Contents. Chapter 1 Hidradenitis Suppurativa Introduction Albert Kligman References... 3

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1 Contents Chapter 1 Hidradenitis Suppurativa Introduction Albert Kligman References Chapter 2 Verneuil and Verneuil s Disease: an Historical Overview Gérard Tilles 2.1 Biographical Landmarks of a Surgeon-Venereologist L Hidradénite Phlegmoneuse (Verneuil s Disease), Primary Observations Further Observations and Discussions in Europe and Overseas Hidrosadenitis and Acne Conglobata: Controversial Views Acne Inversa, the Last Metamorphosis of Verneuil s Disease?.. 9 References Chapter 3 Clinical Presentation Florence Poli, Gregor B.E. Jemec, Jean Revuz 3.1 Introduction Individual Lesions Primary (Early) Lesions Secondary Lesions Tertiary Lesions Comedones Other Lesions Topography Involved Areas Two Main Zones The Two Less Frequently Involved Zones Atypical Localizations Other Zones May Be Involved Distribution of Lesions Evolution of the Disease and its Severity Age at Onset and Resolution Clinical Course Intermittent/Benign Course of the Disease Intermediate Course of the Disease Continuous Disease: Moderate, Severe Severity Indexes Hurley s Clinical Staging [2] Sartorius [9] Diagnosis Diagnostic Criteria Delay in Diagnosis Differential Diagnoses References Chapter 4 Pathology of Hidradenitis Suppurativa Alison Layton 4.1 Introduction Glandular Elements of the Skin Sebaceous Glands and the Pilosebaceous Unit Apocrine Glands Eccrine Glands Apoeccrine Glands Histological Appearance of HS Early Lesions Established Hidradenitis Suppurativa Immunohistochemistry of Hidradenitis Suppurativa Cytokeratin Expression in HS

2 4.6 Comparison with Other Disorders Fox Fordyce Disease Acne Follicular Occlusion Triad Pilonidal Sinus Crohn s Disease Conclusions Acknowledgements References Chapter 5 Imaging Ximena Wortsman, Gregor B.E. Jemec 5.1 Introduction Ultrasound Magnetic Resonance Imaging (MRI) X-Ray Examination Discussion References Chapter 6 Associated Diseases: Causality or Complications? Aude S. Nassif and Gregor B.E. Jemec 6.1 Introduction Cutaneous Diseases (Excluding Squamous Cell Carcinomas) Follicular Occlusion Diseases Acne Conglobata Dissecting Folliculitis of the Scalp Pilonidal Sinus Pigmentation Disorders of the Skin Folds: Dowling Degos and Kitamura s Diseases Rheumatological Disorders Clinical Picture Radiographic Features Treatment Mechanisms Associated Cancers Non-Skin Cancers Squamous Cell Carcinoma Incidence and Prevalence Clinical Picture Differential Diagnosis Treatment Prognosis Other Co-Occurrences Conclusion References Chapter 7 Hidradenitis Suppurativa and Crohn s Disease Philippe Seksik, Jean-François Contou, Anne Cosnes and Jacques Cosnes 7.1 Definition and Course of Crohn s Disease Pathophysiology Genetic Factors Enteric Microflora Immune-Mediated Tissue Injury Pathology Clinical Aspects Disease Location Clinical Presentation Therapeutic Aspects and Prognosis Cutaneous Crohn s Disease Clinical Presentation of Cutaneous Lesions Therapeutic Aspects and Prognosis Crohn s Disease and Hidradenitis Suppurativa Differential Diagnosis Co-Existence of HS and CD Treatment of Perianal Co-existent CD and HS References Chapter 8 Epidemiology Luigi Naldi 8.1 Introduction Descriptive Epidemiology Analytic Epidemiology Clinical Epidemiology: Natural History and Prognosis Perspectives References Chapter 9 Nosology and Classification Jean Revuz, Gregor B.E. Jemec and James Leyden 9.1 Introduction Morphology Anatomy Clinical Features Etiology; Pathogenesis Infection Inflammation

3 9.3.3 Hormones Treatments Conclusion Chapter 10 Genetics of Hidradenitis Suppurativa Jan von der Werth, Pam Wood, Alan D. Irvine, W.H. Irwin McLean 10.1 Clinical Genetics of Hidradenitis Suppurativa Genodermatoses Methods for Identifying Disease Genes Genetic Markers Positional Cloning of Dominantly Inherited Disease Genes Genome-Wide Linkage Analysis in Familial HS Linkage of Hidradenitis Suppurativa to 6q Identification of a Second HS Locus to Chromosome Candidate Genes Analysed in Linked HS Kindreds Oestrogen receptor- and HS ZNF91 and HS TIZ Association with HS Potential Contribution of the VIP Gene to HS Additional Candidate Gene-Protein Systems in HS Interleukin TNF Signalling and HS Discussion Conclusions References Chapter 11 Bacteriology of Hidradenitis Suppurativa Cristina Oprica, Carl Erik Nord 11.1 Normal Microflora of the Skin Bacteria Found in HS Lesions General Factors About Bacterial Involvement in HS Pathogenicity The Role of Antibiotics in the Treatment of HS Possible Consequences for Bacterial Ecology due to Antibiotic Treatment in HS References Chapter 12 Endocrinology Michel Faure, Evelyne Drapier-Faure 12.1 Introduction Hyperandrogenism and the Skin Androgenization Androgen Metabolism Causes of Hyperandrogenism Lack of Association between HS and Endocrinopathies HS and Biological Hyperandrogenism End-Organ Androgen Sensitivity?. 98 References Chapter 13 Immunity István Nagy, Lajos Kemény 13.1 Introduction Ancient and Modern: Innate and Acquired Immunity TLR/IL-1R Superfamily and their Signaling Pathways in the Skin Members of the TLR Family Expressed by Keratinocytes IL-1 Receptors in the Skin Signaling Pathways via TLR/IL-1R MyD88-Dependent Signaling Pathway MyD88-Independent Signaling Pathway Keratinocyte-Derived Effector Molecules in the Innate Immune System of the Skin Antimicrobial Peptides Defensins Cathelicidins RNase Antileukoprotease (ALP) Pro-Inflammatory Chemokines Pro-Inflammatory Cytokines TLR Recognition and the Commensal Microbiota of the Skin Skin Infections and Innate Immune Responses of the Epidermis Hidradenitis Suppurativa and the Skin Immune System Conclusions References

4 Chapter 14 Quality of Life in Hidradenitis Suppurativa Pierre Wolkenstein 14.1 Introduction Concept and Measure of Quality of Life Why Try to Measure Quality of Life? Reporting the Viewpoint of Patients: Methods of Measurement of Quality of Life Reporting the Viewpoint of Physicians: Uniform Outcome Variables Practical Approach Reconciling the Viewpoints of Patients and Physicians Impact of HS upon Quality of Life Altered Self-Reported Health: Qualitative Approach Measure of Quality of Life: Quantitative Approach Impaired Quality of Life: What Conclusion? Conclusion for Patients Conclusion for Physicians References Chapter 15 Antibiotic Therapy James Leyden, Jean Revuz 15.1 Introduction Clinical Experience Therapy References Chapter 16 Antiandrogens Evelyne Drapier-Faure, Michel Faure 16.1 Introduction Antiandrogens and Antiandrogen Therapies Cyproterone Acetate Spironolactone Finasteride Antiandrogenic Progestins Oral Contraceptives Antiandrogens in HS Cyproterone Acetate Spironolactone Finasteride References Chapter 17 Oral Retinoids for Hidradenitis Suppurativa Jurr Boer 17.1 Introduction Isotretinoin Mechanism of Action [1, 2] Clinical Experience Isotretinoin Monotherapy for HS Isotretinoin Therapy in Patients with Acne and Coexistent HS Isotretinoin in the Preand Postoperative Phase Isotretinoin in Combination Treatment of HS Side-Effects Etretinate and Acitretin Mechanism of Action Clinical Experience: Etretinate and Acitretin for HS Side-Effects References Chapter 18 Immunosuppressive Therapy in Hidradenitis Suppurativa Hanne Nybæk, Gregor B.E. Jemec 18.1 Introduction What Can We Achieve With Therapy? Immunosuppressive Therapies Prednisolone and Other Corticosteroids Ciclosporin Dapsone Methotrexate Hidradenitis Suppurativa as a Side-Effect of Immunosuppressive Drugs Practical Use of Immunosuppressive Therapy References

5 Chapter 19 Zinc and Other Experimental Medical Treatments Brigitte Dreno, Anabelle Brocard 19.1 Introduction Zinc Salts Mechanisms of Action: Modulation of the Proliferation and Differentiation of Keratinocytes and Cell Apoptosis Anti-Inflammatory Activity Activity Against 5 -Reductase Healing Zinc Salts in Verneuil s Disease Other Experimental Drugs References Chapter 20 Biologics For Hidradenitis Suppurativa (Verneuil s Disease in the Era of Biologics) Sharon E. Jacob, Francisco A. Kerdel 20.1 Introduction Background Crohn s Disease and Hidradenitis Suppurativa Co-Occurrence: A Rationale for Anti-TNF Therapy Anti-TNF Drugs in HS Future Prospects References Chapter 21 Topical Treatment Karin Sartorius, Jurr Boer, Gregor B.E. Jemec 21.1 Introduction Antibiotics Topical Clindamycin Keratolytics Resorcinol as a Keratolytic Agent in Hidradenitis Suppurativa Indications Mechanism of Action Side-Effects of Resorcinol Treatment Schedule with Resorcinol Creams Clinical Experience Topical Anti-Inflammatory Therapy Azelaic Acid Fusidic Acid Intralesional Therapy The Use of Local Therapy Publication Bias References Chapter 22 Surgery Jan Lapins, Lennart Emtestam 22.1 Introduction Methods of Closure Anatomical Regions Involved Axillary HS Inguinal Disease Gluteal-Perianal-Perineal Disease Excision Margins Complications Recurrent Disease After Surgery Carbon Dioxide Laser Surgery in HS Anesthesia Postoperative Wound Care Practical Hints and Comments The Carbon Dioxide Laser Technique The Use of Scanners How Radical Should the Therapy Be? Concluding Remarks References Chapter 23 Radiation Therapy Renato G. Panizzon 23.1 Introduction Treatment Regimens Case Series Reports Conclusion References Chapter 24 Experimental Physical Therapies Gregor B.E. Jemec 24.1 Introduction Photodynamic Therapy Depilation Cryosurgery Discussion Practical Guidelines for Testing New Physical Therapies References

6 Chapter 25 Treatment Gregor B.E. Jemec, Jean Revuz 25.1 Introduction Staging the Disease Treatment of Hidradenitis Suppurativa Hurley Stage I Treatment of Hidradenitis Suppurativa Hurley Stage II Treatment of Hidradenitis Suppurativa Hurley Stage III Hidradenitis Suppurativa with Cysts Experimental Therapies General Requirements for Treatment Chapter 26 Hidradenitis Suppurativa Patients Frequently Asked Questions Jean Revuz 26.1 General Questions Heredity Pregnancy Treatment Practical Questions Hints For Daily Life Relations To Other Factors Psychological Aspects Children and Adolescents Social Security Chapter 27 An Uncommon Valor Sylvia Shawcross 27.1 Kevin Deborah Elizabeth Melony Kerrie John Caroline Arnold Mabel Nassim Mira Mindy Subject Index