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Zagelbaum Ward NK, Jun JA, Vecerek N, Donaldson M, Quismorio FP. Dissecting cellulitis of the scalp associated with peripheral and axial spondyloarthritis: report of a case and review of the literature. Clin Rheumatol 2022; 41:2553-2560. [PMID: 35460009 DOI: 10.1007/s10067-022-06128-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2022] [Revised: 03/04/2022] [Accepted: 03/07/2022] [Indexed: 11/29/2022]
Abstract
Dissecting cellulitis of the scalp (DCS) is a rare, primary neutrophilic cicatricial alopecia of unknown etiology. The disease follows a chronic, relapsing, and remitting course which may ultimately lead to scar formation and alopecia. The association of seronegative peripheral and/or axial spondyloarthritis in patients with hidradenitis suppurativa (HS) and acne conglobata (AC) is well established. However, the occurrence of spondyloarthropathy in patients with either isolated or combined DCS is relatively rare and therefore underrecognized by clinicians. We report a patient with DCS with inflammatory peripheral arthritis and asymptomatic radiographic sacroiliitis. Using PubMed, Ovid, and Google scholar, we searched for case reports of inflammatory arthritis in HS, AC, and DCS in the English literature from 1982 to present. We identified 12 patients with DCS who had associated spondyloarthropathy with adequate clinical details for a systematic analysis. We outline key clinical features, radiographic findings, and treatment utilized for these patients. Seronegative axial and peripheral spondyloarthritis may occur in the setting of isolated DCS as well with concomitant HS and AC. The inflammatory arthritis often develops during acute flares of the cutaneous disease. Choosing optimal drug therapy may be challenging. Current options include anti-TNF-α medications, which have been reported to be effective for both the cutaneous lesions and the associated spondyloarthritis. The complex pathophysiology of the conditions that comprise the follicular occlusion triad warrants further research into the potential role of additional biologic agents.
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Affiliation(s)
- Nicole K Zagelbaum Ward
- Division of Rheumatology, Department of Medicine, and Division of Dermatology, Keck School of Medicine, University of Southern California and Los Angeles County, Los Angeles, CA, USA. .,Division of Rheumatology, Department of Medicine, Keck School of Medicine, University of Southern California and Los Angeles County + University of Southern California (LAC+USC) Medical Center, Los Angeles, CA, USA.
| | - Jenny Ann Jun
- Division of Rheumatology, Department of Medicine, and Division of Dermatology, Keck School of Medicine, University of Southern California and Los Angeles County, Los Angeles, CA, USA.,Division of Rheumatology, Department of Medicine, Keck School of Medicine, University of Southern California and Los Angeles County + University of Southern California (LAC+USC) Medical Center, Los Angeles, CA, USA
| | - Natalia Vecerek
- Division of Rheumatology, Department of Medicine, and Division of Dermatology, Keck School of Medicine, University of Southern California and Los Angeles County, Los Angeles, CA, USA.,Division of Rheumatology, Department of Medicine, Keck School of Medicine, University of Southern California and Los Angeles County + University of Southern California (LAC+USC) Medical Center, Los Angeles, CA, USA
| | - Marie Donaldson
- Division of Rheumatology, Department of Medicine, and Division of Dermatology, Keck School of Medicine, University of Southern California and Los Angeles County, Los Angeles, CA, USA.,Division of Rheumatology, Department of Medicine, Keck School of Medicine, University of Southern California and Los Angeles County + University of Southern California (LAC+USC) Medical Center, Los Angeles, CA, USA
| | - Francisco P Quismorio
- Division of Rheumatology, Department of Medicine, and Division of Dermatology, Keck School of Medicine, University of Southern California and Los Angeles County, Los Angeles, CA, USA.,Division of Rheumatology, Department of Medicine, Keck School of Medicine, University of Southern California and Los Angeles County + University of Southern California (LAC+USC) Medical Center, Los Angeles, CA, USA
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Increased Serum Levels of S100A4 and S100A15 in Individuals Suffering from Hidradenitis Suppurativa. J Clin Med 2021; 10:jcm10225320. [PMID: 34830597 PMCID: PMC8617841 DOI: 10.3390/jcm10225320] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/09/2021] [Revised: 11/02/2021] [Accepted: 11/10/2021] [Indexed: 12/25/2022] Open
Abstract
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease. Recently, some S100 proteins have been suggested to play an important role in the pathogenesis of chronic immune-mediated inflammatory diseases and they may constitute valuable biomarkers for these diseases’ diagnosis and monitoring. The objective of the current study was to investigate, for the first time, serum levels of S100A4 and S100A15 in individuals suffering from HS. Furthermore, we assessed the associations between S100A4 and S100A15 serum levels and the severity of disease, CRP serum concentration and some demographic and clinical data. Serum levels of S100A4 and S100A15 were evaluated with the commercially available ELISA kit according to the manufacturer’s instructions. The serum level of S100A4 in individuals with HS was significantly elevated as compared to controls, with the highest level found in the individuals in Hurley stage II. The S100A15 serum level was positively correlated with the CRP concentration and was associated with the severity of the disease. The serum level of S100A15 in the individuals in Hurley stage III was significantly elevated compared to that of the controls and the individuals with HS in Hurley stages I and II. S100A4 and S100A15 may be considered as new serum biomarkers for the monitoring of HS progression, and they may play a role in the pathogenesis of HS by promoting inflammatory process and fibrosis.
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Batycka-Baran A, Baran W, Nowicka-Suszko D, Koziol-Gałczyńska M, Bieniek A, Matusiak Ł, Łaczmański Ł, Szepietowski JC. Serum Concentration and Skin Expression of S100A7 (Psoriasin) in Patients Suffering from Hidradenitis Suppurativa. Dermatology 2020; 237:733-739. [PMID: 33202403 DOI: 10.1159/000510689] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2020] [Accepted: 07/27/2020] [Indexed: 12/21/2022] Open
Abstract
BACKGROUND Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease. An important role of innate immune dysregulation in the pathogenesis of HS has been highlighted. S100A7 (psoriasin) is an innate, antimicrobial protein that exerts proinflammatory and chemotactic action. OBJECTIVES The objective of the study was to investigate serum concentrations of S100A7 in individuals with HS as compared to healthy controls. Further, we evaluated the expression of S100A7 in lesional HS skin as compared to perilesional (clinically uninvolved) HS skin and normal skin. METHODS Serum concentrations of S100A7 were evaluated with a commercially available ELISA kit. The expression of S100A7 in the skin was assessed using qRT-PCR and immunofluorescence staining. RESULTS We found increased expression of S100A7 in lesional HS skin as compared to perilesional HS skin (p = 0.0017). The expression of S100A7 in lesional HS skin was positively associated with serum C-reactive protein concentration and the severity of disease according to Hurley staging. The serum concentration of S100A7 in individuals with HS was decreased as compared to healthy controls and patients with psoriasis. CONCLUSIONS Upregulated in lesional HS skin, S100A7 may enhance the inflammatory process and contribute to the HS pathogenesis.
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Affiliation(s)
- Aleksandra Batycka-Baran
- Department of Dermatology, Venereology and Allergology, Wrocław Medical University, Wrocław, Poland,
| | - Wojciech Baran
- Department of Dermatology, Venereology and Allergology, Wrocław Medical University, Wrocław, Poland
| | - Danuta Nowicka-Suszko
- Department of Dermatology, Venereology and Allergology, Wrocław Medical University, Wrocław, Poland
| | - Maria Koziol-Gałczyńska
- Department of Dermatology, Venereology and Allergology, Wrocław Medical University, Wrocław, Poland
| | - Andrzej Bieniek
- Department of Dermatology, Venereology and Allergology, Wrocław Medical University, Wrocław, Poland
| | - Łukasz Matusiak
- Department of Dermatology, Venereology and Allergology, Wrocław Medical University, Wrocław, Poland
| | - Łukasz Łaczmański
- Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Wrocław, Poland
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Ayala D, Dhanasekara CS, Thomas K, Tran V, Le A, Hand A, Alhaj Saleh A, Griswold J, Dissanaike S. Surgical management of advanced hidradenitis suppurativa via a one-stage procedure: A single-center experience. Am J Surg 2020; 220:1462-1466. [PMID: 33051068 DOI: 10.1016/j.amjsurg.2020.10.004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/24/2020] [Revised: 06/30/2020] [Accepted: 10/04/2020] [Indexed: 10/23/2022]
Abstract
BACKGROUND Hidradenitis suppurativa (HS) is a debilitating skin condition; in severe forms it requires excision and skin grafting for cure. This is commonly performed as a multi-stage procedure; we explored single-stage operation as a more efficient alternative. METHODS Retrospective review 2007-2018 evaluating outcomes of patients undergoing single-stage surgery. RESULTS 139 one-stage procedures were performed: 35 excision and primary closure, 104 split-thickness skin grafting (STSG). Success rate was higher for STSG at 75% versus 60% with primary closure. Of failed primary closures, 57% required revision by grafting due to recurrence. Axilla procedures were most successful at 91% compared to 70%, 54%, and 50% for inguinal, gluteal, and perineal areas, respectively. Infection was the most common complication (17%), with 38% requiring readmission. CONCLUSION Compared to prior literature on multi-stage HS treatment, one-stage operations are a feasible, cost-effective alternative. STSG should remain the procedure of choice, even when primary closure appears feasible.
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Affiliation(s)
- Donna Ayala
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | | | - Kyle Thomas
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | - Virginia Tran
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | - Audrey Le
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | - Audrey Hand
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | - Adel Alhaj Saleh
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | - John Griswold
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA
| | - Sharmila Dissanaike
- Department of Surgery: Texas Tech University Health Science Center, Lubbock, TX, USA.
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Colosimo C, Yon JR, Fredericks C, Kingsley S, Gupta S, Mentzer CJ, Bokhari F, Poulakidas S. Obesity is Not Associated With Need for Skin Grafting After Hidradenitis Excision. Am Surg 2020; 87:458-462. [PMID: 33047967 DOI: 10.1177/0003134820950686] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/15/2022]
Abstract
INTRODUCTION Hidradenitis suppurativa (HS) is a chronic, debilitating disease associated with inflammation, recurrent abscesses, and fistulae of skin containing apocrine sweat glands. We hypothesize that the need for skin grafting after vacuum-assisted closure was decreased with increasing body mass index (BMI). METHODS Seventy-one consecutive patients with excisions for HS were retrospectively evaluated for demographic data, number of excisions, the total area of excised skin, need for skin grafting, and BMI. Patients were stratified for BMI and underwent logistic regression to compare all other variables. RESULTS Average for BMI was 30.8 ± 7.72, age was 36.89 ±13.52, area excised was 743 cm2 ± 774 cm2, mean operating room trips were 2.62 ± 1.59, and skin grafting was 0.52 ± 0.55. Patients were 60% male. Forty out of 71 patients were obese. There was no correlation between age, BMI, sex, thenumber of excisions, amount of skin excised, or need for a skin graft. There was a statistically significant relationship between the amount of skin excised and the need for skin grafting (P = .006). CONCLUSIONS The amount of skin affected by HS appears to be independent of patient BMI. The need for skin grafting is solely dependent upon the amount of tissue excised. APPLICABILITY OF RESEARCH TO PRACTICE This knowledge will help preoperative planning for all patients with HS, regardless of BMI.
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Affiliation(s)
| | - James R Yon
- Department of Trauma and Acute Care Surgery, Swedish Medical Center, Englewood, CO, USA
| | - Charles Fredericks
- Division of Trauma, Acute Care, and General Surgery, UC Davis Medical Center, Sacramento, CA, USA
| | - Samuel Kingsley
- Department of Trauma and Burn, John H. Stroger, Jr, Hospital of Cook County, Chicago, IL, USA
| | - Sameer Gupta
- Department of Trauma and Burn, John H. Stroger, Jr, Hospital of Cook County, Chicago, IL, USA
| | - Caleb J Mentzer
- Division of Trauma, Critical Care & Acute Care Surgery, Spartanburg Medical Center, Spartanburg, SC, USA
| | - Faran Bokhari
- Department of Trauma and Burn, John H. Stroger, Jr, Hospital of Cook County, Chicago, IL, USA
| | - Stathis Poulakidas
- Department of Trauma and Burn, John H. Stroger, Jr, Hospital of Cook County, Chicago, IL, USA
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Grimstad Ø, Kvammen BØ, Swartling C. Botulinum Toxin Type B for Hidradenitis Suppurativa: A Randomised, Double-Blind, Placebo-Controlled Pilot Study. Am J Clin Dermatol 2020; 21:741-748. [PMID: 32761500 PMCID: PMC7473957 DOI: 10.1007/s40257-020-00537-9] [Citation(s) in RCA: 27] [Impact Index Per Article: 5.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/02/2023]
Abstract
Background Botulinum toxin (BTX) is a potent neurotoxin with a long history of therapeutic application in neurological and dermatological conditions, with a strong efficacy and safety profile. Objective Our aim was to assess whether intradermal injection with BTX-B is an effective treatment for hidradenitis suppurativa (HS). Methods Twenty patients with HS stage I–III disease, according to Hurley’s classification, were consecutively included for treatment with either a placebo or BTX-B. At the next intervention after 3 months, all participants received the active substance and another follow-up at 6 months. The primary outcome was quality of life, measured using the Dermatology Life Quality Index (DLQI), while secondary outcomes were the visual analogue scale (VAS) for pain in the worst boil and HS-related impairment of general health (VAS), as well as changes in physician-reported disease activity assessed as the number of total lesions, and reported adverse effects of treatment. Results The DLQI improved from a median of 17 at baseline to 8 at 3 months in the BTX-B group, compared with a reduction from 13.5 to 11 in the placebo group (p <0.05). Improvement of the patients’ own ratings of symptoms and a reduction in total lesions supplemented the primary outcome. Fifty-five percent of the study population reported some degree of hyperhidrosis. Conclusion BTX-B improves the quality of life in patients with HS. Furthermore, comorbidity between HS and hyperhidrosis is suggested. Trial Registration ClinicalTrials.gov identifier: NCT03103074.
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Affiliation(s)
- Øystein Grimstad
- Department of Dermatology, University Hospital of North Norway, Tromsö, Norway.
- Department of Clinical Medicine, UiT The Arctic University of Norway, Tromsö, Norway.
| | - Bjørn Øivind Kvammen
- Department of Dermatology, University Hospital of North Norway, Tromsö, Norway
- Department of Clinical Medicine, UiT The Arctic University of Norway, Tromsö, Norway
| | - Carl Swartling
- Hidrosis Clinic, Stockholm, Sweden
- Department of Medical Sciences, Dermatology and Venereology, Uppsala University, Uppsala, Sweden
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Nikolakis G, Kaleta KP, Vaiopoulos AG, Wolter K, Baroud S, Wojas-Pelc A, Zouboulis CC. Phenotypes and Pathophysiology of Syndromic Hidradenitis Suppurativa: Different Faces of the Same Disease? A Systematic Review. Dermatology 2020; 237:673-697. [PMID: 32942279 DOI: 10.1159/000509873] [Citation(s) in RCA: 23] [Impact Index Per Article: 4.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/23/2020] [Accepted: 06/27/2020] [Indexed: 12/06/2024] Open
Abstract
BACKGROUND There is growing evidence that (certain) hidradenitis suppurativa (HS) comorbidities comprise syndromes including HS as a key cutaneous manifestation. These apparently autoinflammatory syndromes and their diagnostic delay might have detrimental effects on affected patients. METHODS A systematic review was performed on the databases MEDLINE, EMBASE, and CENTRAL utilizing a standardized extraction form according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. RESULTS Sixty-four eligible articles on syndromic HS were retrieved. The identified syndromes included already described ones (pyoderma gangrenosum-acne-suppurative hidradenitis, pyogenic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, psoriatic arthritis-pyoderma gangrenosum-acne-suppurative hidradenitis, pyoderma gangrenosum-acne vulgaris-hidradenitis suppurativa-ankylosing spondylitis, synovitis-acne-pustulosis-hyperostosis-osteitis) and further novel symptom constellations. Cutaneous signs, including HS lesions, usually precede signs from other organs. The cutaneous signs of a considerable proportion of patients appear refractory to conventional treatment, and monotherapy with biologics does not suffice to sustain remission. CONCLUSION The results are subsequently discussed with focus on the pathophysiology and treatment of the detected syndromes. The dermatologist's role in the precise diagnosis and early treatment administration of HS is pivotal. The purpose of the treatment should be the effective prevention or delay of the autoinflammatory march and its irreversible consequences.
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Affiliation(s)
- Georgios Nikolakis
- Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany,
- European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany,
| | - Katarzyna P Kaleta
- Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany
- Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland
| | - Aristeidis G Vaiopoulos
- Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany
- European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany
| | - Katja Wolter
- Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany
| | - Sumer Baroud
- Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany
- University of Sharjah, Sharjah, United Arab Emirates
| | - Anna Wojas-Pelc
- Department of Dermatology, Jagiellonian University Medical College, Krakow, Poland
| | - Christos C Zouboulis
- Departments of Dermatology, Venereology, Allergology, and Immunology, Dessau Medical Center, Brandenburg Medical School Theodor Fontane, Dessau, Germany
- European Hidradenitis Suppurativa Foundation e.V., Dessau, Germany
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Penno CA, Jäger P, Laguerre C, Hasler F, Hofmann A, Gass SK, Wettstein-Ling B, Schaefer DJ, Avrameas A, Raulf F, Wieczorek G, Lehmann JCU, Loesche C, Roth L, Röhn TA. Lipidomics Profiling of Hidradenitis Suppurativa Skin Lesions Reveals Lipoxygenase Pathway Dysregulation and Accumulation of Proinflammatory Leukotriene B4. J Invest Dermatol 2020; 140:2421-2432.e10. [PMID: 32387270 DOI: 10.1016/j.jid.2020.04.011] [Citation(s) in RCA: 30] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/05/2020] [Revised: 04/08/2020] [Accepted: 04/09/2020] [Indexed: 02/07/2023]
Abstract
Hidradenitis suppurativa (HS) is a chronic, recurring inflammatory dermatosis characterized by abscesses, deep-seated nodules, sinus tracts, and fibrosis in skin lesions around hair follicles of the axillary, inguinal, and anogenital regions. Whereas the exact pathogenesis remains poorly defined, clear evidence suggests that HS is a multifactorial inflammatory disease characterized by innate and adaptive immune components. Bioactive lipids are important regulators of cutaneous homeostasis, inflammation, and resolution of inflammation. Alterations in the lipid mediator profile can lead to malfunction and cutaneous inflammation. We used targeted lipidomics to analyze selected omega-3 and omega-6 polyunsaturated fatty acids in skin of patients with HS and of healthy volunteers. Lesional HS skin displayed enrichment of 5-lipoxygenase (LO)‒derived metabolites, especially leukotriene B4. In addition, 15-LO‒derived metabolites were underrepresented in HS lesions. Changes in the lipid mediator profile were accompanied by transcriptomic dysregulation of the 5-LO and 15-LO pathways. Hyperactivation of the 5-LO pathway in lesional macrophages identified these cells as potential sources of leukotriene B4, which may cause neutrophil influx and activation. Furthermore, leukotriene B4-induced mediators and pathways were elevated in HS lesions, suggesting a contribution of this proinflammatory lipid meditator to the pathophysiology of HS.
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Affiliation(s)
- Carlos A Penno
- Analytical Sciences & Imaging, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Petra Jäger
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Claire Laguerre
- Analytical Sciences & Imaging, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Franziska Hasler
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Andreas Hofmann
- Analytical Sciences & Imaging, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Stephanie K Gass
- Department of Plastic, Reconstructive, Aesthetic and Hand Surgery, University Hospital, Basel, and University of Basel, Basel, Switzerland
| | - Barbara Wettstein-Ling
- Department of Plastic, Reconstructive, Aesthetic and Hand Surgery, University Hospital, Basel, and University of Basel, Basel, Switzerland
| | - Dirk J Schaefer
- Department of Plastic, Reconstructive, Aesthetic and Hand Surgery, University Hospital, Basel, and University of Basel, Basel, Switzerland
| | - Alexandre Avrameas
- Biomarker Development, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Friedrich Raulf
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Grazyna Wieczorek
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Joachim C U Lehmann
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Christian Loesche
- Translational Medicine, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Lukas Roth
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland
| | - Till A Röhn
- Autoimmunity, Transplantation and Inflammation, Novartis Institutes for BioMedical Research, Novartis Pharma AG, Basel, Switzerland.
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Phan K, Charlton O, Smith SD. Hidradenitis suppurativa and acne vulgaris and conglobata—systematic review and meta-analysis. BIOMEDICAL DERMATOLOGY 2019. [DOI: 10.1186/s41702-019-0045-z] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/10/2022]
Abstract
Abstract
Background
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disorder which involves painful nodules and draining abscesses in flexural areas. Acne vulgaris and its more severe variants including acne conglobata and acne fulminans are also disorders involving the follicular unit. Given that follicular obstruction, dilatation and inflammation feature in both HS and acne vulgaris/conglobata, it has been suggested that HS is associated with acne vulgaris/conglobata.
Methods
The present systematic review and meta-analysis was performed according to recommended PRISMA guidelines. All eligible case-control studies comparing patients with HS vs non-HS were included in the present review. All studies must have included either the proportion of patients with acne vulgaris/conglobata in each group, or the summary effect size for association between HS and acne vulgaris/conglobata. The odds ratio (OR) was used as a summary statistic.
Results
From pooled unadjusted meta-analysis, we found a significantly higher proportion of patients with acne vulgaris/conglobata in HS cases compared to controls (OR 3.44, 95% CI 1.95–6.07, P < 0.0001, I2 = 100%). Pooled meta-analysis was also performed with adjusted effect sizes. This demonstrated that HS was significantly associated with acne vulgaris/conglobata after adjustment for potential confounders (OR 3.44, 95% CI 2.43–4.87, P < 0.00001, I2 = 99%).
Conclusions
In summary, a significant association was found between HS and acne vulgaris/conglobata. This has implications in terms of understanding the burden of disease on patient quality of life as well as consideration of optimal management strategies to target both disorders. Physicians taking care of patients with HS should be aware of this association.
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Tugnoli S, Agnoli C, Silvestri A, Giari S, Bettoli V, Caracciolo S. Anger, Emotional Fragility, Self-esteem, and Psychiatric Comorbidity in Patients with Hidradenitis Suppurativa/Acne Inversa. J Clin Psychol Med Settings 2019; 27:527-540. [DOI: 10.1007/s10880-019-09640-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
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11
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Salem I, Kimak M, Conic R, Bragazzi NL, Watad A, Adawi M, Bridgewood C, Pacifico A, Santus P, Rizzi M, Petrou S, Colombo D, Fiore M, Pigatto PDM, Damiani G. Neutrophilic Dermatoses and Their Implication in Pathophysiology of Asthma and Other Respiratory Comorbidities: A Narrative Review. BIOMED RESEARCH INTERNATIONAL 2019; 2019:7315274. [PMID: 31281845 PMCID: PMC6590566 DOI: 10.1155/2019/7315274] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 03/06/2019] [Accepted: 05/14/2019] [Indexed: 01/20/2023]
Abstract
Neutrophilic dermatoses (ND) are a polymorphous group of noncontagious dermatological disorders that share the common histological feature of a sterile cutaneous infiltration of mature neutrophils. Clinical manifestations can vary from nodules, pustules, and bulla to erosions and ulcerations. The etiopathogenesis of neutrophilic dermatoses has continuously evolved. Accumulating genetic, clinical, and histological evidence point to NDs being classified in the spectrum of autoinflammatory conditions. However, unlike the monogenic autoinflammatory syndromes where a clear multiple change in the inflammasome structure/function is demonstrated, NDs display several proinflammatory abnormalities, mainly driven by IL-1, IL-17, and tumor necrosis factor-alpha (TNF-a). Additionally, because of the frequent association with extracutaneous manifestations where neutrophils seem to play a crucial role, it was plausible also to consider NDs as a cutaneous presentation of a systemic neutrophilic condition. Neutrophilic dermatoses are more frequently recognized in association with respiratory disorders than by chance alone. The combination of the two, particularly in the context of their overlapping immune responses mediated primarily by neutrophils, raises the likelihood of a common neutrophilic systemic disease or an aberrant innate immunity disorder. Associated respiratory conditions can serve as a trigger or may develop or be exacerbated secondary to the uncontrolled skin disorder. Physicians should be aware of the possible pulmonary comorbidities and apply this knowledge in the three steps of patients' management, work-up, diagnosis, and treatment. In this review, we attempt to unravel the pathophysiological mechanisms of this association and also present some evidence for the role of targeted therapy in the treatment of both conditions.
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Affiliation(s)
- Iman Salem
- Department of Dermatology, Case Western Reserve University, Cleveland, USA
| | - Mark Kimak
- Department of Dermatology, Case Western Reserve University, Cleveland, USA
| | - Rosalynn Conic
- Department of Dermatology, Case Western Reserve University, Cleveland, USA
| | - Nicola L. Bragazzi
- Department of Health Sciences (DISSAL), School of Public Health, University of Genoa, Genoa, Italy
| | - Abdulla Watad
- Department of Medicine “B”, Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Israel
- Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel
- Section of Musculoskeletal Disease, Leeds Institute of Molecular Medicine, University of Leeds, NIHR Leeds Musculoskeletal Biomedical Research Unit, Chapel Allerton Hospital, Leeds, UK
| | - Mohammad Adawi
- Padeh and Ziv Hospitals, Azrieli Faculty of Medicine, Bar-Ilan University, Ramat Gan, Israel
| | - Charlie Bridgewood
- Section of Musculoskeletal Disease, Leeds Institute of Molecular Medicine, University of Leeds, NIHR Leeds Musculoskeletal Biomedical Research Unit, Chapel Allerton Hospital, Leeds, UK
| | | | - Pierachille Santus
- Department of Biomedical Sciences L. Sacco, University of Milan, Milan, Italy
- Respiratory Unit, Center for Sleep and Respiratory Disorders, “Luigi Sacco” University Hospital, Milan, Italy
| | - Maurizio Rizzi
- Respiratory Unit, Center for Sleep and Respiratory Disorders, “Luigi Sacco” University Hospital, Milan, Italy
| | - Stephen Petrou
- Emergency Medicine, Good Samaritan Hospital Medical Center, New York, USA
| | - Delia Colombo
- Department of Pharmacology, University of Milan, Milan, Italy
| | - Marco Fiore
- Department of Women, Child and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, Naples, Italy
| | - Paolo D. M. Pigatto
- Clinical Dermatology, IRCCS Galeazzi Orthopaedic Institute, Milan, Italy
- Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy
| | - Giovanni Damiani
- Department of Dermatology, Case Western Reserve University, Cleveland, USA
- Department of Women, Child and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, Naples, Italy
- Clinical Dermatology, IRCCS Galeazzi Orthopaedic Institute, Milan, Italy
- Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy
- Young Dermatologists Italian Network, Centro Studi GISED, Bergamo, Italy
- Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Unità Operativa di Dermatologia, IRCCS Fondazione Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy
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Campanati A, Martina E, Giuliodori K, Bobyr I, Consales V, Offidani A. Two cases of Hidradenitis suppurativa and botulinum toxin type a therapy: A novel approach for a pathology that is still difficult to manage. Dermatol Ther 2019; 32:e12841. [DOI: 10.1111/dth.12841] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2018] [Revised: 01/18/2019] [Accepted: 01/24/2019] [Indexed: 01/01/2023]
Affiliation(s)
- Anna Campanati
- Dermatology Unit, Department of Clinical and Molecular SciencesPolytechnic Marche University Ancona Italy
| | - Emanuela Martina
- Dermatology Unit, Department of Clinical and Molecular SciencesPolytechnic Marche University Ancona Italy
| | - Katia Giuliodori
- Dermatology Unit, Department of Clinical and Molecular SciencesPolytechnic Marche University Ancona Italy
| | - Ivan Bobyr
- Dermatology Unit, Department of Clinical and Molecular SciencesPolytechnic Marche University Ancona Italy
| | - Veronica Consales
- Dermatology Unit, Department of Clinical and Molecular SciencesPolytechnic Marche University Ancona Italy
| | - Annamaria Offidani
- Dermatology Unit, Department of Clinical and Molecular SciencesPolytechnic Marche University Ancona Italy
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13
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Vossen ARJV, van der Zee HH, Prens EP. Hidradenitis Suppurativa: A Systematic Review Integrating Inflammatory Pathways Into a Cohesive Pathogenic Model. Front Immunol 2018; 9:2965. [PMID: 30619323 PMCID: PMC6302105 DOI: 10.3389/fimmu.2018.02965] [Citation(s) in RCA: 153] [Impact Index Per Article: 21.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2018] [Accepted: 12/03/2018] [Indexed: 12/19/2022] Open
Abstract
Background: The pathogenesis of hidradenitis suppurativa (HS) is not fully understood. This systematic review examined the latest evidence for molecular inflammatory pathways involved in HS as a chronic inflammatory skin disease. Methods: A systematic literature search was performed in PubMed/Medline and EMBASE from January 2013 through September 2017, according to the preferred reporting items for systematic reviews and meta-analyses (PRISMA). Findings on HS pathogenesis were also compared with those of other immune-mediated inflammatory diseases (IMIDs) in a non-systematic review. In addition, current therapeutic options for HS are briefly discussed on the basis of the findings for the inflammatory pathways involved in HS. Results: A total of 32 eligible publications were identified by the systematic search; these were supplemented with three additional publications. The extracted data indicated that four key themes underlie the pathogenesis of HS and related syndromic conditions. First, nicastrin (NCSTN) and PSTPIP1 mutations are directly associated with auto-inflammatory disease. Secondly, the up-regulation of several cytokines including tumor necrosis factor-α and T helper-17/interleukin-23 are connected to auto-inflammatory mechanisms in the pathogenesis of HS. Thirdly, the microbiome of lesional skin differs significantly vs. normal-appearing skin. Fourthly, HS risk is enhanced through physiological and environmental factors such as smoking, obesity, and mechanical friction. There is significant overlap between the pathogenesis of HS, its syndromic forms and other IMIDs, particularly with respect to aberrations in the innate immune response. Conclusions: The evidence presented in this review supports HS as an auto-inflammatory skin disorder associated with alterations in the innate immune system. Based on these most recent data, an integrative viewpoint is presented on the pathogenesis of HS. Current management strategies on HS consist of anti-inflammatory therapies, surgical removal of chronic lesions, and lifestyle changes such as smoking cessation and weight loss. As large gaps remain in the understanding of the pathogenesis of HS, further research is warranted to ultimately improve the management and treatment of patients with HS and related syndromic conditions.
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Affiliation(s)
- Allard R J V Vossen
- Department of Dermatology, Erasmus University Medical Center, Rotterdam, Netherlands
| | - Hessel H van der Zee
- Department of Dermatology, Erasmus University Medical Center, Rotterdam, Netherlands
| | - Errol P Prens
- Department of Dermatology, Erasmus University Medical Center, Rotterdam, Netherlands
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Outcome of Pedicled Thoracodorsal Artery Perforator Flap in the Surgical Treatment of Stage II and III Hidradenitis Suppurativa of Axilla. Ann Plast Surg 2018; 81:688-693. [DOI: 10.1097/sap.0000000000001658] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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15
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LI CHENGRANG, XU HAOXIANG, WANG BAOXI. Is SAPHO Syndrome Linked to PASH Syndrome and Hidradenitis Suppurativa by Nicastrin Mutation? A Case Report. J Rheumatol 2018; 45:1605-1607. [DOI: 10.3899/jrheum.171007] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/28/2022]
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Abdel Azim AA, Salem RT, Abdelghani R. Combined fractional carbon dioxide laser and long-pulsed neodymium : yttrium-aluminium-garnet (1064 nm) laser in treatment of hidradenitis suppurativa; a prospective randomized intra-individual controlled study. Int J Dermatol 2018; 57:1135-1144. [DOI: 10.1111/ijd.14075] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2018] [Revised: 05/08/2018] [Accepted: 05/16/2018] [Indexed: 12/21/2022]
Affiliation(s)
- Amira A. Abdel Azim
- Dermatology and Venereology Department; Faculty of Medicine for Girls; Al-Azhar University; Cairo Egypt
| | - Rania T. Salem
- Dermatology and Venereology Department; Faculty of Medicine for Girls; Al-Azhar University; Cairo Egypt
| | - Rania Abdelghani
- Dermatology and Venereology Department; Faculty of Medicine for Girls; Al-Azhar University; Cairo Egypt
- Dermatology Department; Armed Forces College of Medicine; Cairo Egypt
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Melnik BC, John SM, Chen W, Plewig G. T helper 17 cell/regulatory T-cell imbalance in hidradenitis suppurativa/acne inversa: the link to hair follicle dissection, obesity, smoking and autoimmune comorbidities. Br J Dermatol 2018; 179:260-272. [PMID: 29573406 DOI: 10.1111/bjd.16561] [Citation(s) in RCA: 43] [Impact Index Per Article: 6.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/08/2018] [Indexed: 12/18/2022]
Abstract
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.
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Affiliation(s)
- B C Melnik
- Department of Dermatology, Environmental Medicine and Health Theory, University of Osnabrück, Osnabrück, Germany
| | - S M John
- Department of Dermatology, Environmental Medicine and Health Theory, University of Osnabrück, Osnabrück, Germany
| | - W Chen
- Department of Dermatology and Allergy, Technical University of Munich, Munich, Germany
| | - G Plewig
- Department of Dermatology and Allergy, Ludwig-Maximilian-University of Munich, Munich, Germany
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18
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Dessinioti C, Tzanetakou V, Zisimou C, Kontochristopoulos G, Antoniou C. A retrospective study of the characteristics of patients with early-onset compared to adult-onset hidradenitis suppurativa. Int J Dermatol 2018; 57:687-691. [PMID: 29604044 DOI: 10.1111/ijd.13985] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/09/2017] [Revised: 02/01/2018] [Accepted: 03/05/2018] [Indexed: 12/28/2022]
Abstract
BACKGROUND The mean age of onset of hidradenitis suppurativa (HS) is between 20 and 24 years. Very few data about patients with early-onset HS exist. OBJECTIVE To investigate the association of early-onset HS with the clinical characteristics: age, gender, body mass index (BMI), smoking, family history of HS, Hurley stage, and number of areas affected. METHODS This was a retrospective study of the reported early age at HS onset (≤17 years old) with clinical characteristics and with the severity of HS at first consultation visit. RESULTS In 166 patients, 42 patients (25.3%) reported early-onset HS. Compared to adult-onset HS, patients with early-onset HS were younger (mean age: 37 years vs. 27 years, P < 0.0001), had a significantly younger mean age of onset (28.2 years old vs. 14.5 years old, respectively, P < 0.0001), longer mean disease duration (8.8 years vs. 12.6 years, respectively, P = 0.011) and were less frequently smokers (P < 0.001), whereas there was no association with gender (P = 0.177) or BMI (0.086). Patients with a family history had increased risk for early-onset HS (OR: 2.45, 95% CI: 1.08-5.56). Early-onset HS was not associated with Hurley stage (OR: 1.12, 95% CI: 0.33-3.74) or with the number of body areas affected (OR: 1.54, 95% CI: 0.49-4.83). CONCLUSION Early-onset HS was frequent and associated with a family history of HS. There was no difference in the severity of HS in adult life for patients with an onset of HS at ≤17 years, compared to patients with adult-onset, which may be reassuring information for these younger patients.
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Affiliation(s)
- Clio Dessinioti
- 1st Department of Dermatology, A.Syggros Hospital, University of Athens, Athens, Greece
| | - Vassiliki Tzanetakou
- 1st Department of Dermatology, A.Syggros Hospital, University of Athens, Athens, Greece
| | - Chrisa Zisimou
- 1st Department of Dermatology, A.Syggros Hospital, University of Athens, Athens, Greece
| | | | - Christina Antoniou
- 1st Department of Dermatology, A.Syggros Hospital, University of Athens, Athens, Greece
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Lu L, Lai H, Pan Z, Hu T, Hou X, Cao K, Ju Q. Clinical and histopathological characteristics in patients with scarring folliculitis type of acne inversa. DERMATO-ENDOCRINOLOGY 2018; 9:e1361575. [PMID: 29484097 PMCID: PMC5821155 DOI: 10.1080/19381980.2017.1361575] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/07/2017] [Accepted: 07/26/2017] [Indexed: 11/05/2022]
Abstract
Objective: This study was designed to study the clinical and histopathological characteristics of patients with the scarring folliculitis type acne inversa in Chinese population. Methods: A total of 21 patients with acne inversa and 6 controls without known dermatological disease were recruited from outpatient department of dermatology and orthopedic surgery. Two-millimeter punch biopsies were taken from 8 patients with acne inversa and 6 controls, fixed in formalin, embedded in paraffin and stained with haematoxylin and eosin prior to histopathological analysis. Results: There were 12 patients (57.14%) belonging to the scarring folliculitis type presented with double comedones, papules, nodules, depressed scars, and were mainly Hurley stage I (66.67%). Many of the scarring folliculitis type were smokers (58.33%), some had a history of occupational exposure (41.67%) and some were overweight (50%), the mean BMI of which is 25.18±3.16 kg/m2. Histopathological changes such as perifollicular inflammation can be observed in scarring folliculitis type of acne inversa and controls as well. However, epidermal hyperplasia, follicular hyperplasia, sebaceous gland disappearance, destruction of hair follicle and sebaceous gland, collagen hyperplasia, perivascular inflammation, granulomatous inflammation, Micro thrombus were only seen in scarring folliculitis type. The mean surface area in patients (8073.36±15798.43 μm2) was smaller than that in controls (302059.08±502813.78 μm2), with statistically significant difference. (P = 0.024). Conclusion: The scarring folliculitis type in acne inversa in Chinese population could be characterized by depressed scars, double-ended comedones, epidermal cysts and had high proportion of smokers, or occupational exposure with lower Hurley stage, as well as diminished sebaceous gland. Further studies are needed to clarify the relations between the clinical subtypes of acne inversa and their corresponding genetypes.
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Affiliation(s)
- Lingyi Lu
- Department of Dermatology, Ningbo First Hospital, Ningbo, Zhejiang, PR China.,Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
| | - Huiying Lai
- Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
| | - Zhanyan Pan
- Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
| | - Tingting Hu
- Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
| | - Xiaoxiao Hou
- Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
| | - Ke Cao
- Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
| | - Qiang Ju
- Department of Dermatology, RenJi Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, PR China
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Andrade TCPCD, Vieira BC, Oliveira AMN, Martins TY, Santiago TM, Martelli ACC. Hidradenitis suppurativa: epidemiological study of cases diagnosed at a dermatological reference center in the city of Bauru, in the Brazilian southeast State of São Paulo, between 2005 and 2015. An Bras Dermatol 2017; 92:196-199. [PMID: 28538878 PMCID: PMC5429104 DOI: 10.1590/abd1806-4841.20175588] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/18/2016] [Accepted: 03/14/2016] [Indexed: 01/08/2023] Open
Abstract
Background Hidradenitis is a chronic inflammatory disease of the hair follicles. A
treatment is necessary due to chronicity and psychological changes that
patient present. Objective To investigate epidemiological aspects and elaborate a risk group profile,
promote early diagnosis and contribute to the knowledge about the
disease. Methods This cross-sectional descriptive study with retrospective analysis of medical
records of 194 patients diagnosed with hidrosadenitis in a dermatological
reference center in the city of Bauru (SP) between 2005 and 2015. Results Females accounted for 74% of cases. The age at diagnosis ranged from 10 to 67
years and the majority was within the 3rd and 4th decade of life. It
occurred Association with diabetes mellitus in 33%, obesity in 55% and
smoking in 61% was observed. Mean time between the onset of the disease and
diagnosis was nine years. Hurley stage II was the most common at diagnosis.
The therapeutic option mostly used in Hurley I and II was systemic
antibiotics and in Hurley III was surgery. Study limitations the main limitation of this study is its retrospective design, which does not
allow the true clinical confirmation of the disease by investigators. Conclusion we outlined the following profile: women, caucasian, between 3rd and 4th
decade of life, associated with obesity, smoking, late diagnosis and
multiple potential therapeutic modalities. We highlight the importance of
studies like this in order to identify risk groups and encourage early
diagnosis.
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Abstract
The association of the metabolic syndrome (MetS) and its components with immune-mediated chronic inflammatory disorders has attracted much interest within the last two decades. In addition to the well-established association of psoriasis with MetS, recent data point to an association between MetS and hidradenitis suppurativa, as well. The association of hidradenitis suppurativa with MetS and its components, such as diabetes, obesity, and dyslipidemia, has been consistently identified in controlled studies. This relationship is not only limited to older individuals but also applicable to younger patients and those with mild disease. This review documents the link between these disorders and discusses its practical implications, mainly highlighting the importance of prevention and routine screening for early diagnosis to prevent cardiovascular-related morbidity.
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Affiliation(s)
- Tulin Ergun
- Department of Dermatology, Marmara University School of Medicine, Istanbul, Turkey.
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22
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Abstract
Originally coined as "syndrome X" in 1988 by Gerald Reaven (1928), the metabolic syndrome (MetS) encompasses a constellation of risk factors, the coincidence of which amounts to an increased cardiovascular and diabetic risk. Rising numbers of dermatoses are being recognized as cutaneous markers of MetS. Dermatologists should look beyond treating the cutaneous condition and quantify the associated increase in cardiovascular risk. The original dermatosis associated with obesity was acanthosis nigricans-described in 1889 by Paul Gerson Unna (1850-1929) and Sigmund Pollitzer (1859-1937). Over the last 20 years, clear associations between psoriasis, hidradenitis suppurativa, and MetS have also emerged. Several studies have shown synergistic improvement in the cutaneous pathology after treatment of components of MetS. This suggests common causalities and is a burgeoning area of research. We review the available evidence about the genetics underlying psoriasis, hidradenitis suppurativa, and acanthosis nigricans. Despite the strong clinical associations, the underlying genetic basis for a link to MetS remains unclear.
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Affiliation(s)
- Emma Fanning
- Department of Medicine, St James Hospital, Trinity College Dublin, Dublin, Ireland
| | - Donal O'Shea
- Department of Endocrinology, St Vincent's University Hospital, University College Dublin, Dublin, Ireland.
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Gallagher C, Kirthi S, Burke T, O'Shea D, Tobin AM. Remission of hidradenitis suppurativa after bariatric surgery. JAAD Case Rep 2017; 3:436-437. [PMID: 28932789 PMCID: PMC5594229 DOI: 10.1016/j.jdcr.2017.06.008] [Citation(s) in RCA: 16] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/02/2022] Open
Affiliation(s)
| | | | - Thomas Burke
- Department of Endocrinology and Diabetes Mellitus, St. Vincent's University Hospital, Dublin, Ireland
| | - Donal O'Shea
- Department of Endocrinology and Diabetes Mellitus, St. Vincent's University Hospital, Dublin, Ireland
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Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment. Am J Clin Dermatol 2017; 18:555-562. [PMID: 28236224 DOI: 10.1007/s40257-017-0265-1] [Citation(s) in RCA: 84] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis usually manifesting as skin ulcers with undermined erythematous-violaceous borders. It may be isolated, associated with systemic conditions or occurring in the context of autoinflammatory syndromes such as PAPA (pyogenic arthritis, PG and acne), PASH (PG, acne and suppurative hidradenitis) or PAPASH (pyogenic arthritis, acne, PG and suppurative hidradenitis). From a physiopathological point of view, all these conditions share common mechanisms consisting of over-activation of the innate immune system leading to increased production of the interleukin (IL)-1 family and 'sterile' neutrophil-rich cutaneous inflammation. From a genetic point of view, a number of mutations affecting the proteins of the inflammasome complex (the molecular platform responsible for triggering autoinflammation) or the proteins that regulate inflammasome function have been described in these disorders. As these debilitating entities are all associated with the over-expression of IL-1 and tumour necrosis factor (TNF)-α, biological drugs specifically targeting these cytokines are currently the most effective treatments but, given the emerging role of IL-17 in the pathogenesis of these syndromes, IL-17 antagonists may represent the future management of these conditions.
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Affiliation(s)
- Massimo Cugno
- Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda, Via Pace, 9, 20122, Milan, Italy.
| | - Alessandro Borghi
- Dipartimento di Scienze Mediche, Sezione di Dermatologia e Malattie Infettive, Università degli Studi di Ferrara, Ferrara, Italy
| | - Angelo V Marzano
- Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Unità Operativa di Dermatologia, IRCCS Fondazione Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy
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26
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A retrospective institutional study of the association of smoking with the severity of hidradenitis suppurativa. J Dermatol Sci 2017; 87:206-207. [DOI: 10.1016/j.jdermsci.2017.04.006] [Citation(s) in RCA: 17] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/04/2017] [Revised: 03/23/2017] [Accepted: 04/06/2017] [Indexed: 11/22/2022]
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Marzano AV, Borghi A, Wallach D, Cugno M. A Comprehensive Review of Neutrophilic Diseases. Clin Rev Allergy Immunol 2017; 54:114-130. [DOI: 10.1007/s12016-017-8621-8] [Citation(s) in RCA: 87] [Impact Index Per Article: 10.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/03/2023]
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Monnier L, Dohan A, Amara N, Zagdanski AM, Drame M, Soyer P, Hoeffel C. Anoperineal disease in Hidradenitis Suppurativa : MR imaging distinction from perianal Crohn's disease. Eur Radiol 2017; 27:4100-4109. [PMID: 28289934 DOI: 10.1007/s00330-017-4776-1] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/18/2016] [Revised: 02/08/2017] [Accepted: 02/13/2017] [Indexed: 12/21/2022]
Abstract
OBJECTIVE To determine whether Hidradenitis suppurativa (HS)-related anoperineal disease can be distinguished from Crohn's disease (CD) using MRI. METHODS Pelvic MRI of 23 HS and 46 CD patients with anoperineal disease between 2007 and 2014 were independently analysed by two radiologists. For diagnosis of HS, sensitivity, specificity and positive likelihood ratios (LRs) of inflammatory features and of their anatomical distribution were calculated and compared to those of CD. RESULTS In HS, fistulae were less present (P = .033) and less frequently involved the sphincters (P = .001) than in CD. Granulomas were more frequent (P = .0005). For anterior/inguinal and posterior localizations, sensitivity, specificity and LR for diagnosis of HS were 70% (49.1, 84.4), 87% (74.3, 93.9), 5.3 (2.41, 11.79) and 57% (36.8, 74.4), 93% (82.5, 97.8), 8.67 (2.74, 27.41), respectively. Combination of signs including posterior involvement, absence of rectal wall thickening and bilaterality of features yielded specificity of 100% (95% CI: 92.3-100) for HS. CONCLUSION Although MRI presentations of anoperineal disease may overlap between CD and HS, specific diagnosis of HS is possible with a combination of three features: absence of features' predominance in perianal area, absence of rectal wall thickening and bilaterality of features. KEY POINTS • Spectrum of MRI features of HS-related anoperineal disease is wide. • Specific diagnosis of HS-related anoperineal disease is possible using MR imaging. • A combination of three MR signs allows distinction between HS and CD.
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Affiliation(s)
- Louise Monnier
- Department of Radiology, Hôpital Maison Blanche, Avenue du Général Koenig, 51092, Reims Cedex, France.
| | - Anthony Dohan
- Department of Radiology, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, 2 rue Ambroise Paré, 75010, Paris, France.,Université Paris-Diderot, Sorbonne Paris Cité, 10 Avenue de Verdun, 75010, Paris, France
| | - Nedjoua Amara
- Department of Radiology, Hôpital Maison Blanche, Avenue du Général Koenig, 51092, Reims Cedex, France
| | - Anne-Marie Zagdanski
- Department of Radiology, Saint Louis Hospital, 1 Avenue Claude Vellefaux, 75010, Paris, France
| | - Moustapha Drame
- Department of Statistics, Hôpital Robert Debré, Avenue du Général Koenig, 51092, Reims Cedex, France
| | - Philippe Soyer
- Université Paris-Diderot, Sorbonne Paris Cité, 10 Avenue de Verdun, 75010, Paris, France
| | - Christine Hoeffel
- Department of Radiology, Hôpital Maison Blanche, Avenue du Général Koenig, 51092, Reims Cedex, France
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Darji K, Dhandha MM, Guo M. Hidradenitis suppurativa, neutrophilic dermatoses and diverticular disease in a young African-American patient. BMJ Case Rep 2017; 2017:bcr-2016-217845. [PMID: 28188167 DOI: 10.1136/bcr-2016-217845] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Abstract
Hidradenitis suppurativa (HS) is a chronic skin disorder of the terminal follicular epithelium of apocrine sweat glands, manifesting as painful and exudative papules, pustules, cysts or nodules. This inflammatory condition often presents with other systemic and cutaneous disorders. We present the case of an African-American man with HS who was also diagnosed with neutrophilic dermatoses and diverticular disease. Neutrophilic dermatosis was identified based on histopathology findings. Our patient underwent multiple surgeries for flaring of his skin condition. Colchicine and doxycycline were started, but the patient was not able to tolerate them. Humira was planned for treatment of HS and neutrophilic dermatosis but could not be pursued because of the pericolic abscess. Colonoscopy and radiological investigation revealed multiple colonic diverticuli, for which he initially underwent percutaneous drainage followed by surgical removal of sigmoid mass and colocutaneous fistula. Culture from the specimen revealed abnormal growth of Actinomyces.
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Affiliation(s)
- Kavita Darji
- Saint Louis University School of Medicine, Saint Louis, Missouri, USA
| | - Maulik M Dhandha
- Department of Dermatology, St Louis University, St Louis, Missouri, USA
| | - Mary Guo
- Department of Dermatology, St Louis University, St Louis, Missouri, USA
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García-Martínez F, Pascual J, López-Martín I, Pereyra-Rodríguez J, Martorell Calatayud A, Salgado-Boquete L, Labandeira-García J. Actualización en hidrosadenitis supurativa en Atención Primaria. Semergen 2017; 43:34-42. [DOI: 10.1016/j.semerg.2015.12.005] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/10/2015] [Revised: 10/30/2015] [Accepted: 12/14/2015] [Indexed: 12/22/2022]
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Matusiak Ł, Szczęch J, Bieniek A, Nowicka-Suszko D, Szepietowski JC. Increased interleukin (IL)-17 serum levels in patients with hidradenitis suppurativa: Implications for treatment with anti-IL-17 agents. J Am Acad Dermatol 2016; 76:670-675. [PMID: 28041632 DOI: 10.1016/j.jaad.2016.10.042] [Citation(s) in RCA: 131] [Impact Index Per Article: 14.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/25/2016] [Revised: 10/22/2016] [Accepted: 10/29/2016] [Indexed: 01/19/2023]
Abstract
BACKGROUND Biologics seem to offer a promising nonsurgical approach in hidradenitis suppurativa (HS), especially in disease with highly pronounced inflammation. Recent studies revealed increased expression of a broad range of cytokines in lesional HS skin, including interleukin (IL)-17. OBJECTIVE This study was undertaken to determine IL-17 serum levels in this group of patients. METHODS Our study was conducted on a group of 86 patients between 16 and 72 years of age with HS. A total of 86 matched healthy volunteers constituted the control group. Enzyme-linked immunosorbent assay kits were used to quantify IL-17 serum concentration. RESULTS The mean IL-17 serum level of patients with HS was 3.68 ± 2.08 pg/mL, which was significantly elevated (P < .0001) compared with that found in healthy volunteers (2.5 ± 1.11 pg/mL). Moreover, there was a tendency toward higher serum concentrations of IL-17 in patients with more advanced disease (P = .005). Disease duration; patient sex, age, and body mass index; and smoking habits were not determining factors for IL-17 serum concentration. LIMITATIONS Hospital-based study population was a limitation, as was a lack of posttreatment assessment. CONCLUSION In light of our findings and literature on increased expression of IL-17 in HS lesions, evaluating the clinical effectiveness of using anti-IL-17 agents in the treatment of patients with HS is justified.
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Affiliation(s)
- Łukasz Matusiak
- Department of Dermatology, Venereology, and Allergology, Wroclaw Medical University, Wrocław, Poland.
| | - Justyna Szczęch
- Department of Dermatology, Venereology, and Allergology, Wroclaw Medical University, Wrocław, Poland
| | - Andrzej Bieniek
- Department of Dermatology, Venereology, and Allergology, Wroclaw Medical University, Wrocław, Poland
| | - Danuta Nowicka-Suszko
- Department of Dermatology, Venereology, and Allergology, Wroclaw Medical University, Wrocław, Poland
| | - Jacek C Szepietowski
- Department of Dermatology, Venereology, and Allergology, Wroclaw Medical University, Wrocław, Poland
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Dessinioti C, Zisimou C, Tzanetakou V, Stratigos A, Antoniou C. Oral clindamycin and rifampicin combination therapy for hidradenitis suppurativa: a prospective study and 1-year follow-up. Clin Exp Dermatol 2016; 41:852-857. [PMID: 27753139 DOI: 10.1111/ced.12933] [Citation(s) in RCA: 36] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 03/22/2016] [Indexed: 12/01/2022]
Abstract
BACKGROUND Limited data exist on the use of systemic antibiotic treatment for hidradenitis supportive (HS). AIM To investigate the effectiveness, safety and relapse rate of HS treated with a combination of daily oral clindamycin and rifampicin. METHODS This was a prospective, hospital-based study of oral clindamycin 600 mg and rifampicin 600 mg daily for 12 weeks for treatment of HS. Patients were followed up for 1 year to monitor for relapse. RESULTS In total, 26 patients with HS received oral clindamycin and rifampicin. Most were overweight or obese (73%), and most were smokers (88%). After 12 weeks, clinical response was noted in 19 patients (73%). Response was associated only with female sex (P = 0.02), and not with body mass index, Hurley stage or lesion location. Eight patients (31%) experienced adverse events. At the 1-year follow-up, there was sustained efficacy in 7 (41%) patients, while 10 (59%) had disease relapse after a mean time of 4.2 months. CONCLUSIONS Oral clindamycin with oral rifampicin for 12 weeks is an effective and tolerable regimen for HS.
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Affiliation(s)
- C Dessinioti
- First Department of Dermatology, Andreas Sygros Hospital, University of Athens, Athens, Greece
| | - C Zisimou
- First Department of Dermatology, Andreas Sygros Hospital, University of Athens, Athens, Greece
| | - V Tzanetakou
- First Department of Dermatology, Andreas Sygros Hospital, University of Athens, Athens, Greece
| | - A Stratigos
- First Department of Dermatology, Andreas Sygros Hospital, University of Athens, Athens, Greece
| | - C Antoniou
- First Department of Dermatology, Andreas Sygros Hospital, University of Athens, Athens, Greece
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Falola RA, DeFazio MV, Anghel EL, Mitnick CDB, Attinger CE, Evans KK. What Heals Hidradenitis Suppurativa. Plast Reconstr Surg 2016; 138:219S-229S. [DOI: 10.1097/prs.0000000000002671] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
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Marzano AV, Borghi A, Meroni PL, Cugno M. Pyoderma gangrenosum and its syndromic forms: evidence for a link with autoinflammation. Br J Dermatol 2016; 175:882-891. [PMID: 27106250 DOI: 10.1111/bjd.14691] [Citation(s) in RCA: 99] [Impact Index Per Article: 11.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/18/2015] [Indexed: 12/13/2022]
Abstract
Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis manifesting as painful ulcers with violaceous, undermined borders on the lower extremities. It may occur in the context of classic syndromes like PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) and SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis), as well as in a recently described entity named PASH (pyoderma gangrenosum, acne and suppurative hidradenitis). Pyoderma gangrenosum has recently been included within the spectrum of autoinflammatory diseases, which are characterized by recurrent episodes of sterile inflammation, without circulating autoantibodies and autoreactive T cells. In PAPA syndrome, different mutations involving the PSTPIP1 gene, via an increased binding affinity to pyrin, induce the assembly of inflammasomes. These are molecular platforms involved in the activation of caspase 1, a protease that cleaves inactive prointerleukin (pro-IL)-1β to its active isoform IL-1β. The overproduction of IL-1β triggers the release of a number of proinflammatory cytokines and chemokines, which are responsible for the recruitment and activation of neutrophils, leading to neutrophil-mediated inflammation. In SAPHO syndrome, the activation of the PSTPIP2 inflammasome has been suggested to play a role in inducing the dysfunction of the innate immune system. Patients with PASH have recently been reported to present alterations of genes involved in well-known autoinflammatory diseases, such as PSTPIP1, MEFV, NOD2 and NLRP3. Pyoderma gangrenosum and its syndromic forms can be regarded as a single clinicopathological spectrum in the context of autoinflammation.
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Affiliation(s)
- A V Marzano
- Unità Operativa di Dermatologia, IRCCS Fondazione Cá Granda, Ospedale Maggiore Policlinico, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, Milan, Italy.
| | - A Borghi
- Dipartimento di Scienze Mediche, Sezione di Dermatologia e Malattie Infettive, Università degli Studi di Ferrara, Ferrara, Italy
| | - P L Meroni
- Dipartimento di Scienze Cliniche e di Comunità, Università degli Studi di Milano, Cattedra di Reumatologia, Istituto G. Pini, IRCCS Istituto Auxologico Italiano, Milan, Italy
| | - M Cugno
- Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Sezione di Medicina Interna, Università degli Studi di Milano, IRCCS Fondazione Cá Granda, Ospedale Maggiore Policlinico, Milan, Italy
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Affiliation(s)
- E C Veysey
- Department of Dermatology, St Vincent's Hospital, 41 Victoria Parade, Fitzroy, Melbourne, Victoria, 3065, Australia.
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Principi M, Cassano N, Contaldo A, Iannone A, Losurdo G, Barone M, Mastrolonardo M, Vena GA, Ierardi E, Di Leo A. Hydradenitis suppurativa and inflammatory bowel disease: An unusual, but existing association. World J Gastroenterol 2016; 22:4802-4811. [PMID: 27239107 PMCID: PMC4873873 DOI: 10.3748/wjg.v22.i20.4802] [Citation(s) in RCA: 37] [Impact Index Per Article: 4.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/18/2016] [Revised: 04/11/2016] [Accepted: 05/04/2016] [Indexed: 02/06/2023] Open
Abstract
Inflammatory bowel disease (IBD) could be associated with several extra-intestinal manifestations (EIMs) involving musculoskeletal, hepatopancreatobiliary, ocular, renal, and pulmonary systems, as well as the skin. In the last years, hidradenitis suppurativa (HS) is acquiring an increasing interest. IBD, especially Crohn's disease (CD), is among the most reported associated diseases in HS patients. The aim of this paper is to give a brief overview of data showing a possible epidemiologic and pathogenetic association between IBD and HS. We performed a pooled-data analysis of four studies and pooled prevalence of HS in IBD patients was 12.8%, with a 95%CI of 11.7%-13.9%. HS was present in 17.3% of subjects with CD (95%CI: 15.5%-19.1%) and in 8.5% of UC patients (95%CI: 7.0%-9.9%). Some items, especially altered immune imbalance, are generally involved in IBD pathogenesis as well as invoked by HS. Smoking is one of the most relevant risk factors for both disorders, representing a predictor of their severity, despite, actually, there being a lack of studies analyzing a possible shared pathway. A role for inheritance in HS and CD pathogenesis has been supposed. Despite a genetic susceptibility having been demonstrated for both diseases, further studies are needed to investigate a genetic mutual route. Although the pathogenesis of IBD and HS is generally linked to alterations of the immune response, recent findings suggest a role for intestinal and skin microbiota, respectively. In detail, the frequent finding of Staphylococcus aureus and coagulase-negative staphylococci on HS cutaneous lesions suggests a bacterial involvement in disease pathogenesis. Moreover, microflora varies in the different cutaneous regions of the body and, consequently, two different profiles of HS patients have been identified on these bases. On the other hand, it is well-known that intestinal microbiota may be considered as "the explosive mixture" at the origin of IBD despite the exact relationship having not been completely clarified yet. A better comprehension of the role that some bacterial species play in the IBD pathogenesis may be essential to develop appropriate management strategies in the near future. A final point is represented by some similarities in the therapeutic management of HS and IBD, since they may be controlled by immunomodulatory drugs. In conclusion, an unregulated inflammation may cause the lesions typical of both HS and IBD, particularly when they coexist. However, this is still a largely unexplored field.
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Zwaenepoel T, Vlam KD. SAPHO: Treatment options including bisphosphonates. Semin Arthritis Rheum 2016; 46:168-173. [PMID: 27369452 DOI: 10.1016/j.semarthrit.2016.04.004] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2015] [Revised: 04/07/2016] [Accepted: 04/25/2016] [Indexed: 11/24/2022]
Abstract
INTRODUCTION Both the diagnosis and treatment of the syndrome of synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) remain difficult. We describe a case series of 21 patients with SAPHO and their response to several pharmacological treatments. METHODS Clinical and biochemical data, along with medical imaging, were collected from the medical records of 21 patients, diagnosed as SAPHO during follow-up between 2005 and 2013. Symptoms and inflammatory markers were recorded twice, once at first patient presentation, and once at the end of follow-up. Synovitis, acne, pustulosis, hyperostosis, and osteitis were labeled as defining features. All treatment options were categorized according to their respective responses (full remission, partial remission, and no disease control). RESULTS There was a female predominance and a median age of 32 years (range: 12-54 years). Median follow-up duration was 45 months (range: 0-188 months). Total prevalence of defining features in this cohort increased for each defining feature during follow-up, except for acne. All patients reached full or partial remission at the end of follow-up. A total of 14 patients were treated with bisphosphonates. Of which 8 of them went into full or partial remission. DISCUSSION AND CONCLUSION In our case series, none of the patients had the full presentation of SAPHO at the first consultation. Some presented with symptoms suggestive for psoriatic arthritis. This explains why diagnosis of SAPHO can be challenging. Full remission was induced in the majority of individuals. Bisphosphonates seem to be a noteworthy treatment option. We suggest a prospective placebo-controlled clinical trial with bisphosphonates to confirm this observation.
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Affiliation(s)
- Tom Zwaenepoel
- Department of Rheumatology, University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium
| | - Kurt de Vlam
- Department of Rheumatology, University Hospitals Leuven, Herestraat 49, B-3000 Leuven, Belgium.
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Woodruff CM, Charlie AM, Leslie KS. Hidradenitis Suppurativa: A Guide for the Practicing Physician. Mayo Clin Proc 2015; 90:1679-93. [PMID: 26653298 DOI: 10.1016/j.mayocp.2015.08.020] [Citation(s) in RCA: 20] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/29/2015] [Revised: 08/19/2015] [Accepted: 08/19/2015] [Indexed: 11/17/2022]
Abstract
Hidradenitis suppurativa is a chronic inflammatory disease of apocrine gland-bearing skin. Although immunologic derangements, genetic predisposition, obesity, and smoking are likely important factors, the pathogenesis of the disease and the effect of available treatments on disease course have not been fully elucidated. In the absence of proper treatment, chronic inflammation results in diffuse scarring and a wide array of complications, including the development of cutaneous squamous cell carcinoma. This severe and chronic disease can have detrimental effects on self-esteem and quality of life. No ideal treatment regimen has been defined, but several therapies have been found to reduce lesion severity and improve symptoms. We reviewed the literature through July 2014 for existing treatments. Published articles were obtained via systematic review of medical databases (PubMed, Embase, Google Scholar) and scrutiny of citation lists using the search terms "hidradenitis suppurativa" and "acne inversa". Given the scarce literature on treatment strategies, we also reviewed data from any case reports or prospective and retrospective studies that were located. On the basis of the existing literature, we provide an evidence-based algorithm for the management of this disease in the primary care setting. More research is needed to evaluate the comparative effectiveness of topical and systemic treatments and to better understand the pathogenesis, natural history, and subtypes of hidradenitis suppurativa.
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Affiliation(s)
- Carina M Woodruff
- Department of Dermatology, University of California, San Francisco, San Francisco
| | - Abbas M Charlie
- Department of Dermatology, University of California, San Francisco, San Francisco
| | - Kieron S Leslie
- Department of Dermatology, University of California, San Francisco, San Francisco.
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Delobeau M, Abdou A, Puzenat E, Deveza E, Biver-Dalle C, van de Laak A, Roche-Kubler B, Vuitton L, Koch S, Wendling D, Aubin F. Observational case series on adalimumab-induced paradoxical hidradenitis suppurativa. J DERMATOL TREAT 2015; 27:251-3. [PMID: 26368546 DOI: 10.3109/09546634.2015.1094179] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
INTRODUCTION Although the efficacy of TNF blockers has been demonstrated in hidradenitis suppurativa (HS), many paradoxical effects have also been described with TNF antagonists. We wished to describe patients with adalimumab (ADA)-induced paradoxical HS. METHODS This is a retrospective descriptive case series of four patients with ADA-induced paradoxical HS. RESULTS All the patients had a good response to TNFa antagonist therapy at the time of HS occurrence. The time from TNFa antagonist initiation to HS onset or exacerbation ranged from a few weeks to 24 months. The outcome of HS was variable. Systemic antibiotics were required in all the cases to control HS. TNF blockers were continued in three cases with a switch to another anti-TNF class in one case. Switch to ustekinumab was prescribed in one patient with SA and Crohn's disease. CONCLUSION Although the imputability of TNF blockers in paradoxical HS is still debatable, further research and observation are needed to confirm and distinguish patients with genetic and clinical predisposition in the onset or exacerbation of HS during anti-TNF treatment.
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Affiliation(s)
- Marine Delobeau
- a Department of Dermatology , University Hospital , Besançon , France
| | - Ahlam Abdou
- a Department of Dermatology , University Hospital , Besançon , France .,b Department of Dermatology , IBN SINA University Hospital , Rabat , Morocco
| | - Eve Puzenat
- a Department of Dermatology , University Hospital , Besançon , France
| | - Elise Deveza
- a Department of Dermatology , University Hospital , Besançon , France
| | | | | | | | - Lucine Vuitton
- c Department of Gastroenterology , University Hospital , Besançon , France .,d University of Franche Comté , EA3181 SFR FED 4234 , Besançon , France , and
| | - Stéphane Koch
- c Department of Gastroenterology , University Hospital , Besançon , France
| | - Daniel Wendling
- e Department of Rheumatology , University Hospital , Besançon , France
| | - François Aubin
- a Department of Dermatology , University Hospital , Besançon , France .,d University of Franche Comté , EA3181 SFR FED 4234 , Besançon , France , and
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Applications and limitations of lipid nanoparticles in dermal and transdermal drug delivery via the follicular route. Eur J Pharm Biopharm 2015; 97:152-63. [PMID: 26144664 DOI: 10.1016/j.ejpb.2015.06.020] [Citation(s) in RCA: 88] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/27/2015] [Revised: 04/10/2015] [Accepted: 06/08/2015] [Indexed: 01/22/2023]
Abstract
Lipid nanoparticles (LN) such as solid lipid nanoparticles (SLN) and nanolipid carriers (NLC) feature several claimed benefits for topical drug therapy including biocompatible ingredients, drug release modification, adhesion to the skin, and film formation with subsequent hydration of the superficial skin layers. However, penetration and permeation into and across deeper skin layers are restricted due to the barrier function of the stratum corneum (SC). As different kinds of nanoparticles provide the potential for penetration into hair follicles (HF) LN are applicable drug delivery systems (DDS) for this route in order to enhance the dermal and transdermal bioavailability of active pharmaceutical ingredients (API). Therefore, this review addresses the HF as application site, published formulations of LN which showed follicular penetration (FP), and characterization methods in order to identify and quantify the accumulation of API delivered by the LN in the HF. Since LN are based on lipids that appear in human sebum which is the predominant medium in HF an increased localization of the colloidal carriers as well as a promoted drug release may be assumed. Therefore, sebum-like lipid material and a size of less or equal 640 nm are appropriate specifications for FP of particulate formulations.
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Arjona-Aguilera C, Linares-Barrios M, Albarrán-Planelles C, Jiménez-Gallo D. Dowling-Degos Disease Associated With Hidradenitis Suppurativa: A Case Report. ACTAS DERMO-SIFILIOGRAFICAS 2015. [DOI: 10.1016/j.adengl.2015.03.015] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
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Insulin resistance and skin diseases. ScientificWorldJournal 2015; 2015:479354. [PMID: 25977937 PMCID: PMC4419263 DOI: 10.1155/2015/479354] [Citation(s) in RCA: 60] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/18/2015] [Accepted: 03/17/2015] [Indexed: 12/16/2022] Open
Abstract
In medical practice, almost every clinician may encounter patients with skin disease. However, it is not always easy for physicians of all specialties to face the daily task of determining the nature and clinical implication of dermatologic manifestations. Are they confined to the skin, representing a pure dermatologic event? Or are they also markers of internal conditions relating to the patient's overall health? In this review, we will discuss the principal cutaneous conditions which have been linked to metabolic alterations. Particularly, since insulin has an important role in homeostasis and physiology of the skin, we will focus on the relationships between insulin resistance (IR) and skin diseases, analyzing strongly IR-associated conditions such as acanthosis nigricans, acne, and psoriasis, without neglecting emerging and potential scenarios as the ones represented by hidradenitis suppurativa, androgenetic alopecia, and hirsutism.
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Matusiak Ł, Batycka-Baran A, Bieniek A, Szepietowski JC. Decreased number of circulating endothelial progenitor cells in hidradenitis suppurativa patients. Dermatology 2015; 230:228-233. [PMID: 25721353 DOI: 10.1159/000371877] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2014] [Accepted: 01/04/2015] [Indexed: 01/02/2025] Open
Abstract
BACKGROUND Hidradenitis suppurativa (HS) is nowadays regarded as a systemic disease associated with metabolic syndrome. Some recent studies have also demonstrated an increased cardiovascular risk in patients with HS. Circulating endothelial progenitor cells (EPCs) play an integral role in the regulation and protection of the endothelium and in the maintenance of vascular homeostasis. OBJECTIVE This study was undertaken to determine the possible alterations in the number of EPCs in patients with HS compared to controls. METHODS The number of EPCs, identified as CD133+/KDR+ cells, was determined with flow cytometry in the peripheral blood of 25 HS patients and 31 controls. RESULTS The number of EPCs was significantly reduced in HS sufferers compared with controls (p < 0.0001). The mean number of EPCs was assessed as 191.3 ± 118.5/ml and 672.4 ± 343.0/ml for patients and controls, respectively. CONCLUSION A decreased number of EPCs among HS sufferers may contribute to endothelial malfunction resulting in increased cardiovascular risk in this group of patients.
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Arjona-Aguilera C, Linares-Barrios M, Albarrán-Planelles C, Jiménez-Gallo D. Dowling-Degos disease associated with hidradenitis suppurativa: a case report. ACTAS DERMO-SIFILIOGRAFICAS 2014; 106:337-8. [PMID: 25529465 DOI: 10.1016/j.ad.2014.09.010] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2014] [Revised: 08/17/2014] [Accepted: 09/16/2014] [Indexed: 11/18/2022] Open
Affiliation(s)
- C Arjona-Aguilera
- Servicio de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España.
| | - M Linares-Barrios
- Servicio de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España
| | - C Albarrán-Planelles
- Servicio de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España
| | - D Jiménez-Gallo
- Servicio de Dermatología Médico-Quirúrgica y Venereología, Hospital Universitario Puerta del Mar, Cádiz, España
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Abstract
Hidradenitis suppurativa (HS) is a burdensome disease and has the potential to affect the life course of patients. It is a rare disease in children, and the recorded literature is correspondingly scarce. This article reviews the therapeutic options for HS in children and adolescents, and highlights particular differences or challenges with treating patients in this age group compared with adults. The work-up of paediatric patients with HS should include considerations of possible endocrine co-morbidities and obesity. Medical therapy of lesions may include topical clindamycin. Systemic therapy may include analgesics, clindamycin and rifampicin, finasteride, corticosteroids or tumour necrosis factor alpha (TNFα) blockers. Superinfections should be appropriately treated. Scarring lesions generally require surgery.
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Affiliation(s)
- Peter Riis Mikkelsen
- Department of Dermatology, Roskilde Hospital, Health Sciences Faculty, University of Copenhagen, Copenhagen, Denmark,
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