Autoimmune Bullous Diseases: Text and Review

Description

This extensively revised new edition provides a quick study and reference guide for physicians who treat patients with autoimmune bullous diseases (AIBDs), the most life-threatening inflammatory diseases of the skin. AIBDs are rare, comprising less than 1% of the dermatological patients and as such, they are often given little focus in general dermatology literature, despite being complicated and demanding diseases to manage. Chapters cover the latest developments in diagnostic assays and the use of pemphigoids. New contributions include how AIBD affects children and the care of wounds in instances of blistering disease from the perspective of a nurse practitioner.   Autoimmune Bullous Diseases: Text and Review is written in a direct and simple style with tables and figures to illustrate this difficult field practically. At the end of each chapter the reader is challenged to check the learned matter with multiple-choice questions, while it contains protocols and follows international guidelines, making it suitable study material for courses on autoimmune bullous diseases. Residents and researchers in dermatology and adjacent disciplines will find this book useful as a quick guide when dealing with these patients. Preface to the Second Edition Contents 1: Basic Principles of the Immune System and Autoimmunity The Immune System: A Short Introduction Two Systems: The Innate and Adaptive System The Adaptive System in More Detail A Closer Look at the Skin Autoimmunity Review Questions References Suggested Further Reading 2: Dermatological Examination of Bullous Diseases Introduction & AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers Interest Facts & Figures Definitions and Classification Symptoms Signs Definitions and Activity Scores Pemphigus Definitions Pemphigus Disease Area Index (PDAI) Bullous Pemphigoid Bullous Pemphigoid Area Index (BPDAI) Mucous Membrane Pemphigoid Area Index (MMPDAI) Review Questions Answers On the web References Additional Reading 3: How to Take a Biopsy Introduction & AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers Interest Histopathology Procedure of the Punch Biopsy Biopsy Site Transport and Handling of Biopsies Direct Immunofluorescence Procedure of the Biopsy Skin Buccal Mucosa Conjunctiva Biopsy Sites (Table 3.1) Transport and Handling of Biopsies Review Questions Answers On the Web References Additional Reading 4: Direct Immunofluorescence Microscopy Introduction & Aims Laboratory Preparation Immunofluorescence Patterns Pemphigus Pemphigoid Dermatitis Herpetiformis Porphyria Cutanea Tarda and Pseudoporphyria Review Questions Answers References 5: Indirect Immunofluorescence Microscopy Introduction Technique Monkey Esophagus Human Salt Split Skin Rat bladder Knock-Out Skin Review Questions Answers References 6: Immuno-Assays Introduction & AIMS Immunoblotting Immunoprecipitation ELISA Keratinocyte Binding Assay Keratinocyte Footprint Assay Review Questions Answers References 7: Structure of Desmosomes Introduction and Aim Desmosomes: Cell-Cell Adhesion Structures Desmosomal Proteins and Their Isoforms Review Questions Answers References Additional Reading 8: Pemphigus Vulgaris Introduction and AIMS Short Definition in Layman Terms Didactical Questions: Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Desmoglein Compensation Hypothesis Steric Hindrance Cell Signalling Assembly and Disassembly Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Immunological Tests Direct Immunofluorescence Indirect Immunofluorescence ELISA Treatment Tricks Initial Treatment and Escalator Systemic Corticosteroids Rituximab Immunosuppressive Agents High Dose Human Intravenous Immunoglobulin Plasmapheresis and Immunoadsorption Follow-Up and Tapering Review Questions Answers On the Web References 9: Pemphigus Foliaceus Pemphigus Foliaceus Introduction and AIMS Short Definition in Layman Terms Didactical Questions: Cross Section of Questions to Prime the Reader’s Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Endemic Pemphigus Introduction and AIMS Facts and Figures Diagnosis Paths Treatment Tricks Pemphigus Herpetiformis Introduction Facts and Figures Diagnosis Paths Treatment Tricks Pemphigus Erythematosus Introduction Facts and Figures Diagnosis Paths Treatment Tricks Review Questions Answers On the Web References Further Reading 10: Paraneoplastic Pemphigus Introduction and AIMS Short Definition in Layman Terms Didactical Questions Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination Diagnostics Treatment and Prognosis Review Questions Answers References 11: IgA Pemphigus Introduction and AIMS Short Definition in Layman Terms Didactical Questions: Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Review Questions Answers On the Web References Further Reading 12: Drug-Induced Pemphigus Short Introduction in Layman Terms Facts and Figures Diagnosis Paths Treatment Tricks Review Questions Answers On the Web References 13: Structure of Hemidesmosomes and the Epidermal Basement Membrane Zone Introduction and Aims Facts and Figures Hemidesmosomes Epidermal Basement Membrane Zone Review Questions Answers On the Web References Additional Reading 14: Pemphigoid Diseases Affecting the Skin Bullous Pemphigoid Introduction and Aims Short Definition in Layman Terms Didactical Questions: Cross Section of Questions to Prime the Reader’s Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Nonbullous Pemphigoid Short Definition in Layman Terms Definitions and Classification Epidemiology Pathogenesis Clinical Symptoms Diagnosis Paths Treatment Tricks Brunsting-Perry Cicatricial Pemphigoid Short Definition in Layman Terms Facts and Figures Clinical Symptoms Diagnosis Paths Treatment Tricks Lichen Planus Pemphigoides Introduction and AIMS Facts and Figures Diagnosis Paths Treatment Tricks Pemphigoid Gestationis Short Definition in Layman Terms Facts and Figures Epidemiology Pathogenesis Clinical Symptoms Diagnosis Paths Treatment Tricks Anti-p200 Pemphigoid Introduction Facts and Figures Clinical Symptoms Diagnosis Paths Treatment Tricks Review Questions Answers References 15: Mucous Membrane Pemphigoid Mucous Membrane Pemphigoid Short Definition in Layman Terms Didactical Questions: Cross Section of Questions to Prime the Readers Interest Facts and Figures Diagnosis Paths History and Physical Examination Diagnostics Treatment Tricks Initial Treatment and Treatment Ladder Follow-Up and Tapering Ocular Mucous Membrane Pemphigoid Short Definition in Layman Terms Clinical Symptoms Diagnosis Paths Treatment Tricks Localized Vulvar Pemphigoid Short Definition in Layman Terms Definitions and Classification Clinical Symptoms Diagnosis Paths Treatment Tricks Anti-laminin-332 Mucous Membrane Pemphigoid Introduction Short Definition in Layman Terms Definitions and Classification Pathogenesis Clinical Symptoms Diagnosis Paths Treatment Tricks Review Questions Answers References 16: Epidermolysis Bullosa Acquisita Introduction and AIMS Short Definition in Layman Terms Didactical Questions: Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Review Questions Answers References Additional Reading 17: Bullous Systemic Lupus Erythematosus Introduction and AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Review Questions Answers References Additional Reading 18: Linear IgA Bullous Dermatosis Introduction and AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Review Questions Answers On the Web References 19: Drug-Induced Pemphigoid and Linear IgA Disease Short Introduction in Layman Terms Facts and Figures Contact Pemphigoid Diagnosis Paths Treatment Tricks Facts and Figures Diagnosis Paths Treatment Tricks Review Questions Answers: References 20: Dermatitis Herpetiformis Introduction and AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers’ Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Escalator Follow-Up and Tapering Review Questions Answers References 21: Stevens Johnson Syndrome/Toxic Epidermal Necrolysis and Erythema Exsudativum Multiforme Introduction and Aims Short Introduction in Layman Terms Didactical Questions; Cross Sections to Prime the Readers Interest Definitions and Classification Stevens Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) Facts and Figures Epidemiology Pathogenesis Aetiology Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Erythema Exsudativum Multiforme (EEM) Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Review Questions Answers References Additional Reading 22: Porphyria Cutanea Tarda and Pseudoporphyria Introduction and AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Tapering Review Questions Answers References Additional Reading 23: Bullous Dermatitis Artefacta Introduction and AIMS Short Definition in Layman Terms Didactical Questions; Cross Section of Questions to Prime the Readers Interest Facts and Figures Definitions and Classification Epidemiology Pathogenesis Diagnosis Paths History and Physical Examination General Diagnostics Specific Diagnostics Treatment Tricks Narrow Escape Dual Approach Review Questions Answers On the Web References Additional Reading 24: Autoimmune Bullous Diseases in Childhood Introduction and Aims Short Definition in Layman Terms Didactical Questions Facts and Figures Diagnosis Paths History and Physical Examination Diagnostics Treatment Tricks Initial Treatment and Therapeutic Ladder Follow-Up and Long-Term Prognosis Review Questions Answers References 25: Wound Care in Autoimmune Bullous Diseases Introduction and Aims Didactical Questions Goals and Challenges in Wound Care in AIBDs Factors Influencing Wound Healing in AIBDs The ‘Mise En Place’ for Proper Wound and Skin Care Severity of the Disease Pain Management Infection Prevention Skin and Mucosal Wound Care in AIBDs Removal of Dressing and Cleansing of the Wounds Blister Management Wound Care and Dressings Frequency of Dressing Changes Mucosae Wound Care Plan Conclusion Review Questions Answers References Appendix A: Patient Support Groups and International Centers for AIBD Index