflammatory skin diseases is debatable, the local microenvironment is considered essential concerning compositional changes and functional alterations of the skin microbiota. Indeed, various local nutrients (e.g., lipids), pH values, water, oxygen, and antimicrobial peptides may affect the level of skin dysbiosis in these skin diseases. In particular, in atopic dermatitis and hidradenitis suppurativa, significant changes in skin dysbiosis have been associated with local aberrant host immune changes. In this review, the potential pathogenic crosstalk between the host and the microbiota is reviewed in relation to the physical, chemical, and biological microenvironments of various chronic inflammatory skin diseases.

Uncommon neoplasms mistakenly diagnosed as hidradenitis suppurativa: Report of three consecutive cases

Hidradenitis Suppurativa (HS) poses diagnostic challenges due to its clinical overlap with various skin conditions and neoplasms, potentially leading to misdiagnoses. The absence of a definitive diagnostic test and infrequent use of histopathology contribute to diagnostic complexities, exacerbated by the recent increased focus on HS. Three cases initially diagnosed and treated as HS underwent through clinical work-up and skin biopsies to resolve diagnostic complexities. Initially labelled as HS, the cases revealed a breast carcinomaon axillary ectopic tissue, a cutaneous gamma-delta T-cell lymphoma, and an infiltrating squamous cell carcinoma. Delayed recognition led to misguided therapies and adverse outcomes.This report stresses the need to explore alternative diagnoses for chronic skin nodules with or without ulcerations on the flexures. Timely skin biopsies are crucial for accurate diagnoses. Ongoing clinician education is essential to avoid misdiagnosis in challenging cases, where histopathology aids in reaching a correct diagnosis.

To what Extent does Vitamin D and its Serum Levels Influence the Severity of Hidradenitis Suppurativa: A Literature Review.

Vitamin D plays a role in inflammatory skin conditions and can improve them. Hidradenitis suppurativa (HS) is an autoinflammatory chronic skin disease in which most patients exhibit a hypovitaminosis D. However, it is uncertain whether vitamin D supplementation could relieve the severity of HS. A systematic literature search of PubMed and Web of Science was conducted on 4 September 2023. Studies that investigated vitamin D and its potential implications for the severity of HS were included. In contrast, studies that focused on the prevalence of vitamin D deficiency were excluded, as well as studies on syndromic HS. Seven studies with a total of 575 patients were included in the qualitative synthesis, of which 3 utilized a cross-sectional design, 2 were pilot studies, 1 a controlled cohort study, and 1 a prospective case-control study. In all included studies, HS patients were vitamin D deficient. There was evidence indicating that serum vitamin D levels negatively correlated with the severity of the disease, and at least suggestive evidence that vitamin D supplementation could have a positive impact on the course of HS. To better understand these correlations, conducting a randomized controlled trial study on vitamin D and its effects on HS severity is imperative.