Ridley's The Vulva

1 Cover

2 Title Page Ridley’s The Vulva Edited by Fiona M. Lewis, MD, FRCP Consultant Dermatologist St John’s Institute of Dermatology Guy’s & St Thomas’ NHS Foundation Trust Guy’s Hospital London UK Fourth Edition

3 Copyright Page

4 Dedication

5 List of Contributors

6 Preface

7 Acknowledgements

8 About the Companion Website

9 Section 1: The Basics of Vulval Disease 1 Vulval Embryology and Developmental Abnormalities Vulval embryology Disorders of sexual development Structural developmental defects External genitalia Vulval and urinary system abnormalities Vulval and intestinal abnormalities Patient Support Groups References 2 The Normal Vulva Normal vulval anatomy Associated structures Blood supply of the vulva Lymphatic drainage of the vulva Nerve supply of the vulva Normal microscopic anatomy and histological features References 3 Vulval Physiology and Changes Through Life Barrier function Immune responsiveness Changes through life Resources Patient support groups References 4 Normal Microbiology of the Vulva The vulval microbiome The vaginal microbiome References 5 The Vulval Clinic, History, and Examination The vulval clinic The consultation Helpful websites for patient information Dermatoses Sexually transmitted infections References 6 Symptoms and Signs in Vulval Disease Symptoms in vulval disease Signs in vulval disease References 7 Investigations in Vulval Disease Biopsy Cytology Microbiological investigation Wood’s lamp examination Patch testing Prick testing Blood tests Imaging Dermoscopy Vulvoscopy References 8 Topical and Non‐Surgical Treatments Topical treatments General vulval hygiene Types of preparation Non‐surgical treatments Resources References

10 Section 2: Infection and the Vulva 9 Bacterial Vaginosis Pathophysiology Clinical features Diagnosis Differential diagnosis Complications Treatment Prognosis and follow‐up Resources References 10 Vulvovaginal Candidiasis Epidemiology Pathophysiology Clinical features Diagnosis Differential diagnosis Complications Associated disease Treatment Prognosis and follow‐up Resources References 11 Syphilis Epidemiology Pathophysiology Histological features Clinical features Differential diagnosis Diagnosis Complications Treatment Prognosis and follow‐up Resources References 12 Gonorrhoea Epidemiology Clinical features Diagnosis Differential diagnosis Complications Treatment Prognosis and follow‐up Resources References 13 Chlamydial Infection Genital chlamydia Resources Lymphogranuloma venereum Resources References 14 Trichomoniasis Epidemiology Clinical features Diagnosis Complications Treatment Prognosis and follow‐up Resources Guidelines References 15 Genital Herpes Epidemiology Pathophysiology Clinical features Diagnosis Complications Treatment Resources References 16 Human Papillomavirus Infections Epidemiology Pathophysiology Clinical features Diagnosis Treatment Human papilloma virus vaccines Prognosis and follow‐up Resources References 17 Other Sexually Transmitted Infections Mycoplasma genitalium Resources Chancroid Resources Donovanosis Molluscum contagiosum Scabies Pubic lice References 18 Tropical Infections Introduction Infection with nematodes, tapeworms, and flukes Infection with protozoa Infection with fungi Mycobacterial infections Infection with viruses Other infections References 19 Non‐Sexually Transmitted Infections Introduction Infection with nematodes Infection with fungi Infection with bacteria Infection with viruses Resources Other infections Acute genital ulcers (Lipschutz ulcers) References 20 HIV Infection and Vulval Disease HIV and other infections HIV and human papillomavirus (HPV)‐related disease Immune reconstitution inflammatory syndrome (IRIS) References

11 Section 3: Inflammatory Dermatoses 21 Vulval Psoriasis Epidemiology Genetics Pathophysiology Histological features Clinical features Differential diagnosis Complications Associated disease Treatment Prognosis and follow‐up Resources References 22 Eczema and Allergic Reactions Endogenous eczema Resources Exogenous eczema Resources References 23 Lichen Sclerosus Historical aspects Epidemiology Genetics Pathophysiology Histological features Clinical features Impact on quality of life Grading systems Extra‐genital lesions Dermoscopy Differential diagnosis Complications Associated disease Treatment Management in special situation Prognosis and follow‐up Resources References 24 Lichen Planus Epidemiology Genetics Pathophysiology Histological features Clinical features Investigations Differential diagnosis Complications Associated disease Treatment Prognosis and follow‐up Resources References 25 Hidradenitis Suppurativa Epidemiology Genetics Pathophysiology Histological features Clinical features Staging systems Differential diagnosis Associated disease Complications Current therapeutic options for HS Surgical management Prognosis Resources – Support groups and patient information References 26 Immunobullous Disease Diagnosis Differential diagnosis of the immunobullous disorders Principles of treatment References 27 Other Inflammatory Dermatoses Nodular prurigo Intertrigo Autoimmune progesterone dermatitis Malacoplakia Reiter’s syndrome (reactive arthritis) Vulval aphthous ulcers Behcet’s syndrome Resources Magic syndrome Febrile ulcero‐necrotic Mucha‐Habermann’s disease (Pityriasis lichenoides et varioliformis acuta fulminans) Angiolymphoid hyperplasia with eosinophilia Amyloidosis Genital papular acantholytic dyskeratosis Acquired dyskeratotic leukoplakia Vasculitis Desquamative inflammatory vaginitis Plasma cell vulvitis (Zoon’s vulvitis) Vulvovaginal adenosis References

12 Section 4: Other Dermatological Conditions 28 Genetic Conditions Epidermolysis bullosa (EB) Darier’s disease (keratosis follicularis) Hailey‐Hailey disease (benign familial chronic pemphigus) Resources Goltz syndrome (focal dermal hypoplasia) Ehlers–Danlos syndrome Resources Pseudoxanthoma elasticum Congenital familial lipodystrophy Neurofibromatosis Ligneous disease Anderson‐Fabry disease White sponge naevus Cowden’s disease Netherton’s syndrome (ichthyosis linearis circumflexa) Incontinentia pigmenti References 29 Disorders of Pigmentation Hyperpigmentation Hypopigmentation Resources References 30 Disorders of Skin Appendages and Hair Disorders of sebaceous glands Disorders of sweat glands Disorders of hair References 31 Drug Reactions and the Vulva Fixed drug eruption Stevens‐Johnson syndrome/toxic epidermal necrolysis (SJS/TEN syndrome) Acute generalised exanthematous pustulosis (AGEP) Resources and patient information References 32 Conditions Associated with Systemic Disease Vulval involvement in connective tissue disease Resources Vulval disorders associated with metabolic disturbances Vulval disorders associated with inflammatory bowel disease Resources Vulval disorders associated with neutrophilic disorders Vulval disorders associated with granulomatous disease Vulval disorders associated with haematological disorders References 33 Lymphatic Abnormalities and the Vulva Epidemiology Pathophysiology Histological features Clinical features Differential diagnosis Complications Treatment Prognosis and follow‐up Resources References 34 Vascular Abnormalities and the Vulva Vascular malformations Vulval varicose veins References

13 Section 5: Vulvodynia 35 Vulvodynia – History, Classification, and Terminology History Classification References 36 Vulvodynia – Pathophysiology and Clinical Features The physiology of acute and persistent pain Epidemiology of vulvodynia Pain mechanisms in vulvodynia Aetiology of vulvodynia Clinical features Examination and differential diagnosis Resources References 37 Vulvodynia – Management Provoked vulvodynia (PVD) Generalised unprovoked vulvodynia References

14 Section 6: Benign Tumours of the Vulva 38 Benign Vulval Tumours Benign cysts Benign tumours of the epithelium Benign tumours of the anogenital mammary‐type glands Benign lesions of the sweat glands Benign tumours of the hair follicle and pilosebaceous duct Benign tumours of smooth muscle Benign tumours of striated muscle Benign lesions of connective tissue Myofibroblastic lesions Benign lesions of fat Benign lesions of the lymphatic system Benign lesions of perivascular cells Benign vascular lesions Benign neural lesions Miscellaneous benign proliferations and neoplasms Benign lesions of the Bartholin’s gland Vulval hamartomas References 39 Benign Vulval Naevi Benign melanocytic naevi Atypical genital naevi (AGN) Blue naevi Bulky naevocytoma of the perineum Inflammatory linear verrucous epidermal naevus References