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      Hidradenitis suppurativa: from pathogenesis to diagnosis and treatment

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          Abstract

          Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease primarily affecting apocrine gland-rich areas of the body and presenting with painful nodules, abscesses, sinus tracts, and scarring. HS is a multifactorial disease in which genetic and environmental factors play a key role. The primary defect in HS pathophysiology involves follicular occlusion of the folliculopilosebaceous unit, followed by follicular rupture, and immune responses (perifollicular lympho-histiocytic inflammation), finally leading to the development of clinical HS lesions. HS has a destructive impact on the patient’s quality of life, being a very challenging disease. Available treatments are limited, mostly off-label and with high variability in the reported efficacy. Fortunately, a monoclonal antibody against tumor necrosis factor alpha has been recently approved for treatment of moderate to severe HS, offering patients a promising new option. This review focuses on the main features of HS, including epidemiology, clinical aspects, pathogenesis, severity classifications, comorbidities, and currently available treatments.

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          Most cited references116

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          Two Phase 3 Trials of Adalimumab for Hidradenitis Suppurativa.

          Hidradenitis suppurativa is a painful, chronic inflammatory skin disease with few options for effective treatment. In a phase 2 trial, adalimumab, an antibody against tumor necrosis factor α, showed efficacy against hidradenitis suppurativa.
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            Notch signaling is a direct determinant of keratinocyte growth arrest and entry into differentiation.

            The role of Notch signaling in growth/differentiation control of mammalian epithelial cells is still poorly defined. We show that keratinocyte-specific deletion of the Notch1 gene results in marked epidermal hyperplasia and deregulated expression of multiple differentiation markers. In differentiating primary keratinocytes in vitro endogenous Notch1 is required for induction of p21WAF1/Cip1 expression, and activated Notch1 causes growth suppression by inducing p21WAF1/Cip1 expression. Activated Notch1 also induces expression of 'early' differentiation markers, while suppressing the late markers. Induction of p21WAF1/Cip1 expression and early differentiation markers occur through two different mechanisms. The RBP-Jkappa protein binds directly to the endogenous p21 promoter and p21 expression is induced specifically by activated Notch1 through RBP-Jkappa-dependent transcription. Expression of early differentiation markers is RBP-Jkappa-independent and can be induced by both activated Notch1 and Notch2, as well as the highly conserved ankyrin repeat domain of the Notch1 cytoplasmic region. Thus, Notch signaling triggers two distinct pathways leading to keratinocyte growth arrest and differentiation.
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              Adalimumab for the treatment of moderate to severe Hidradenitis suppurativa: a parallel randomized trial.

              Hidradenitis suppurativa (HS) is a chronic, painful skin disease characterized by abscesses, nodules, and draining fistulas in the axilla and groin of young adults.
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                Author and article information

                Journal
                Clin Cosmet Investig Dermatol
                Clin Cosmet Investig Dermatol
                Clinical, Cosmetic and Investigational Dermatology
                Clinical, Cosmetic and Investigational Dermatology
                Dove Medical Press
                1178-7015
                2017
                19 April 2017
                : 10
                : 105-115
                Affiliations
                [1 ]Dipartimento di Medicina e Scienze della Salute “Vincenzo Tiberio”, Università degli Studi del Molise, Campobasso
                [2 ]Section of Dermatology, Department of Clinical Medicine and Surgery, University of Naples Federico II, Naples, Italy
                [3 ]Department of Dermatovenereology, Saint Petersburg State Pediatric Medical University, Saint Petersburg, Russia
                Author notes
                Correspondence: Maddalena Napolitano, Dipartimento di Medicina e Scienze della Salute “Vincenzo Tiberio”, Università degli Studi del Molise, C/da Tappino, 86100 Campobasso, Italy, Email maddalena.napolitano@ 123456unimol.it
                Article
                ccid-10-105
                10.2147/CCID.S111019
                5402905
                28458570
                de4c946e-2f78-45d5-970c-c277b7d3758b
                © 2017 Napolitano et al. This work is published and licensed by Dove Medical Press Limited

                The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

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                Categories
                Review

                Dermatology
                hidradenitis suppurativa,pathogenesis,diagnosis,treatment
                Dermatology
                hidradenitis suppurativa, pathogenesis, diagnosis, treatment

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