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While the use of isotretinoin has revolutionized the treatment of acne vulgaris, isotretinoin is increasingly recognized as a useful therapeutic option for many other cutaneous conditions. We review the evidence underlying the use of isotretinoin for a variety of dermatological indications including hidradenitis suppurativa, sebaceous gland pathology, rosacea, scarring alopecia, cosmetic dermatology, and non-melanoma skin cancer prophylaxis amongst other uses, and thus consider alternative uses within dermatology practice. The studies found benefit of isotretinoin, however most trials lacked statistical power and in many cases the use was limited to case series. Isotretinoin, if used within the correct cohort with appropriate pretreatment counseling regarding side-effects, is a well-tolerated medication with potential as either an adjunctive treatment or a second-line agent in those recalcitrant cases unresponsive to first-line therapy.

We here report the case of a 58-year old nulliparous patient with a history of Niemann-Pick disease who presented with vulvoperineal, fluctuant, fistulized swelling measuring 10 cm associated with ulceration. The patient was febrile with impaired general state This mass caused some concern about necrotic and infected malignant tumor of the vulva associated with chronic dermatosis. Incision and purulent drainage associated with multiple biopsies excluded the presence of a neoplastic process. The diagnosis of suppurative hidradenitis or Verneuil's disease was based on the associations between lesions and their distributions. Then, the problem that arose was that of the choice between drug treatment and surgical procedures. This study, including a short review of the recent literature, aims to outline the diagnostic modalities, the complications and the therapeutic management of this disease., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts.

Importance: Hidradenitis suppurativa (HS) is a disease of the terminal hair follicle in apocrine gland-enriched skin areas, where immunobiology dysregulation of mesenchymal stem cells (MSCs) may have a key role., Objective: To investigate the MSC profile in patients with HS and in healthy controls., Design, Setting, and Participants: In this prospective case-control study, patients with HS were recruited from the Dermatological Clinic at the Department of Clinical and Molecular Sciences, Università Politecnica delle Marche, Ancona, Italy. Biopsy specimens were analyzed at the Histology Section of the Department of Clinical and Molecular Sciences. Participants included 11 patients with HS and 9 healthy controls, who were recruited into the study between January 20, 2015, and September 20, 2016, and underwent punch biopsy from axillary skin. None of the participants had received any antibiotics (systemic or topical therapy) within almost 12 weeks before the study., Main Outcomes and Measures: The immunophenotypic profile of MSCs was characterized following the minimal criteria established by the International Society for Cellular Therapy for the identification of MSCs. Levels of 12 cytokines belonging to helper T-cell subtypes 1, 2, and 17 pathways were examined on the secretome of isolated cells by enzyme-linked immunoabsorbent assay., Results: Skin MSCs were characterized in 11 patients with HS (8 women and 3 men; mean [SD] age, 35.8 [7.9] years) and 9 healthy controls (7 women and 2 men; mean [SD] age, 36.7 [6.9] years). The healthy controls were matched with patients with HS for body mass index. Mesenchymal stem cells isolated from patients with HS (HS-MSCs) and from healthy controls (C-MSCs) met the International Society for Cellular Therapy minimal criteria. Compared with C-MSCs, cytokine analyses of HS-MSCs revealed statistically significant overexpression of interleukin (IL) 6 (median [interquartile range {IQR}], 8765.00 [7659.00-9123.00] vs 2849.00 [2609.00-3001.00] pg/mL; P = .008), IL-10 (median [IQR], 29.46 [26.35-35.79] vs 21.36 [19.89-23.33] pg/mL; P = .004), IL-12 (median [IQR], 15.25 [13.27-16.25] vs 11.89 [10.73-12.33] pg/mL; P = .03), IL-17A (median [IQR], 15.24 [13.23-17.24] vs 11.24 [10.28-11.95] pg/mL; P = .008), tumor necrosis factor (median [IQR], 42.54 [42.20-43.94] vs 32.55 [31.78-33.28] pg/mL; P = .004), transforming growth factor β1 (median [IQR], 1728.00 [1535.00-1979.00] vs 500.80 [465.00-634.50] pg/mL; P = .004), and interferon γ (median [IQR], 11.49 [10.71-12.35] vs 9.45 [9.29-10.01] pg/mL; P = .005)., Conclusions and Relevance: Mesenchymal stem cells isolated from the skin of patients with HS seem to be activated toward an inflammatory status. The imbalance between proinflammatory and anti-inflammatory activities of MSCs favors the hypothesis of their pathogenic involvement in HS.

We have read with great interest the case report recently published in Acta Dermatovenerologica Croatica by Boer and Mihajlovic (1), which describes a 33-year-old woman with an 18-year history of classic hidradenitis suppurativa (HS) who developed HS-like lesions at the position of the bra strap. We agree with Boer and Mihajlovic: according to our clinical experience, we are convinced that mechanical stress may contribute or, in some cases, result in the development of HS. In this regard, we will describe the case of a patient with a monster hernia who presented HS-like lesions on his abdomen corresponding to the perihernial skin area, suggesting that mechanical stress was an important pathogenic factor for HS development. A 54-year-old man without any previous history of HS developed chronic, recurrent, inflammatory nodules, cysts, and depressed scars at the location of a giant abdominal hernia (Figure 1). The lesions occurred four years ago, six months after the hernia had reached its current size. Cultures from skin swabs showed commensal skin flora and moderate mixed anaerobic bacteria, as would be expected in HS lesions. No lesions occurred in other inverse areas generally affected by HS, such as the axillary and anogenito-crural regions. It is likely that the mechanical stress in the abdominal region was greater than the one occurring at the other folds due to the pressure of the trousers and belt. In fact, there are many kinds of mechanical stress: friction, pressure, pulling, tension, and pinching. Friction can be defined as the resistance to motion in the direction of the common boundary of the two surfaces. The body areas which are at greater risk for such frictional forces are mainly the skin folds, such as axillae, groins, and buttocks. There is a substantial difference between frictional and pressor stress: pressure is defined as the force per unit exerted parallel to the plane of interest. Pressure on the skin can be caused by tight dressings (2). Frictional and pressor forces can act concurrently and synergistically, starting the series of events that lead to clinical onset of HS, as most likely happened in our patient at the abdomen right below the hernia, where the trousers and belt are tightened. It is believed today that the primary event in HS is follicular inflammation: in predisposed subjects, folliculitis can develop into HS. Follicular enlargement has been observed in HS skin. It has been hypothesized that constant mechanical forces, such as pressure and friction, may lead to follicular occlusion, dilatation, microtears, and ruptures with ensuing abscesses (1-6). This case report corroborates observations from the last three decades: HS is a disease of the follicular epithelium rather than the apocrine glands (7,8), and mechanical stress, friction, and pressure may have an important role in its pathogenesis.

Aim: Hidradenitis suppurativa (HS) is a chronic, recurrent, inflammatory disease of the apocrine sweat glands. Tumor necrosis factor-alpha (TNF-α) inhibitors are commonly used to treat HS. However, prior to initiating therapy patients must be screened for mycobacterium tuberculosis (mTB) exposure. Several mTB screening tests based on interferon gamma release assays are commercially available, but the performance of these assays in the HS population is unknown. The purpose of this study was to investigate the performance of the QuantiFERON gold in-tube assay (QFT-GIT) in a cohort of patients with HS., Methods: This prospective study was conducted through the Wound Etiology and Healing (WE-HEAL) study. QFTGIT testing was performed using a commercial laboratory. Patients with positive test results underwent follow-up testing to evaluate for latent tuberculosis infection (LTBI). Data were collected on demographics and disease activity scores including Hurley stage, HS Sartorius score (HSS) and active nodule (AN) count., Results: Of the 69 patients with a confirmed diagnosis of HS, seven (10.1%) tested QFT-GIT positive and 5.8% were diagnosed with LTBI., Conclusions: QFT-GIT results did not correlate with demographic characteristics or HS disease activity.

Introduction: Hidradenitis suppurativa (HS) is a chronic, inflammatory, and debilitating skin disease, which usually occurs after puberty with painful, deep-seated, inflammatory lesions in the apocrine gland-bearing areas of the body. Several pharmacologic agents have been described to reduce lesion activity and inflammation in HS. However, conventional treatment may not always get the desired results. Therefore, unconventional therapies must be taken into account. Areas covered: Recently, the better understanding of HS pathogenesis has been used to improve treatment strategies with many emerging conventional and unconventional therapeutics options. Adalimumab is the only FDA-approved biologic available for therapy of moderate-to-severe HS. Nevertheless, novel therapeutic approach, including both topical and systemic as well as novel laser device, showed good clinical outcome. Several molecules, such as tumor necrosis factor-alpha (TNF-α), interleukin-1 (IL-1), IL-17, IL-12, IL-23, phosphodiesterase 4 (PDE4), lymphocyte function-associated antigen 1 (LFA-1), and complement component 5a (C5a), are modulated by such new biologic agents in HS. Expert commentary: In the next years, many therapeutic options for HS will be available. Clinical trials showed the efficacy of several biologic drugs, antibiotics, laser light device. Novel therapeutic options seem to be promising, but dermatologists will have to evaluate their effectiveness and safety in daily clinical practice.

Background: Disintegration of the infundibula of terminal hair follicles (HFs) in intertriginous skin areas exhibits the histological hallmark of hidradenitis suppurativa (HS)/acne inversa, featuring a dissecting terminal hair folliculitis. Elevated serum levels of interleukin (IL)-17 and local increase in the ratio of proinflammatory T helper (Th)17 cells and anti-inflammatory regulatory T cells (Tregs) have been reported. Perifollicular Tregs play a key role in HF stem cell homeostasis and infundibular integrity., Objectives: In this review, we evaluate the Th17/Treg ratio in HS, its aggravating conditions and associated comorbidities. Furthermore, we intended to clarify whether drugs with reported beneficial effects in the treatment of HS readjust the deviated Th17/Treg axis., Methods: PubMed-listed, peer-reviewed original research articles characterizing Th17/Treg regulation in HS/acne inversa and associated comorbidities were selected for this review., Results: This review presents HS as a disease that exhibits an increased Th17/Treg ratio. Perifollicular deficiencies in Treg numbers or function may disturb HF stem cell homeostasis, initiating infundibular dissection of terminal HFs and perifollicular inflammation. The Th17/Treg imbalance is aggravated by obesity, smoking and decreased Notch signalling. In addition, HS-associated autoimmune diseases exhibit a disturbed Th17/Treg axis resulting in a Th17-dominant state. All drugs that have beneficial effects in the treatment of HS normalize the Th17/Treg ratio., Conclusions: HS immunopathogenesis is closely related to deviations of the Th17/Treg balance, which may negatively affect Treg-controlled HF stem cell homeostasis and infundibular integrity. Pharmacological intervention should not only attenuate Th17/IL-17 signalling, but should also improve Treg function in order to stabilize HF stem cell homeostasis and infundibular integrity., (© 2018 British Association of Dermatologists.)

Purpose: Targeted, immune-modulating drugs are at the forefront of therapy for HS, and a comprehensive clinical trial registry is needed to facilitate data pooling and clinical efficacy comparison., Materials and Methods: A systematic review of the ClinicalTrials.gov database was searched for planned, in-progress, completed, or terminated trials investigating the effect of targeted biologic therapies for hidradenitis suppurativa (HS). When results of RCTs were not available, case reports or series were included., Results: Inflammatory mediators that are targeted by biologic agents include tumor necrosis factor-alpha (TNF-α), interleukin-1 (IL-1), IL-17, IL-12, IL-23, phosphodiesterase 4 (PDE4), lymphocyte function-associated antigen 1 (LFA-1), and complement component 5a (C5a). Clinical efficacy was measured by reduction in Sartorius score, Hidradenitis Suppurativa Clinical Response (HiSCR), Dermatology Life Quality Index (DLQI), or pain Visual Analog Scale (VAS). TNF inhibitors (adalimumab, etanercept, and infliximab), IL-1 receptor antagonist (Anakinra), IL-17A inhibitor (secukinumab), IL-12/23 inhibitor (ustekinumab), and PDE4 inhibitor (apremilast) show promise due to statistically significant improvements in disease severity., Conclusions: Currently, adalimumab is the only FDA-approved biologic available for the treatment of HS. However, results from trials of other biologic agents targeting downstream mediators are promising. Large-scale, randomized, placebo-controlled trials in patients with skin of color, as well as weight-based dosing trials, are needed.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease. This type of dermatosis with underlying chronic inflammation significantly affects the quality of life and may be accompanied by many comorbidities. In this case, Demodex spp. was associated with treatment-resistant and persistent course of skin diseases. A 46-year-old female patient applied to our clinic with complaints of lesions on the body and hip. Her dermatological examination revealed abscess formation and post-inflammatory pigmentation. Millimetric scar formation and improved folliculitis-like lesions were observed on both glutei. These complaints started 7 years ago and become more intense and severe by time. Owing to the diagnoses of diabetes mellitus, hypertension, and hyperlipidemia, the patient was monitored for metabolic syndrome. In the cultures taken from the lesions, no growth was seen. A standardized skin surface biopsy of the patient demonstrated demodicosis. The patient was treated with oral metronidazole and topical permethrin lotion, whereupon a pronounced recovery was observed in her clinical condition. In the light of this case, we recommend that patients with HS should be checked for the presence of Demodex spp., and if it is detected, an appropriate treatment should be applied. To our knowledge, this is the first case report presenting the relationship between HS and Demodex infestation.

Importance: In spite of progress in understanding the mechanisms underlying hidradenitis suppurativa (HS) as an inflammatory skin disease, there is still a demand for an overview on immunopathogenesis of HS., Objective: To demonstrate the importance of the type 1/type 17 immune response in lesional HS skin by drawing a semantic connectivity map., Design, Setting, and Participants: Single-center case series of 24 patients with HS. Association of HS with T helper 1/T helper 17 (TH1/TH17) phenotype was assessed using semantic map analysis., Main Outcomes and Measures: Association of HS with TH1/TH17 phenotype., Results: The analysis was performed on 24 lesional HS biopsy samples from untreated patients with HS (16 [67%] female; median age, 36.5 years [range, 21-51 years]) with a mean (SD) Hurley stage of 2.29 (0.62) and 9 punch biopsy samples from healthy controls (6 [67%] female; median age, 43 years [range, 23-66 years]). The map shows a clustering of all TH1/TH17-associated cytokines (interleukin 17 [IL-17], interferon γ, IL-12, IL-23, IL-32, IL-1β, tumor necrosis factor) around overall lesional inflammation. Tumor necrosis factor, IL-12, and IL-17 are even directly connected via interferon γ. In contrast, IL-13, a TH2-associated cytokine, was inversely correlated with the presence of TH1/TH17-associated cytokines, further highlighting the importance of the TH1/TH17 cytokines in HS pathogenesis., Conclusions and Relevance: These findings suggest that HS may be a TH1/TH17-driven inflammatory skin disease.

Introduction: Hidradenitis suppurativa is a chronic, inflammatory disease characterized by painful nodules, abscesses, and sinuses in the intertriginous areas, with significant associated comorbidities. The pathogenesis remains unclear, although advances have been made in understanding the disease process. Management of the disease is challenging, with a wide range of treatment options available with variable clinical response. Areas covered: This review discusses the most updated studies on the complex pathogenic mechanisms of hidradenitis suppurativa and the relevant literature on the current treatment options for this condition. Expert commentary: There is increasing evidence supporting the role of Th17 cells and enhanced expression of IL-17 and IL-1β, which represent potential targets for therapy. Bacteria and biofilms are likely contributory but secondary drivers of inflammation. There is also evolving evidence to suggest the presence of systemic comorbidities associated with HS, which underlie the importance of better understanding the pathogenesis and treatment of this disease.

Neutrophils constitute essential players in inflammatory responses and are the first line of defence against harmful stimuli. However, dysregulation of neutrophil homeostasis can result in excessive inflammation and subsequent tissue damage. Neutrophilic dermatoses are a spectrum of inflammatory disorders characterized by skin lesions resulting from a neutrophil-rich inflammatory infiltrate in the absence of infection. The exact molecular pathophysiology of neutrophilic dermatoses has long been poorly understood. Interestingly, neutrophil-rich cutaneous inflammation is also a cardinal feature of several autoinflammatory diseases with skin involvement, the latter being caused by aberrant innate immune responses. Overactivation of the innate immune system leading to increased production of interleukin-1 family members and 'sterile' neutrophil-rich cutaneous inflammation are features of both inherited autoinflammatory syndromes with skin involvement and an increasing number of neutrophilic dermatoses. Therefore, we propose that autoinflammation may be a cause of neutrophilic dermatoses., (© 2016 British Association of Dermatologists.)

Background: T-helper (Th)-17 lymphocytes and neutrophils are the main sources of the proinflammatory cytokines involved in the pathogenesis of hidradenitis suppurativa (HS)., Objectives: This study aims to evaluate the improvement of the inflammatory serum markers (ISM) levels in patients with moderate-to-severe HS who receive adalimumab., Methods: Nineteen moderate-to-severe HS patients were prospectively recruited. Each of the patients received 40 mg of adalimumab weekly. The ISM levels and modified Hidradenitis Suppurativa Score (mHSS) scores were assessed at baseline and at week 36. Nineteen healthy volunteers (HC) constituted the control group., Results: Before adalimumab treatment, the HS patients showed significantly increased levels of interleukin (IL)-6, IL-8, IL-10, IL-17A, soluble TNF receptor II (sTNF-RII), and C-reactive protein (CRP) as well as an increased erythrocyte sedimentation rate (ESR) (all P < .01). At week 36, the circulating levels of IL-1β, IL-6, IL-8, IL-10, IL-17A, soluble TNF receptor I (sTNF-RI), sTNF-RII, and CRP, as well as the ESR (all P < .05), decreased significantly in the HS patients who received adalimumab. The decrease in levels of IL-6 (r = 0.65, P = .003), IL-8 (r = 0.52, P = .024), sTNF-RI (r = 0.55, P = .015), and CRP (r = 0.47, P < .040) and the ESR (r = 0.60, P < .006) were significantly well correlated with clinical improvements according to the mHSS., Conclusions: Adalimumab improves the ISM-based systemic inflammatory burden in patients with moderate-to-severe HS. IL-6, IL-8, sTNF-RI and CRP and the ESR may serve as novel biomarkers for a therapeutic response., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Background: Two patient-reported outcome (PRO) questionnaires, the Hidradenitis Suppurativa Symptom Assessment (HSSA) and Hidradenitis Suppurativa Impact Assessment (HSIA), were developed to measure signs, symptoms and impacts of HS in treatment efficacy studies., Methods: In accordance with FDA guidelines and published best practices, four stages of research were conducted to create the questionnaires: concept elicitation, questionnaire construction, content evaluation and psychometric evaluation., Results: Subjects (N = 20) who participated in the concept elicitation stage reported 15 unique HS-related signs and symptoms and 51 impacts. Following this, eight sign and symptom concepts and 21 impacts were selected for construction of the HSSA and HSIA, respectively. During content evaluation, cognitive debriefing interviews with HS subjects (N = 20) confirmed subjects could read, comprehend and meaningfully respond to both questionnaires. Modifications made after this stage of work resulted in a nine-item HSSA and a 17-item HSIA. The HSSA and HSIA were subsequently entered into a US-based observational study (N = 40), and the scores produced by each were found to be reliable, construct valid, and able to distinguish among clinically distinct groups., Conclusions: The HSSA and HSIA are content-valid, HS-specific, PRO questionnaires with demonstrated ability to generate reliable, valid scores when administered to patients with HS in a research setting.

Rationale: Hidradenitis suppurativa (HS) is a chronic inflammatory condition characterized by recurrent swollen, deep, and painful abscesses. Several autoimmune conditions have been shown to be associated with HS including inflammatory bowel disease and spondyloarthropathies., Patient Concerns: 40-year-old female with systemic lupus erythematous (SLE) presented with recurrent abscesses and nodules on her extremities., Diagnosis: Early considerations related the described dermatologic findings to the dermatologic manifestations of SLE, however findings from lesion biopsy were suggestive of HS., Interventions: Prednisone and antibiotic therapy with clindamycin were started. Subsequently upon discharge, the patient was also treated with rifampicin and azathioprine., Outcome: In this communication, we demonstrate a case of HS in a patient with SLE that significantly improved under antibiotic and immunosuppressant therapy., Lessons: HS can coexist in patients with SLE. Evidence pertinent to the etiology of HS and its association with other autoimmune conditions implies a possible denominator in the disease etiopathogenesis. Increased awareness of the co-occurrence of the two conditions calls for increased efforts to devise better treatment modalities.

Acne and hidradenitis suppurativa (HS) both centre on hair follicles. They often occur together as part of the acne tetrad, but are found in distinct localizations. Acne is primarily defined by the presence of comedones and inflammatory lesions. However, in HS the intertriginous localization and chronicity play equally important roles for the diagnosis to the inflammatory lesions. Genetics, bacteria, environmental factors and innate inflammation have all been found to play a role in acne and/or HS. Surprisingly, there is little overlap between the findings so far. The genetics of acne and HS are distinct, bacteria have not been shown convincingly to play a role in HS, and the important risk factors obesity and smoking in HS cannot be easily translated to acne. The one driving factor central to both diseases is innate inflammation, most strikingly involving interleukin-1. Hence the interleukin-1 family, as already shown in autoinflammatory conditions associated with acne, could represent attractive treatment targets., (© 2018 British Association of Dermatologists.)

Hidradenitis suppurativa (HS) is a chronic and debilitating skin disorder characterized by the formation of painful abscesses, draining sinus tracts, and scarring, predominantly in skin folds such as the axillae and the perineum. The exact prevalence of HS is unknown because the disease is often underreported, but it has been estimated to be as high as 4.1%. HS is three times more prevalent in women than in men. Various comorbidities have been associated with HS, including inflammatory bowel disease and squamous cell carcinoma (SCC). The transformation of chronic HS to SCC is often considered the most severe complication of HS. The prevalence of SCC associated with HS is approximately 4.6%, and is more common among men. It is likely that the chronic irritation and inflammation characteristic of HS drive the malignant transformation to SCC, and recent work has identified other potential risk factors for this malignant transformation, including human papillomavirus (HPV) infection and tobacco use. Treatment modalities have expanded in recent years to include triple antibiotic therapy, immunomodulatory biologic agents, laser therapy, and surgical excision. Knowledge of these comorbid associations of HS, especially the malignant transformation to SCC, is highly important for the management and follow-up of this chronic disease.

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder characterized by recurrent nodules, abscesses, and sinus tracts that can be debilitating and significantly impair quality of life. Small studies and case reports have suggested a possible association between HS and inflammatory bowel disease (IBD)., Aims: We performed a case-control study to further characterize IBD patients with HS in terms of smoking status, BMI, sites affected by HS, IBD type and features, and IBD medication history., Methods: A total of 38 patients with HS and IBD were identified and matched on age, gender, and IBD type to 136 controls with IBD but not HS. Clinical characteristics of interest were obtained through extensive review of the electronic health record., Results: Among patients with HS and IBD, the most common sites affected by HS were the inguinal, perianal, and axillary regions. Relative to patients with IBD alone, patients with HS and IBD were nearly six times more likely to be current smokers (p < 0.01) and nearly 11 times more likely to be obese (p < 0.01). Patients with HS and Crohn's were significantly more likely to have ileocolonic and perianal disease than patients with CD only (OR 8.31, 95% CI 2.90-23.80 and OR 2.85, 95% CI 1.19-6.81, respectively; p < 0.01 for both)., Conclusions: Relative to patients with IBD who do not develop HS, patients with IBD and HS are more likely to be overweight or obese, to be former or current smokers, and to have ileocolonic and/or perianal disease.

Background: Hidradenitis suppurativa (HS) is a chronic inflammatory disease presenting as painful subcutaneous nodules, characterized by multiple abscess, inter-networking sinus tracts. We present the option of surgical treatment involving wide surgical excision and methods of reconstruction as well as the rate of recurrence., Method: This study reviewed 44 sites in 21 patients with moderate to extensive HS treated surgically in our hospital from 2000 to 2016, with a follow up of at least 24 months., Results: A total number of 44 operative procedures were performed during the study period with 13.6% (6 sites) involving axilla, 38.6% (17 sites) involving the gluteal area, 29.5% (13 sites) involving the perineal and perianal area and 11.4% (5 sites) involving the inguinal region, 4.5% (2 sites) involving the scrotal area, and 1.3% (one case) retrorectal abscess., Conclusion: Conservative treatment methods have little or no effects especially on gluteal, perineal/perianal and axillary hidradenitis suppurativa. The morbidity associated with the established disease is significant, and the only successful treatment is wide surgical excision., (Copyright © 2017. Published by Elsevier Ltd.)

Objectives: In recent years, Hidradenitis suppurativa has received increased attention but still lack well-defined, robust patient-reported outcome measures. Such measures are likely to be influenced by contextual factors. We, therefore, aim to describe the association of biological and other factors with HS patient's self-evaluated health as reflected by the EQ5D VAS score., Methods: This cross-sectional study measured basic information, Dermatology Life Quality Index, Major Depression Inventory and Euro-QoL-5D. Information obtained through questionnaires was used to create a model showing the relation between biological factors, questionnaire results and self-evaluated health using the stepwise method for linear regression. Patients with HS (n = 805) registered at our department were invited to participate. A total of 503 (62.5%) patients replied to this survey. There was no difference in basic demographics between responders and non-responders., Results: Possible predictors for this study were DLQI, MDI, BMI, smoking, gender. A final model for correlation with self-evaluated health was obtained. Significant predictors for the model were DLQI (P < 0.05), BMI points above 25 and active smoking (both: P < 0.01) and MDI (P < 0.001)., Conclusion: As this is a cross-sectional study, it reveals several clinically relevant correlations. Smoking appears to correlate with the highest change in VAS score, but an MDI of 10 (lower than study average) or a BMI of 40 influences the results more. This study provides an assessment of how much BMI and smoking, in relation to other factors, correlate to the self-evaluated health state of patients with HS. These may, therefore, be relevant contextual factors to the construction and interpretation of specific patient-reported outcome measures. To improve QoL, we advocate smoking cessation and weight loss, and advice active screening for depression as over 10% of our patients qualify for a depressive diagnosis according to ICD-10., (© 2017 European Academy of Dermatology and Venereology.)

Hidradenitis suppurativa is an inflammatory disease associated with subcutaneous nodules/abscesses that occur commonly in the axillary, inguinal, and perineal regions. We describe a case of a 64-year-old man presenting for F-FDG PET/CT for staging of a left vocal cord squamous cell carcinoma. The scan showed uptake in the left vocal cord malignancy and multiple hypermetabolic subcutaneous foci in the right axilla, right buttocks, and scalp in known locations of skin lesions related to hidradenitis suppurativa. This case illustrates an unusual inflammatory cause of F-FDG incidental uptake that should not be mistaken for metastases.

Importance: Hidradenitis suppurativa (HS) is relatively common, with the prevalence of 0.05% to 4.10%, yet many patients receive inadequate treatment., Objective: To review the diagnosis, epidemiology, and treatment of HS with an emphasis on advances in the last 5 years., Evidence Review: A literature search was conducted using PubMed, MEDLINE (Medical Subject Headings [MeSH]), and EMBASE to include recently published treatment studies (searched from September 1, 2011, to May 1, 2017). Reviews, guidelines, conference abstracts, and studies with less than 10 patients were excluded. Furthermore, internet searches for guidelines on hidradenitis suppurativa using Baidu, Bing, Google, and Qwant browsers were performed., Findings: The diagnosis of HS is made by lesion morphology (nodules, abscesses, tunnels, and scars), location (axillae, inframammary folds, groin, perigenital, or perineal), and lesion progression (2 recurrences within 6 months or chronic or persistent lesions for ≥3 months). HS is more common than was previously thought based on epidemiological analysis (0.05%-4.10%). Disability from HS can be significant. Patients with HS may have significant comorbidities (eg, obesity, metabolic syndrome, diabetes, and arthritis) and increased all-cause mortality (incidence rate ratio, 1.35 [95% CI, 1.15-1.59]). Antibiotic treatment with combinations of clindamycin and rifampicin, or ertapenem followed by combination rifampicin, moxifloxacin, and metronidazole for 6 months is effective. Adalimumab is effective in a significant proportion of patients and treatment with IL-1 and IL-12 receptor subunit beta 1 (Rb1) antibodies may also be useful. Tissue-sparing surgical techniques and carbon dioxide laser treatments also are available, but the evidence on clinical outcomes with these approaches is limited., Conclusions and Relevance: Hidradenitis suppurativa is more common than previously thought and may be treated by an array of pharmacological and surgical techniques. Hidradenitis suppurativa should be considered in the differential diagnosis of nodular lesions or sinus tracts present in the axillae, groin, perineal, and mammillary fold regions.

Hidradenitis suppurativa (HS) is a chronic, inflammatory, and debilitating disease of hair follicles with 1-4% prevalence and high morbidity. There is a dearth of information on the pathogenesis and immune dysregulation underlying HS; therefore, we carried out a detailed analysis of skin-infiltrating T cells. Cells isolated from skin biopsy samples and blood from HS patients and healthy control subjects were analyzed by 16-parameter flow cytometry to provide detailed profiles of CD4 T-cell subsets. We observed substantial infiltration of inflammatory T cells with a striking T helper (Th) type 17-skewed cytokine profile in HS skin; these cells expressed the Th17 lineage marker CD161 and IL-17, as well as proinflammatory cytokines GM-CSF, IL-22, IFN-γ, and tumor necrosis factor. Regulatory T cells were also enriched in HS lesional skin; however, the ratio of Th17 to regulatory T cells was nonetheless highly dysregulated in favor of Th17 cells. In contrast, lesional skin from anti-tumor necrosis factor-treated HS patients who showed substantial clinical improvement exhibited a significant reduction in the frequency of Th17 cells and normalization of the Th17 to regulatory T cell ratio. These data suggest that inhibition of pathogenic IL-17 via tumor necrosis factor blockade is associated with improvement in immune dysregulation in HS and may provide a rationale for targeting IL-17 in the disease., (Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.)

Hidradenitis suppurativa (HS) is a debilitating suppurative disease of the apocrine/follicular glands. Medical treatment has some efficacy in early-stage disease but is costly and requires frequent physician visits. Advanced disease usually requires surgical intervention. This treatment has not been well described in the literature. We sought to review our experience with HS treatment in a large surgical cohort. A retrospective review of 98 consecutive HS patients from 2000 to 2014 was performed. A two-stage operative approach was used. The first stage involved the removal of all the hair-bearing skin in the affected areas down to healthy tissue followed by the application of split-thickness skin grafts 48 to 72 hours later. Descriptive statistics were performed to describe the population and assess outcomes. The study population was young (36 ± 12 years) and predominately female (65, 66%). The patients were obese (body mass index, 36 ± 8.3 kg/m) and smokers (73, 73.7%). The majority presented with axillary disease (73%). There were 144 debridement procedures and 146 grafting procedures performed with a mean area grafted being 416 ± 500 cm (90-3400 cm). Only nine (9%) required regrafting, with the mean area regrafted being 140 ± 93 cm. Graft failure of less than 30% did not require regrafting. At 30 days after the initial procedure, 94.7% of all wounds were fully grafted and closed. Advanced stages of HS require ablation of the infected distorted glands for control of recurrent infections. Ablative excision of HS-affected skin and wound closure with split-thickness skin grafts is a safe, reliable, and effective therapy.

Background: A validated tool for the dynamic severity assessment of hidradenitis suppurativa/acne inversa (HS) is lacking., Objectives: To develop and validate a novel dynamic scoring system to assess the severity of HS., Methods: A Delphi voting procedure was conducted among the members of the European Hidradenitis Suppurativa Foundation (EHSF) to achieve consensus towards an initial HS Severity Score System (HS4). Strengths and weaknesses of HS4 were examined by a multicentre prospective study. Multivariate logistic regression, discriminant analysis and receiver operating characteristic curves, as well as examination for correlation (Spearman's rho) and agreement (Cohen's kappa) with existing scores, were engaged to recognize the variables for a new International HS4 (IHS4) that was established by a second Delphi round., Results: Consensus HS4 was based on number of skin lesions, number of skin areas involved and Dermatology Life Quality Index (DLQI), and was evaluated by a sample of 236 patients from 11 centres. Subsequently, a multivariate regression model calculated adjusted odds ratios for several clinical signs. Nodules, abscesses and draining tunnels resulted as the scoring variables. Three candidate scores were presented to the second Delphi round. The resulting IHS4 score is arrived at by the number of nodules (multiplied by 1) plus the number of abscesses (multiplied by 2) plus the number of draining tunnels (multiplied by 4). A total score of 3 or less signifies mild, 4-10 signifies moderate and 11 or higher signifies severe disease. Cohen's kappa was fair (κ = 0·32) compared with Hurley classification, and moderate (κ = 0·49) compared with Expert Opinion. Correlation was good (ρ > 0·6) with Hurley classification, Expert Opinion, Physician's Global Assessment and Modified Sartorius score, and moderate for DLQI (ρ = 0·36)., Conclusions: The novel IHS4 is a validated tool to dynamically assess HS severity and can be used both in real-life and the clinical trials setting., (© 2017 British Association of Dermatologists.)

Background: Pruritus is still a forgotten aspect of hidradenitis suppurativa (HS) and, to date, has never been adequately studied., Objective: The aim of this study was to determine the prevalence, and explore the characteristics, of pruritus in a well-defined cohort of HS patients., Setting: An academic hospital-based cross-sectional study in The Netherlands., Methods: A numerical rating scale (NRS, 0-10) was used to determine the prevalence of HS-related itch (NRS score ≥3). Candidate predictors for pruritus were subsequently determined using logistic regression models, and the impact of pruritus was assessed using a modified five-dimensional (5-D) itch scale. Associated serological and histological markers of pruritus were (semi-)quantitatively investigated in a subpopulation., Results: The prevalence rate of pruritus in 211 HS patients was 57.3%, with a mean NRS score of 6.1 ± 2.0. Patients with a pruritus NRS score ≥3 had more HS-affected body sites than patients with a score <3 (p < 0.001). The occurrence of a pruritus NRS score ≥3 was associated with Hurley III disease (odds ratio [OR] 7.73; p = 0.003) and pain (OR 1.34; p < 0.001). Pruritus affected sleep and activities of daily living (ADL) in the majority of cases, with an associated modified 5-D itch score of 13.7 ± 3.6 (on a scale from 5 to 25) in 52 HS patients. Histological examination showed eosinophilic granulocytes were present in 25% (2/8) of the perilesional skin and 63% (10/16) of the lesional skin, while a perineural infiltrate was found in 25% (2/8) and 69% (11/16) of the perilesional and lesional skin, respectively., Conclusion: Pruritus is a frequent but underreported symptom in patients with HS. Its moderate to severe intensity and significant impact on daily activities have great potential to impair patients' quality of life.

Recently, we could show that the expression levels of the key regulators of the microRNA (miRNA) maturation and transport were dysregulated in inflamed hidradenitis suppurativa (HS) tissue (Heyam et al. in Wiley Interdiscip Rev RNA 6:271-289, 2015). The RNA-induced silencing complex (RISC) is the central element of the miRNA pathway and regulates miRNA formation and function. We investigated the expression of the RISC components, namely transactivation-responsive RNA-binding protein-1 (TRBP1), TRBP2, protein activator (PACT) of the interferon-induced protein kinase R, Argonaute RISC Catalytic Component-1 (AGO1) and Component-2 (AGO2), metadherin, and staphylococcal nuclease and Tudor domain-containing-1 (SND1) in inflamed HS tissue compared to healthy and psoriatic controls by real-time reverse transcription polymerase chain reaction. Expression levels of all investigated components were significantly lower in lesional HS skin (n = 18) compared to healthy controls (n = 10). TRBP1, PACT, AGO1, AGO2, and SND1 expression levels were significantly down-regulated in lesional HS skin compared to healthy-appearing perilesional skin (n = 7). TRBP2 and SND1 expression levels were significantly lower in healthy-appearing perilesional skin compared to healthy controls. In lesional HS skin, expression levels of PACT, AGO1, and AGO2 were significantly lower compared to psoriatic skin (n = 10). In summary, our data showed that all investigated components of RISC are dysregulated in the skin of HS patients, providing support for the hypothesis that miRNAs may have a pathological role in the inflammatory pathogenesis of HS.

A 49-year-old woman with a past history of several infundibular cysts in the head and neck region presented with a 6-month history of a nonhealing perianal abscess on a background of 13 years of chronic sacral-buttock abscesses and discharging sinuses as part of hidradenitis suppurativa. After 2 surgical procedures and inadequate healing, a wide local excision was performed. The surgical specimen revealed an extensive, infiltrating, cystic and deeply penetrating, dermal and subcutaneous neoplasm with multiple fistulous tracts extending to the skin surface. The histopathology was consistent with carcinoma cuniculatum, a rare, slow growing, verucciform variant of squamous cell carcinoma. An entirely subcutaneous verrucous carcinoma of the sacrogluteal region is exceptionally rare, and this represents the first documentation of such a case in a female.

Background: Hidradenitis suppurativa is a chronic inflammatory cutaneous disorder that involves the infundibular terminal follicles in areas rich in apocrine glands and associated with the formation of abscesses and fistulating sinus. The clinical course is variable, and definitive evidence-based guidelines for the management are not well-established. Conservative treatment is controversial, and surgical excision is the recommended method. Herein, we aim to analyze the results of a modified conservative tissue preserving management protocol for stage I axillary hidradenitis suppurativa., Methods: A prospective cohort study of the results of 627 patients who were diagnosed and treated for stage I axillary hidradenitis suppurativa between January 2001 and December 2010 was done. Three hundred and forty nine (55.7%) were females, and 278 (44.3%) were males. The modified conservative treatment protocol consisted of three times per day washing the affected area with antibacterial soap, application of warm compresses for 10 min, followed by application of topical sodium fusidate 2% ointment (fusidic acid), with abstinence of armpits shaving during the treatment period., Results: Four hundred and sixty one (73.5%) patients had complete healing within two weeks, 127 (20.3%) within three weeks, 39 (6.2%) within four weeks. The recurrence rate was 5.9% (37 patients) in which all patients were retreated conservatively and had complete healing, none required surgical intervention., Conclusions: We conclude that hidradenitis suppurativa continues to be a challenging disease for patients and physicians. Tissue preserving conservative method using the fusidic acid ointment for treating stage I axillary hidradenitis suppurativa showed a promising result. Further research should be conducted on the optimal treatment regimen.

Clinical Question: Which treatments have been shown to be effective in randomized clinical trials (RCTs) for hidradenitis suppurativa (HS) in adults?, Bottom Line: There is high-quality evidence of benefit from adalimumab given weekly, while every other week dosing is ineffective, with reductions in Dermatology Life Quality Index (DLQI) scores compared with placebo of 2.8 points (95% CI, -3.7 to -2.0 points) and 1.6 points (95% CI, -3.9 to 0.6 points), respectively. Moderate-quality evidence suggests that infliximab is beneficial; RCT evidence for other interventions was lower in quality or absent, limiting further conclusions.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease defined by recurrent nodules, tunnels and scarring involving the intertriginous regions. Although patients with HS present a distinct symptomatology with a subsequent impairment of quality of life, an overall diagnostic time of seven years has been reported. A better and broader understanding of this disease may potentially reduce the diagnostic delay. This review provides an updated overview of diagnosis, monitoring tools as well as early treatment interventions.

Hidradenitis suppurativa (HS) has been associated with marked inflammatory perturbation. The mechanisms regulating the inflammatory network remain elusive. microRNAs (miRNAs) have been described as gene regulators of inflammation. We evaluated the messenger RNA (mRNA) expression levels of six selected inflammation-related miRNAs in lesional and perilesional skin samples of HS patients and in healthy controls. Samples of 15 HS patients and 10 healthy controls were included in this prospective study. Expression levels of the miRNAs miRNA-155-5p, miRNA-223-5p, miRNA-31-5p, miRNA-21-5p, miRNA-125b-5p, and miRNA-146a-5p were studied by quantitative real-time reverse transcription polymerase chain reaction. We observed a significant overexpression of miRNA-155-5p, miRNA-223-5p, miRNA-31-5p, miRNA-21-5p, and miRNA-146a-5p in lesional HS skin compared to healthy controls. Expression of these miRNAs was also significantly increased in lesional HS skin when compared to perilesional skin. Only miRNA-155-5p showed an increased expression in perilesional skin compared to healthy controls. In contrast, miRNA-125b-5p had a significantly lower expression in lesional HS skin compared to perilesional skin. We found that the studied inflammation-related miRNAs were significantly dysregulated in lesional HS skin and may have regulatory roles in the inflammatory process of HS. Given their predicted targets and functions, our findings point to these miRNAs as potential disease biomarkers, and manipulation might be used therapeutically to target the inflammatory pathway in HS.

Hidradenitis suppurativa (HS) is a chronic, debilitating disease with definitive treatment consisting of wide surgical excision of all affected tissue. Originally described in burns, Marjolin's ulcer (MU) has been described in a variety of wound types, including hidradenitis. HS patients often have long delays to diagnosis and management of their chronic wounds, leading to increased risk of MU. A retrospective review of our burn database was performed from 2008 to 2014. Seventy-two consecutive patients taken to the operating room for HS were retrospectively evaluated for demographic data, number of excisions, total area of excised skin, need for skin grafting, pathology results, and outcome. Fifty-eight percent of patients were male. Mean age was 36.88 ± 13.52 years. Mean size of excision was 743.71 ± 774.75 cm. Total number of operative procedures was 187, averaging 2.612 per patient, with 52% of patients undergoing skin grafting. Two patients had confirmed pathology of squamous cell carcinoma. Both were women with perineal HS and had metastatic disease on further workup. Both patients underwent chemotherapy and radiation with progression of their disease. The incidence of MU of 2.78% in this study is similar to the 2% incidence described historically in the burn population. All practitioners who treat HS patients should be aware of the devastating complication of MU arising in the chronic wound bed. We recommend that all excised tissue be sent to pathology for evaluation, and to consider early wide local excision of any chronically inflamed tissues to alleviate the risk of MU for this patient population.

A sporadic association between hidradenitis suppurativa and other diseases is reported in the literature, but few authors have described the association with Crohn's disease. Adalimumab is a fully human monoclonal antibody targeted at tumor necrosis factor alpha approved for the treatment of Crohn's disease and, recently, for active moderate to severe hidradenitis suppurativa in adult patients that do not respond adequately to systemic conventional treatment. We report an unusual case of a paradoxical effect of adalimumab in the onset of hidradenitis suppurativa in a 40-year-old woman during the treatment of Crohn's disease.

Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease defined by recurrent nodules, tunnels (sinus tracts) and scarring involving the intertriginous regions. The clinical course of HS is compatible with a biofilm-driven disease, and biofilm has been described in lesional HS skin. We therefore hypothesized that clinically unaffected HS skin would also have an increased presence of biofilm compared with that of healthy controls. We conducted a case-control study, investigating the morphology of the axillary skin microbiota. Peptide nucleic acid - fluorescence in situ hybridization probes were used in combination with confocal laser scanning microscopy. Significant differences were found in both distribution and quantity of the cutaneous microbiota in clinically non-affected axillary skin of patients with HS compared with healthy controls. Surprisingly, we detected fewer bacteria and less biofilm in patients with HS. The reduced microbiota in patients with HS may play an important role in the early course of the disease.

Hidradenitis suppurativa (HS) is a chronic relapsing suppurativa skin disease localized in the apocrine-gland-bearing areas of the body, predominantly in the axillae and groin. Although important risk factors have been identified, the pathomechanisms of the disease have not been fully understood. In the absence of proper treatment, chronic inflammation results in debilitating, and sometimes life-threatening complications. This severe and chronic disease can have detrimental effects on self-esteem and quality of life and is associated with high comorbidity, such as the metabolic syndrome and its components. Early detection and treatment in patients with HS may prevent late direct complications, such as severe scarring, and indirect complications, i.e. cardio-vascular disease. Thus appropriate screening and treatment should be prompt. No definitive treatment has been recognized, but several therapies have been found to reduce lesion severity and improve symptoms of HS. Additional research is needed for evaluation of the comparative effectiveness of topical and systemic treatments and for better understanding of the pathogenesis, natural history, and subtypes of HS.

Background: The etiology of hidradenitis suppurativa (HS) involves genetic and environmental factors. Well-established associated systemic factors are obesity, smoking, inflammation, and bacteria. Whether localized environmental factors such as friction and pressure may also play a causative role is still unclear., Objective: To describe the possible Koebner phenomenon (KP) in HS., Methods: The case notes on 14 patients with typical HS and additional HS lesions in previously uninvolved skin areas exposed to mechanical stress, e.g., on the abdomen at the level of the waistband, were reviewed with regard to the clinical characteristics of the cohort., Results: All 14 patients (7 females) were obese with a mean BMI of 35.8 (range 30.1-45.0). All patients developed additional ectopic HS lesions at the sites of friction on the convex abdomen. The ectopic lesions were clinically and histopathologically similar to HS., Conclusion: Mechanical stress factors or trauma play a role in obese patients with HS. It is suggested that these new induced lesions fit the concept of the KP. This phenomenon seems to occur in the so-called frictional furunculoid type of HS and may explain the unique distribution at certain localizations on the body of this follicular cutaneous eruption. Removal in an early stage of HS of external stress factors that can lead to the KP should be part of the treatment, especially in obese patients., (© 2017 S. Karger AG, Basel.)