Table of Contents

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

About the Author

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.