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.
Author(s): Barbara Horváth
Edition: 2
Publisher: Springer
Year: 2022
Language: English
Pages: 215
City: Cham
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
