A Practical Guide to Vulval Disease
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FIONA LEWIS, St John's Institute of Dermatology, Guy's & St Thomas' Hospital and Wexham Park Hospital (Frimley Health Foundation Trust), UK.
FABRIZIO BOGLIATTO, Chivasso Civic Hospital in Turin, Italy.
MARC VAN BEURDEN, Netherlands Cancer Institute.
Content
Acknowledgements xvii
1 The Normal Vulva 1
Normal Vulval Anatomy 1
Normal Vulval and Vaginal Flora 4
Further Reading 4
Normal Anatomical Variants 4
Normal Changes Over the Lifetime 6
Childhood 6
Pregnancy 6
Menopause 6
Further Reading 7
2 Taking a History and Examination 9
Taking a History 9
General Medical History 9
Gynaecological History 10
Dermatological History 10
Vulval History 10
Examination of the Vulva 11
Further Reading 13
3 How to Take a Vulval Biopsy and the Importance of Clinico-Pathological Correlation 15
Incisional Biopsy 16
Fixation of the Biopsy 20
Further Reading 21
4 Basic Histology of the Vulva 23
Special Histological Stains 24
Immunohistochemical Stains 26
Other Tests 27
Further Reading 28
5 Investigations in Vulval Disease 29
Investigations for Infection 29
Bacterial Swabs 29
Viral Swabs 29
Fungal Scrapings 29
Wood's Light Examination 32
Serological Tests 32
Investigations for Allergy 32
Rast Tests 32
Prick Tests 32
Patch Tests (see Chapter 9) 32
Investigations for Inflammatory Disease 33
Direct Immunofluorescence 33
Indirect Immunofluorescence 34
Imaging Investigations 34
Useful Web Sites for Patient Information 34
Further Reading 34
6 Topical Treatment in Vulval Disease 35
Introduction 35
General Principles 35
Lotions 35
Gels 36
Ointments 36
Creams 36
Pastes 36
Topical Treatments Used for Vulval Disease 36
Emollient 37
Topical Steroids 38
Adverse Effects 38
Topical Steroids in Pregnancy 41
Antiseptics 41
Antibacterials 42
Antifungals 42
Barriers 42
Others 42
Podophyllotoxin 42
Imiquimod 42
Calcineurin Inhibitors 43
Tar 44
Emla ® 44
Practice Points 44
Further Reading 44
Useful Web Sites for Patient Information 44
7 Symptoms in Vulval Disease 45
Pruritis (Itch) 45
Causes of Vulval Pruritis 46
Soreness 46
Pain 47
Dyspareunia 48
Discharge 48
No Symptoms 49
Useful Web Site for Patient Information 49
8 Signs in Vulval Disease 51
Differential Diagnosis Based on Appearance 51
Algorithms 51
Further Reading 56
9 Eczema, Allergy and the Vulva 57
Seborrhoeic Eczema 57
Incidence 57
Pathophysiology 57
Symptoms 58
Clinical Features 58
Basic Management 58
When to Refer 58
Practice Points 59
Further Reading 59
Useful Web Sites for Patient Information 59
Allergic Contact Eczema / Dermatitis 59
Introduction 59
Incidence 59
Pathophysiology 60
Patch Testing (see Chapter 5) 60
Symptoms 60
Clinical Features 60
Basic Management 60
Who to Refer for Patch Testing 60
Practice Points 61
Further Reading 61
Useful Web Sites for Patient Information 62
Irritant Eczema / Dermatitis 62
Introduction 62
Incidence 62
Pathophysiology 63
Histological Features 63
Symptoms 63
Clinical Features 63
Basic Management 64
When to Refer 64
Practice Points 64
Further Reading 64
Urticaria 64
Allergic Contact Urticaria 64
Clinical Features 65
Management 65
References 65
10 Psoriasis 67
Introduction 67
Incidence 67
Pathophysiology 67
Histological Features 67
Symptoms 68
Clinical Features 68
Basic Management 69
When to Refer 72
Practice Points 73
Further Reading 73
Useful Web Sites for Patient Information 73
11 Lichen Simplex 75
Introduction 75
Epidemiology 75
Incidence 75
Pathophysiology 75
Histological Features 76
Symptoms 76
Clinical Features 76
Basic Management 77
When to Refer 78
Practice Points 78
Further Reading 79
Useful Web Sites for Patient Information 79
12 Lichen Sclerosus 81
Introduction 81
Incidence 81
Pathophysiology 81
Genetic 82
Epigenetics 82
Autoimmunity 82
Hormonal Factors 82
Infection 82
Trauma 82
Role of the Skin Immune System 82
Histological Features 82
Symptoms 83
Clinical Features 83
Associated Disease 90
Risk of Malignancy 90
Basic Management 92
Follow Up 92
When to Refer 92
Practice Points 93
Further Reading 93
Useful Web Sites for Patient Information 93
13 Lichen Planus 95
Introduction 95
Epidemiology 95
Incidence 95
Pathophysiology 95
Histological Features 96
Clinical Features of Lichen Planus 96
Vulval Lichen Planus 98
Classic LP 98
Hypertrophic LP 98
Erosive LP 98
Risk of Malignancy 99
Basic Management 100
When to Refer 101
Practice Points 102
Further Reading 102
Useful Web Sites for Patient Information 102
14 Hidradenitis Suppurativa and Crohn's Disease 103
Hidradenitis Suppurativa 103
Introduction 103
Epidemiology 103
Incidence 103
Pathophysiology 103
Histological Features 103
Symptoms 104
Clinical Features 104
Basic Management 105
Medical Management 105
Surgery 105
When to Refer 105
Practice Points 106
Further Reading 106
Useful Web Sites for Patient Information 106
Crohn's Disease 106
Introduction 106
Epidemiology 106
Pathophysiology 107
Histological Features 107
Symptoms 107
Clinical Features 107
Basic Management 109
When to Refer 109
Practice Points 109
Further Reading 110
Useful Web Sites for Patient Information 110
15 Disorders of Pigmentation on the Vulva 111
Introduction 111
Postinflammatory Pigmentation 111
Post-Traumatic Pigmentation 111
Acanthosis Nigricans 111
Management 113
Melanosis 114
Pigmented Lesions 115
History and Examination 115
Other Techniques 116
Lentigines 116
Benign Naevi 116
Atypical Genital Naevi 117
Seborrhoeic Keratoses 117
Pigmented Basal Cell Carcinoma 118
Hypopigmentation 118
Vitiligo 118
When to Refer 119
Practice Points 119
Further Reading 119
Useful Web Sites for Patient Information 120
16 Other Dermatoses 121
Genetic Disorders 121
Hailey-Hailey Disease (Familial Benign Chronic Pemphigus) 121
Management 121
When to Refer 121
Practice Points 121
Further Reading 122
Useful Web Site for Patient Information 122
Auto-Immune Bullous Disease 123
Practice Points 126
Further Reading 126
Drug Eruptions 126
Fixed Drug Eruption 126
Common Drugs Causing a Fixed Drug Eruption 126
Further Reading 126
Useful Web Site for Patient Information 126
Stevens-Johnson Syndrome 127
Symptoms 127
Clinical Features 127
Toxic Epidermal Necrolysis 127
Symptoms 127
Clinical Features 127
Basic Management 127
Further Reading 128
Useful Web Site for Patient Information 128
Manifestations of Underlying Disease 128
Necrolytic Migratory Erythema 128
Symptoms 129
Clinical Features 129
Basic Management 129
Further Reading 129
Acrodermatitis Enteropathica 129
Clinical Features 129
Basic Management 129
Practice Point 130
Further Reading 130
Inflammatory Ulcers 130
Aphthous Ulcers 130
Clinical Features 130
Management 130
Practice Point 130
Behcet's Syndrome 130
Further Reading 132
Useful Resources 132
Lipschutz Ulceration 132
Clinical Features 132
Basic Management 133
Further Reading 133
Others 133
Graft-Versus-Host Disease 133
Further Reading 134
Zoon's Vulvitis (Plasma Cell Vulvitis) 134
Histology 134
Symptoms 134
Clinical Features 135
Basic Management 135
Further Reading 135
Vulvovaginal Adenosis 135
Further Reading 135
Langerhans Cell Histiocytosis 136
Further Reading 136
17 Vulval Infection - Sexually Transmitted 137
Normal Flora 137
Trichomoniasis 137
Pathophysiology 137
Clinical Features 138
Diagnosis 138
Basic Management 138
Further Reading 138
Useful Web Sites for Patient Information 138
Chlamydia 139
Clinical Features 139
Diagnosis 139
Treatment 139
Further Reading 139
Useful Web Site for Patient Information 139
Lymphogranuloma Venereum 139
Clinical Features 140
Diagnosis 140
Treatment 140
Further Reading 140
Useful Web Site for Patient Information 140
Gonorrhoea 140
Clinical Features 140
Diagnosis 140
Treatment 141
Further Reading 141
Useful Web Sites for Patient Information 141
Syphilis 141
Pathophysiology 141
Clinical Features 141
Treatment 142
Further Reading 142
Useful Web Site for Patient Information 142
Chancroid 142
Pathophysiology 142
Clinical Features 143
Treatment 143
Further Reading 143
Useful Web Sites for Patient Information 143
Donovanosis (Granuloma Inguinale) 143
Clinical Features 143
Diagnosis 143
Treatment 143
Further Reading 144
Useful Web Site for Patient Information 144
Herpes Simplex Infection 144
Incidence 144
Pathophysiology 144
Clinical Features 144
Differential Diagnosis 145
Diagnosis 146
Basic Management 146
Hsv Infection and Pregnancy 146
Further Reading 147
Useful Web Sites for Patient Information 147
Human Papillomavirus Infection 147
Epidemiology 147
Pathophysiology 148
The Concept of Vulval Subclinical Lesion 148
Histology 148
Clinical Features 149
Diagnosis 149
Treatment 150
Hpv Vaccine 150
Genital Warts in Children 150
Further Reading 150
Useful Web Sites for Patient Information 150
Molluscum Contagiosum 150
Symptoms 151
Clinical Appearance 151
Diagnosis 151
Treatment 151
Further Reading 152
Useful Web Site for Patient Information 152
Scabies 152
Clinical Features 152
Diagnosis 152
Treatment 153
Further Reading 153
Useful Web Site for Patient Information 153
Pubic Lice 153
Clinical Features 153
Treatment 153
Useful Web Site for Patient Information 153
18 Vulval Infection - Nonsexually Transmitted 155
Bacterial Infections 155
Bacterial Vaginosis 155
Clinical Features 155
Diagnosis 155
Treatment 155
Further Reading 157
Useful Web Sites for Patient Information 157
Erythrasma 157
Clinical Features 157
Differential Diagnosis 157
Diagnosis 158
Basic Management 158
Useful Web Site for Patient Information 158
Staphylococcal and Streptococcal Infections 158
Folliculitis 159
Pathophysiology 159
Clinical Features 159
Diagnosis 159
Basic Management 159
Further Reading 159
Bartholin Abscess 159
Clinical Features 160
Differential Diagnosis 160
Basic Management 160
Drainage of the Abscess 160
Marsupialization 160
Further Reading 160
Other Staphylococcal Infections 161
Toxic Shock Syndrome 161
Staphylococcal Scalded Skin Syndrome 161
Cellulitis 161
Clinical Features 161
Diagnosis 161
Differential Diagnosis 161
Other Streptococcal Infections 161
Further Reading 162
Vulvovaginal Candidiasis 162
Pathophysiology 162
Clinical Features 163
Differential Diagnosis 163
Diagnosis 164
Treatment 164
Further Reading 164
Useful Web Sites for Patient Information 164
Tinea Cruris 165
Pathophysiology 165
Clinical Features 165
Differential Diagnosis 165
Diagnosis 166
Basic Management 166
Further Reading 166
Useful Web Site for Patient Information 166
Viral Infections 166
Varicella Zoster (Shingles) 166
19 Vulval Intraepithelial Neoplasia 167
Introduction 167
Epidemiology 167
Aetiology 168
Prevention 168
Histological Features 168
Symptoms 169
Clinical Features 169
Management 173
Progression 174
Melanoma in situ 176
When to Refer 176
Practice Points 176
Further Reading 177
Useful Web Site for Patient Information 177
20 Extramammary Paget's Disease 179
Introduction 179
Epidemiology 179
Histological Features 179
Classification 180
Symptoms 181
Clinical Features 181
Management 183
Recurrences 185
Progression 185
Follow Up 186
When to Refer 186
Practice Points 186
Further Reading 186
Useful Web Site for Patient Information 187
21 Vulval Squamous Cell Carcinoma 189
Introduction 189
Epidemiology 189
Aetiology / Histology 189
Symptoms and Clinical Features 190
Management 190
Surgery 190
Complications 191
Radiotherapy 192
Follow Up 193
Prognosis 193
When to Refer 193
Practice Points 193
Further Reading 194
Useful Web Site for Patient Information 194
22 Other Vulval Cancers 195
Basal Cell Carcinoma 195
Epidemiology 195
Incidence 195
Pathophysiology 195
Histological Features 195
Symptoms 196
Clinical Features 196
Basic Management 196
When to Refer 196
Practice Points 197
Further Reading 197
Useful Web Site for Patient Information 197
Malignant Melanoma 197
Epidemiology 198
Pathophysiology 198
Histological Features 198
Symptoms 198
Clinical Features 198
Basic Management 198
Further Reading 199
Other Malignant Tumours and the Vulva 200
Further Reading 200
23 Vulvodynia 201
Definition 201
Epidemiology 201
Pathophysiology 203
Psychological Aspects of Vulvodynia 203
Histology 204
Symptoms 204
Signs 204
Diagnosis 205
Management 205
Local Topical Pain Modifiers 205
Systemic Pain Modifiers 205
Physical Therapy 206
Psychosexual Therapy 207
Surgical Treatment 207
Further Reading 207
Useful Web Sites for Patient Information 207
24 Psychosexual Aspects of Vulval Disease 209
Clinical Psychological Assessment 210
Further Reading 210
25 Benign Lesions 211
Epidermoid Cysts 211
Comedones 211
Syringomata 211
Hidradenoma Papilliferum 213
Lymphangioma / Lymphangiectasia 214
Further Reading 214
Index 215
