Autoimmune Bullous Diseases: Text and Review

Preface
References
Contents
Contributors

Part I: Immunology and Diagnostic Tests

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
References
Suggested Further Reading
2: Dermatological Examination of Bullous Diseases
Introduction and Aims
Short Definition in Layman Terms
Learning Objectives
Didactical Questions: Cross Section of Questions to Prime the Readers Interest
Facts and 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)
References
Additional Reading
On the Web
3: How to Take a Biopsy
Introduction and AIMS
Short Definition in Layman Terms
Didactical Questions: Cross Section of Questions to Prime the Readers’ Interest
Histopathology
Procedure of the Punch Biopsy
What to Biopsy
Transport and Handling of Biopsies
Direct Immunofluorescence
Procedure of the Biopsy
Skin
Buccal Mucosa
Conjunctiva
What to Biopsy
Transport and Handling of Biopsies
References
Additional Reading
On the Web
4: Direct Immunofluorescence Microscopy
Introduction and Aims
Laboratory Preparation
Immunofluorescence Patterns
Pemphigus
Pemphigoid
Dermatitis Herpetiformis
Porphyria Cutanea Tarda and Pseudoporphyria
References
5: Indirect Immunofluorescence Microscopy
Introduction
Technique
Monkey Esophagus
Human Salt-Split Skin
Rat Bladder
Knock-Out Skin
References
6: Immunoassays
Introduction and AIMS
Immunoblotting
Immunoprecipitation
ELISA
References

Part II: Pemphigus

7: Structure of Desmosomes
Introduction and Aim
Desmosomes: Cell-Cell Adhesion Structures
Desmosomal Proteins and Their Isoforms
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
Immunosuppressive Agents
High-Dose Human Intravenous Immunoglobulin
Plasmapheresis and Immunoadsorption
Rituximab
Follow-Up and Tapering
References
Additional Reading
On the Web
9: 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 Escalator
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
References
Further Reading
On the Web
10: Paraneoplastic Pemphigus
Introduction and Aims
Short Definition in Layman’s Terms
Didactical Questions
Facts and Figures
Definitions and Classification
Epidemiology
Pathogenesis
Diagnosis Paths
History and Physical Examination
Diagnostics
Treatment and Prognosis
References
11: IgA Pemphigus
Introduction and Aims
Short Definition in Layman’s 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
References
For Further Reading
On the Web
12: Drug-Induced Pemphigus
Short Introduction in Layman Terms
Facts and Figures
Diagnosis Paths
Treatment Tricks
References
On the Web

Part III: Pemphigoids

13: Structure of Hemidesmosomes and the Epidermal Basement Membrane Zone
Introduction and Aims
Facts and Figures
Hemidesmosomes
Epidermal Basement Membrane Zone
References
Additional Reading
On the Web
14: Cutaneous Pemphigoid
Bullous Pemphigoid
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
Nonbullous Cutaneous 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 and Aims
Facts and Figures
Clinical Symptoms
Diagnosis Paths
Treatment Tricks
Anti-Plectin Pemphigoid
References
Further Reading
On the Web
15: Mucous Membrane Pemphigoid
Mucous Membrane Pemphigoid
Short Definition in Layman’s 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’s Terms
Clinical Symptoms
Diagnosis Paths
Treatment Tricks
Localized Vulvar Pemphigoid
Short Definition in Layman’s Terms
Definitions and Classification
Clinical Symptoms
Diagnosis Paths
Treatment Tricks
Anti-laminin 332 Mucous Membrane Pemphigoid
Introduction
Short Definition in Layman’s Terms
Definitions and Classification
Pathogenesis
Clinical Symptoms
Diagnosis Paths
Treatment Tricks
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 Escalator
Follow-Up and Tapering
References
Additional Reading
On the Web
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 Escalator
Follow-Up and Tapering
References
Additional Reading
On the Web
18: Linear IgA Disease
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
References
On the Web
19: Drug-Induced Pemphigoid and Linear IgA Disease
Short Introduction in Layman Terms
Drug-Induced Pemphigoid
Facts and Figures
Contact Pemphigoid
Diagnosis Paths
Treatment Tricks
Drug-Induced Linear IgA Disease
Facts and Figures
Diagnosis Paths
Treatment Tricks
References

Part IV: Other Inflammatory Bullous Diseases

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
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
Facts and Figures
Definitions and Classification
Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN)
Facts and Figures
Epidemiology
Pathogenesis and Aetiology
Aetiology
Diagnosis Paths
History and Physical Examination
General Diagnostics
Specific Diagnostics
Treatment Tricks
Initial Treatment and Escalator
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
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 Escalator
Follow-Up and Tapering
References
Additional Reading
On the Web
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
References
Additional Reading
On the Web

Part V: Resources

24: Patient Support Groups and International Centers for AIBD
Index