Urticaria and Angioedema (eBook)

Urticaria and Angioedema 2
Title Page 3
Copyright page 4
Preface 5
Contents 6
Contributors 8
Chapter 1 10
History of Urticaria 10
1.1 Introduction 11
1.2 The Cellular and Molecular Basis of Urticaria: First Steps 11
1.3 The Enigma of Chronic “Idiopathic” Urticaria 12
1.4 Treatment of Urticaria: Antihistamines 13
1.5 Take Home Pearls 14
References 15
Chapter 2 17
Aetiopathogenesis of Urticaria 17
2.1 Lessons from Histopathology 18
2.2 A Central Role for the Mast Cell 19
2.2.1 Mast Cell Mediators of Urticaria 20
2.2.1.1 Histamine 20
2.2.1.2 Cysteinyl Leukotrienes 20
2.3 Involvement of Other Infl ammatory Cells in Urticaria 21
2.4 Urticaria Not Implicating the Mast Cell (Non-histaminergic) 22
2.5 What Causes Mast Cell Mediator Release in the First Place? 23
2.5.1 Immunological Stimuli of Mast Cell Secretion 25
2.5.1.1 Allergens 25
2.5.1.2 Autoantibodies 25
2.5.1.3 Complement 26
2.5.2 Mast Cell Liberators and Pseudoallergens 26
2.6 Tests for Autoimmune Urticaria 26
2.6.1 Laboratory Assays 26
2.6.2 The Autologous Serum Skin Test 27
2.7 A Concept Model for Understanding Chronic Urticaria 27
2.8 Clinical Take Home Pearls 29
References 29
Chapter 3 32
Classification of Urticaria 32
3.1 Definition 32
3.2 Histology 35
3.3 Classifi cation of Urticaria on the Basis of Its Symptoms, Duration, Frequency, and Causes 35
3.4 Take Home Pearls 38
References 38
Chapter 4 39
Impact of Urticaria: QOL and Performance 39
4.1 Take Home Pearls 42
References 42
Chapter 5.1 43
Acute Urticaria 43
5.1.1 Definition 43
5.1.2 Epidemiology 44
5.1.3 Clinical Aspects 44
5.1.4 Aetiology 45
5.1.5 Natural Course 46
5.1.6 Diagnosis 47
5.1.7 Treatment 47
5.1.8 Take Home Pearls 48
References 48
Chapter 5.2 50
Chronic Urticaria 50
5.2.1 Definition 50
5.2.2 Clinical Picture 52
5.2.3 Epidemiology 52
5.2.4 Pathogenesis 52
5.2.5 Causes and Triggers 53
5.2.5.1 CU Due to Autoreactivity 53
5.2.5.2 CU Due to Infection 55
5.2.5.3 CU Due to Intolerance 55
5.2.5.4 CU Due to Other Causes 55
5.2.5.5 Triggers and Eliciting Factors 56
5.2.6 Diagnostics 56
5.2.7 Therapy 57
5.2.7.1 Removing Underlying Causes, Relevant Triggers and Aggravating Factors 57
5.2.7.2 Mast Cell-Targeting Therapies 58
5.2.7.2.1 Inhibitors of Mast Cell Activation 58
5.2.7.2.2 Antagonists of Mast Cell Activators 59
5.2.7.3 Blocking of Mast Cell Mediators 59
5.2.8 Take Home Pearls 59
References 60
Chapter 5.3 62
Dermographic and Delayed Pressure Urticaria 62
5.3.1 Symptomatic Dermographism (Dermatographism: Urticaria Factitia) 62
5.3.1.1 Making the Diagnosis 62
5.3.1.2 Taking the History 62
5.3.1.3 Clinical Findings 63
5.3.1.4 Confi rming the Diagnosis 63
5.3.1.5 Diagnostic Implications and Treatment 63
5.3.1.6 Take Home Pearls 64
5.3.2 Delayed Pressure Urticaria 64
5.3.2.1 Making the Diagnosis 65
5.3.2.2 Taking the History 65
5.3.2.3 Clinical Features 65
5.3.2.4 Diagnostic Implications and Treatment 66
5.3.2.5 Take Home Pearls 66
References 67
Chapter 5.4 68
Heat and Cold Urticaria 68
5.4.1 Introduction: The Disease Spectrum 68
5.4.2 Cold and Heat Urticaria 69
5.4.2.1 Epidemiology 69
5.4.2.2 Pathogenesis 70
5.4.2.3 Clinical Features 71
5.4.2.4 Confi rmation of the Diagnosis 71
5.4.2.5 Differential Diagnosis 72
5.4.2.6 Treatment 73
5.4.2.6.1 Pharmacological Treatment 73
5.4.2.6.2 Tolerance Induction 73
5.4.2.7 Prognosis 74
5.4.3 Take Home Pearls 74
References 74
Chapter 5.5 77
Solar Urticaria 77
5.5.1 Clinical Features 77
5.5.2 Pathogenesis 78
5.5.3 Treatment and Prognosis 81
5.5.4 Take Home Pearls 82
References 82
Chapter 5.6 85
Cholinergic Urticaria and Exercise-Induced Anaphylaxis 85
5.6.1 Introduction: The Disease Spectrum 85
5.6.2 Cholinergic Urticaria 86
5.6.2.1 Epidemiology 86
5.6.2.2 Pathogenesis 86
5.6.2.3 Clinical Features 87
5.6.2.4 Confirmation of the Diagnosis 88
5.6.2.5 Differential Diagnosis 88
5.6.2.6 Treatment 89
5.6.2.7 Prognosis 89
5.6.3 Exercise-Induced Anaphylaxis 90
5.6.4 Take Home Pearls 91
References 91
Chapter 5.7 94
Contact Urticaria 94
5.7.1 Etiology and Pathogenesis 94
5.7.2 Clinical Findings 96
5.7.3 Diagnostic Approach 97
5.7.4 Course and Prognosis 97
5.7.5 Therapy 97
5.7.6 Take Home Pearls 97
References 98
Chapter 5.8 99
Urticarial Syndromes and Autoinfl ammation 99
5.8.1 Distinct Molecular Pathways Underlying Urticarial Syndromes Associated with Autoinflammation 100
5.8.2 Urticaria: Inherited or Acquired 100
5.8.3 Familial Urticarial Syndromes 100
5.8.4 Genotype–Phenotype Disparity in Autoinfl ammatory Disease 104
5.8.5 Nonfamilial Autoinfl ammatory Syndromes: Acquired or Polygenic Etiology 106
5.8.6 Diagnosis 106
5.8.7 Treatment 107
5.8.8 Take Home Pearls 107
References 108
Chapter 5.9 111
Urticaria Vasculitis 111
5.9.1 Definition 112
5.9.2 Epidemiology 112
5.9.3 Histopathologic Findings 112
5.9.4 Pathophysiology 113
5.9.5 Clinical Features 113
5.9.6 Systemic Manifestations 114
5.9.7 Differential Diagnosis and Investigations 115
5.9.8 Prognosis 116
5.9.9 Treatment 116
5.9.10 Take Home Pearls 116
References 118
Chapter 5.10 119
Angioedema 119
5.10.1 Classification of Angioedema 120
5.10.2 Pathophysiology of Angioedema 122
5.10.3 Clinical Features of Angioedema 122
5.10.4 Differential Diagnosis of Angioedema 123
5.10.5 Clinical Approach to a Patient with Angioedema 124
5.10.6 Recent Advances and Perspectives 125
5.10.7 Take Home Pearls 126
References 127
Chapter 6 130
Therapy of Urticaria 130
6.1 General Treatment Advice for All Urticaria Subtypes 131
6.2 Evidence-Based Treatment of Spontaneous Urticaria 131
6.2.1 Spontaneous Acute Urticaria 131
6.2.2 Spontaneous Chronic Urticaria 134
6.2.2.2 Diet 134
6.2.2.3 Non-Sedating H1 Antihistamines 135
6.2.2.4 H2-Antihistamines 135
6.2.2.5 Glucocorticosteroids 135
6.2.2.6 Leukotriene Antagonists 136
6.2.2.7 Cyclosporin A 136
6.2.2.8 Thyroid Hormones 136
6.2.2.9 Other Therapeutic Options 137
6.3 Evidence-Based Treatment of Physical Urticaria 137
6.4 Evidence-Based Treatment of Special Urticaria Subtypes 138
6.5 Take Home Pearls 138
References 139
Chapter 7 141
Standard Operating Procedures: A Practical Approach 141
7.1 Assessing Urticaria Activity Score 142
7.2 Testing for Autoreactivity 142
7.3 Testing for Food Elicited Urticaria Diet 144
7.4 Ins and Outs of the Food Labelling Rules 145
7.5 Use of Diary Cards 146
7.6 Questionnaire 146
7.7 Prick Test 149
7.8 Take Home Pearls 151
References 151
Appendix 152
Index 154

"6 Therapy of Urticaria (p. 129-130)

Bettina Wedi

Core Messages

The different urticaria subtypes are common skin diseases persisting often for › years with multifactorial triggering factors and greatly reduced quality of life as well as occupational disability. Therapy can be problematic and is not standardised. Regarding the long duration › of this annoying skin disease, every attempt should be made to identify and avoid speci? c or unspeci? c triggering factors. The treatment goal is to maximise the quality of life, to maintain vigilance and ability to work or to attend school and to minimise drug-related side effects.

Besides antihistamines and the eradication or avoidance of identi? ed triggering › factors, many drugs, most of which are not licensed for urticaria, are administered. The highest grade of recommendation (GoR) according to the criteria of › evidence-based medicine can only be given for second-generation H1 antihistamines. They should be administered in an adequate dose, up to fourfold the normal dose while considering potential side effects. The data on treatment alternatives are totally insuf? cient. Good evidence exists in › severely affected patients for cyclosporin A. Subgroups might bene? t from addition of leukotrienes antagonists. Chloroquine and dapsone may be worthy of further investigation within randomised controlled trials.

The risk–bene? t pro? le of each alternative (off-label use) to second-generation › H1 antihistamines should be carefully considered before treatment. This applies especially to immunosuppressive agents. The clinical pictures of urticaria are of heterogeneous aetiology and therefore subclassi? ed into distinct groups, which can coexist: spontaneous urticaria, physical urticaria and special types. To optimise urticaria treatment, the ? rst strategy should be to correctly classify the respective urticaria subtype. The treatment goal is to maximise the quality of life and ability to work or to attend school and to minimise drug-related side effects.

This is of particular importance regarding the long year duration of many urticaria subtypes. Relevant outcomes of treatment trials include pruritus, weal size, number and frequency, loss of awakening, overall physician and patient assessment and permanent remission of disease. This chapter is based on the results of two recent publications evaluating randomised controlled trials (RCTs) of urticaria therapy (until October 2004) according to the criteria of evidence-based medicine [1, 2 ].

Details of the individual studies, inclusion and exclusion criteria as well as the cited literature are available in the publications [1, 2 ], if not otherwise cited. Additionally, this review includes at least the RCTs published until December 2006 [3–7 ] and also interesting treatment approaches. In the text, the respective GoR according to the criteria of evidence-based medicine of the Scottish Intercollegiate Guidelines Network (SIGN; www.sign.ac.uk, GoR A to D, whereas A represents best evidence) is given in parentheses. Table 6.1 summarises the recommended therapeutic approaches."