Practical Guide to Vulval Disease

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.

• Acknowledgements xvii1 The Normal Vulva 1Normal Vulval Anatomy 1Normal Vulval and Vaginal Flora 4Further Reading 4Normal Anatomical Variants 4Normal Changes Over the Lifetime 6Childhood 6Pregnancy 6Menopause 6Further Reading 72 Taking a History and Examination 9Taking a History 9General Medical History 9Gynaecological History 10Dermatological History 10Vulval History 10Examination of the Vulva 11Further Reading 133 How to Take a Vulval Biopsy and the Importance of Clinico‐Pathological Correlation 15Incisional Biopsy 16Fixation of the Biopsy 20Further Reading 214 Basic Histology of the Vulva 23Special Histological Stains 24Immunohistochemical Stains 26Other Tests 27Further Reading 285 Investigations in Vulval Disease 29Investigations for Infection 29Bacterial Swabs 29Viral Swabs 29Fungal Scrapings 29Wood’s Light Examination 32Serological Tests 32Investigations for Allergy 32Rast Tests 32Prick Tests 32Patch Tests (see Chapter 9) 32Investigations for Inflammatory Disease 33Direct Immunofluorescence 33Indirect Immunofluorescence 34Imaging Investigations 34Useful Web Sites for Patient Information 34Further Reading 346 Topical Treatment in Vulval Disease 35Introduction 35General Principles 35Lotions 35Gels 36Ointments 36Creams 36Pastes 36Topical Treatments Used for Vulval Disease 36Emollient 37Topical Steroids 38Adverse Effects 38Topical Steroids in Pregnancy 41Antiseptics 41Antibacterials 42Antifungals 42Barriers 42Others 42Podophyllotoxin 42Imiquimod 42Calcineurin Inhibitors 43Tar 44Emla ® 44Practice Points 44Further Reading 44Useful Web Sites for Patient Information 447 Symptoms in Vulval Disease 45Pruritis (Itch) 45Causes of Vulval Pruritis 46Soreness 46Pain 47Dyspareunia 48Discharge 48No Symptoms 49Useful Web Site for Patient Information 498 Signs in Vulval Disease 51Differential Diagnosis Based on Appearance 51Algorithms 51Further Reading 569 Eczema, Allergy and the Vulva 57Seborrhoeic Eczema 57Incidence 57Pathophysiology 57Symptoms 58Clinical Features 58Basic Management 58When to Refer 58Practice Points 59Further Reading 59Useful Web Sites for Patient Information 59Allergic Contact Eczema / Dermatitis 59Introduction 59Incidence 59Pathophysiology 60Patch Testing (see Chapter 5) 60Symptoms 60Clinical Features 60Basic Management 60Who to Refer for Patch Testing 60Practice Points 61Further Reading 61Useful Web Sites for Patient Information 62Irritant Eczema / Dermatitis 62Introduction 62Incidence 62Pathophysiology 63Histological Features 63Symptoms 63Clinical Features 63Basic Management 64When to Refer 64Practice Points 64Further Reading 64Urticaria 64Allergic Contact Urticaria 64Clinical Features 65Management 65References 6510 Psoriasis 67Introduction 67Incidence 67Pathophysiology 67Histological Features 67Symptoms 68Clinical Features 68Basic Management 69When to Refer 72Practice Points 73Further Reading 73Useful Web Sites for Patient Information 7311 Lichen Simplex 75Introduction 75Epidemiology 75Incidence 75Pathophysiology 75Histological Features 76Symptoms 76Clinical Features 76Basic Management 77When to Refer 78Practice Points 78Further Reading 79Useful Web Sites for Patient Information 7912 Lichen Sclerosus 81Introduction 81Incidence 81Pathophysiology 81Genetic 82Epigenetics 82Autoimmunity 82Hormonal Factors 82Infection 82Trauma 82Role of the Skin Immune System 82Histological Features 82Symptoms 83Clinical Features 83Associated Disease 90Risk of Malignancy 90Basic Management 92Follow Up 92When to Refer 92Practice Points 93Further Reading 93Useful Web Sites for Patient Information 9313 Lichen Planus 95Introduction 95Epidemiology 95Incidence 95Pathophysiology 95Histological Features 96Clinical Features of Lichen Planus 96Vulval Lichen Planus 98Classic LP 98Hypertrophic LP 98Erosive LP 98Risk of Malignancy 99Basic Management 100When to Refer 101Practice Points 102Further Reading 102Useful Web Sites for Patient Information 10214 Hidradenitis Suppurativa and Crohn’s Disease 103Hidradenitis Suppurativa 103Introduction 103Epidemiology 103Incidence 103Pathophysiology 103Histological Features 103Symptoms 104Clinical Features 104Basic Management 105Medical Management 105Surgery 105When to Refer 105Practice Points 106Further Reading 106Useful Web Sites for Patient Information 106Crohn’s Disease 106Introduction 106Epidemiology 106Pathophysiology 107Histological Features 107Symptoms 107Clinical Features 107Basic Management 109When to Refer 109Practice Points 109Further Reading 110Useful Web Sites for Patient Information 11015 Disorders of Pigmentation on the Vulva 111Introduction 111Postinflammatory Pigmentation 111Post‐Traumatic Pigmentation 111Acanthosis Nigricans 111Management 113Melanosis 114Pigmented Lesions 115History and Examination 115Other Techniques 116Lentigines 116Benign Naevi 116Atypical Genital Naevi 117Seborrhoeic Keratoses 117Pigmented Basal Cell Carcinoma 118Hypopigmentation 118Vitiligo 118When to Refer 119Practice Points 119Further Reading 119Useful Web Sites for Patient Information 12016 Other Dermatoses 121Genetic Disorders 121Hailey–Hailey Disease (Familial Benign Chronic Pemphigus) 121Management 121When to Refer 121Practice Points 121Further Reading 122Useful Web Site for Patient Information 122Auto‐Immune Bullous Disease 123Practice Points 126Further Reading 126Drug Eruptions 126Fixed Drug Eruption 126Common Drugs Causing a Fixed Drug Eruption 126Further Reading 126Useful Web Site for Patient Information 126Stevens–Johnson Syndrome 127Symptoms 127Clinical Features 127Toxic Epidermal Necrolysis 127Symptoms 127Clinical Features 127Basic Management 127Further Reading 128Useful Web Site for Patient Information 128Manifestations of Underlying Disease 128Necrolytic Migratory Erythema 128Symptoms 129Clinical Features 129Basic Management 129Further Reading 129Acrodermatitis Enteropathica 129Clinical Features 129Basic Management 129Practice Point 130Further Reading 130Inflammatory Ulcers 130Aphthous Ulcers 130Clinical Features 130Management 130Practice Point 130Behcet’s Syndrome 130Further Reading 132Useful Resources 132Lipschutz Ulceration 132Clinical Features 132Basic Management 133Further Reading 133Others 133Graft‐Versus‐Host Disease 133Further Reading 134Zoon’s Vulvitis (Plasma Cell Vulvitis) 134Histology 134Symptoms 134Clinical Features 135Basic Management 135Further Reading 135Vulvovaginal Adenosis 135Further Reading 135Langerhans Cell Histiocytosis 136Further Reading 13617 Vulval Infection – Sexually Transmitted 137Normal Flora 137Trichomoniasis 137Pathophysiology 137Clinical Features 138Diagnosis 138Basic Management 138Further Reading 138Useful Web Sites for Patient Information 138Chlamydia 139Clinical Features 139Diagnosis 139Treatment 139Further Reading 139Useful Web Site for Patient Information 139Lymphogranuloma Venereum 139Clinical Features 140Diagnosis 140Treatment 140Further Reading 140Useful Web Site for Patient Information 140Gonorrhoea 140Clinical Features 140Diagnosis 140Treatment 141Further Reading 141Useful Web Sites for Patient Information 141Syphilis 141Pathophysiology 141Clinical Features 141Treatment 142Further Reading 142Useful Web Site for Patient Information 142Chancroid 142Pathophysiology 142Clinical Features 143Treatment 143Further Reading 143Useful Web Sites for Patient Information 143Donovanosis (Granuloma Inguinale) 143Clinical Features 143Diagnosis 143Treatment 143Further Reading 144Useful Web Site for Patient Information 144Herpes Simplex Infection 144Incidence 144Pathophysiology 144Clinical Features 144Differential Diagnosis 145Diagnosis 146Basic Management 146Hsv Infection and Pregnancy 146Further Reading 147Useful Web Sites for Patient Information 147Human Papillomavirus Infection 147Epidemiology 147Pathophysiology 148The Concept of Vulval Subclinical Lesion 148Histology 148Clinical Features 149Diagnosis 149Treatment 150Hpv Vaccine 150Genital Warts in Children 150Further Reading 150Useful Web Sites for Patient Information 150Molluscum Contagiosum 150Symptoms 151Clinical Appearance 151Diagnosis 151Treatment 151Further Reading 152Useful Web Site for Patient Information 152Scabies 152Clinical Features 152Diagnosis 152Treatment 153Further Reading 153Useful Web Site for Patient Information 153Pubic Lice 153Clinical Features 153Treatment 153Useful Web Site for Patient Information 15318 Vulval Infection – Nonsexually Transmitted 155Bacterial Infections 155Bacterial Vaginosis 155Clinical Features 155Diagnosis 155Treatment 155Further Reading 157Useful Web Sites for Patient Information 157Erythrasma 157Clinical Features 157Differential Diagnosis 157Diagnosis 158Basic Management 158Useful Web Site for Patient Information 158Staphylococcal and Streptococcal Infections 158Folliculitis 159Pathophysiology 159Clinical Features 159Diagnosis 159Basic Management 159Further Reading 159Bartholin Abscess 159Clinical Features 160Differential Diagnosis 160Basic Management 160Drainage of the Abscess 160Marsupialization 160Further Reading 160Other Staphylococcal Infections 161Toxic Shock Syndrome 161Staphylococcal Scalded Skin Syndrome 161Cellulitis 161Clinical Features 161Diagnosis 161Differential Diagnosis 161Other Streptococcal Infections 161Further Reading 162Vulvovaginal Candidiasis 162Pathophysiology 162Clinical Features 163Differential Diagnosis 163Diagnosis 164Treatment 164Further Reading 164Useful Web Sites for Patient Information 164Tinea Cruris 165Pathophysiology 165Clinical Features 165Differential Diagnosis 165Diagnosis 166Basic Management 166Further Reading 166Useful Web Site for Patient Information 166Viral Infections 166Varicella Zoster (Shingles) 16619 Vulval Intraepithelial Neoplasia 167Introduction 167Epidemiology 167Aetiology 168Prevention 168Histological Features 168Symptoms 169Clinical Features 169Management 173Progression 174Melanoma in situ 176When to Refer 176Practice Points 176Further Reading 177Useful Web Site for Patient Information 17720 Extramammary Paget’s Disease 179Introduction 179Epidemiology 179Histological Features 179Classification 180Symptoms 181Clinical Features 181Management 183Recurrences 185Progression 185Follow Up 186When to Refer 186Practice Points 186Further Reading 186Useful Web Site for Patient Information 18721 Vulval Squamous Cell Carcinoma 189Introduction 189Epidemiology 189Aetiology / Histology 189Symptoms and Clinical Features 190Management 190Surgery 190Complications 191Radiotherapy 192Follow Up 193Prognosis 193When to Refer 193Practice Points 193Further Reading 194Useful Web Site for Patient Information 19422 Other Vulval Cancers 195Basal Cell Carcinoma 195Epidemiology 195Incidence 195Pathophysiology 195Histological Features 195Symptoms 196Clinical Features 196Basic Management 196When to Refer 196Practice Points 197Further Reading 197Useful Web Site for Patient Information 197Malignant Melanoma 197Epidemiology 198Pathophysiology 198Histological Features 198Symptoms 198Clinical Features 198Basic Management 198Further Reading 199Other Malignant Tumours and the Vulva 200Further Reading 20023 Vulvodynia 201Definition 201Epidemiology 201Pathophysiology 203Psychological Aspects of Vulvodynia 203Histology 204Symptoms 204Signs 204Diagnosis 205Management 205Local Topical Pain Modifiers 205Systemic Pain Modifiers 205Physical Therapy 206Psychosexual Therapy 207Surgical Treatment 207Further Reading 207Useful Web Sites for Patient Information 20724 Psychosexual Aspects of Vulval Disease 209Clinical Psychological Assessment 210Further Reading 21025 Benign Lesions 211Epidermoid Cysts 211Comedones 211Syringomata 211Hidradenoma Papilliferum 213Lymphangioma / Lymphangiectasia 214Further Reading 214Index 215