A Practical Guide to Vulval Disease E-Book

Fiona M. Lewis

• Herausgeber: John Wiley & Sons
• Kategorie: Fachliteratur
• Sprache: Englisch

Seitenzahl: 287

Veröffentlichungsjahr: 2017

Beschreibung

A PRACTICAL GUIDE TO VULVAL DISEASE DIAGNOSIS AND MANAGEMENT

A PRACTICAL GUIDE TO VULVAL DISEASE DIAGNOSIS AND MANAGEMENT

Patients with vulval disease frequently experience delays in diagnosis due to a lack of training for physicians. A Practical Guide to Vulval Disease: Diagnosis and Management offers practical, up-to-date and expert guidance on the diagnosis and management of vulval disorders. It provides the knowledge required for diagnosis and treatment of these conditions at both trainee and specialist level. Key information about diagnosis, investigation and basic management is included, with a section on signs and symptoms to direct the reader to the appropriate chapter for the particular disease. Current classification and terminology of vulval disease is featured, along with guidance on when a patient should be referred to a specialist.

Well illustrated, with 185 high quality photographs, this user-friendly clinical guidebook integrates clinical and histological features of vulval disorders, so the reader can understand the disease from a microscopic to macroscopic level.

Written by an experienced author team, A Practical Guide to Vulval Disease: Diagnosis and Management is essential reading for gynaecologists, dermatologists, genito-urinary physicians, general practitioners and nurses, both in practice and in training.

Leseprobe

Table of Contents

Cover

Title Page

Acknowledgements

1 The Normal Vulva

Normal vulval anatomy

Normal vulval and vaginal flora

Further reading

Normal anatomical variants

Normal changes over the lifetime

Further reading

2 Taking a History and Examination

Taking a history

Examination of the vulva

Further reading

3 How to Take a Vulval Biopsy and the Importance of Clinico‐Pathological Correlation

Incisional biopsy

Fixation of the biopsy

Further reading

4 Basic Histology of the Vulva

Special histological stains

Immunohistochemical stains

Other tests

Further reading

5 Investigations in Vulval Disease

Investigations for infection

Investigations for allergy

Investigations for inflammatory disease

Imaging investigations

Useful web sites for patient information

Further reading

6 Topical Treatment in Vulval Disease

Introduction

General principles

Topical treatments used for vulval disease

Practice points

Further reading

Useful web sites for patient information

7 Symptoms in Vulval Disease

Pruritis (Itch)

Causes of vulval pruritis

Soreness

Pain

Dyspareunia

Discharge

No symptoms

Useful web site for patient information

8 Signs in Vulval Disease

Differential Diagnosis Based on Appearance

Algorithms

Further reading

9 Eczema, Allergy and the Vulva

Seborrhoeic eczema

Allergic Contact Eczema / Dermatitis

Clinical features

Irritant Eczema / Dermatitis

Urticaria

10 Psoriasis

Introduction

Incidence

Clinical features

Basic management

When to refer

Practice points

Further reading

Useful Web Sites for Patient Information

11 Lichen Simplex

Introduction

Epidemiology

Incidence

Pathophysiology

Histological features

Symptoms

Clinical features

Basic management

When to refer

Practice points

Further reading

Useful Web Sites for Patient Information

12 Lichen Sclerosus

Introduction

Incidence

Pathophysiology

Histological features

Symptoms

Clinical features

Associated disease

Risk of malignancy

Basic Management

Follow Up

When to Refer

Practice points

Further reading

Useful Web Sites for Patient Information

13 Lichen Planus

Introduction

Epidemiology

Incidence

Pathophysiology

Histological Features

Clinical Features of Lichen Planus

Vulval Lichen Planus

Risk of Malignancy

Basic management

When to refer

Practice points

Further reading

Useful Web Sites for Patient Information

14 Hidradenitis Suppurativa and Crohn's Disease

Hidradenitis Suppurativa

Crohn’s disease

15 Disorders of Pigmentation on the Vulva

Introduction

Postinflammatory Pigmentation

Post‐Traumatic Pigmentation

Acanthosis Nigricans

Melanosis

Pigmented lesions

Lentigines

Benign naevi

Atypical Genital Naevi

Seborrhoeic Keratoses

Pigmented Basal Cell Carcinoma (see Chapter 22)

Hypopigmentation

Vitiligo

When to Refer

Practice points

Further reading

Useful Web Sites for Patient Information

16 Other Dermatoses

Genetic disorders

Auto‐Immune Bullous Disease

Drug Eruptions

Manifestations of Underlying Disease

Inflammatory ulcers

Others

17 Vulval Infection – Sexually Transmitted

Normal flora

Trichomoniasis

Chlamydia

Lymphogranuloma Venereum

Gonorrhoea

Syphilis

Chancroid

Donovanosis (Granuloma Inguinale)

Herpes Simplex Infection

Human Papillomavirus Infection

Molluscum contagiosum

Scabies

Pubic lice

18 Vulval Infection – Nonsexually Transmitted

Bacterial Infections

Bacterial Vaginosis

Erythrasma

Staphylococcal and Streptococcal Infections

Folliculitis

Bartholin abscess

Other Staphylococcal Infections

Cellulitis

Other Streptococcal Infections

Vulvovaginal Candidiasis

Tinea cruris

Viral infections

19 Vulval Intraepithelial Neoplasia

Introduction

Epidemiology

Aetiology

Prevention

Histological features

Symptoms

Clinical features

Management

Progression

Melanoma in situ

When to refer

Practice points

Further reading

Useful Web Site for Patient Information

20 Extramammary Paget’s Disease

Introduction

Epidemiology

Histological features

Classification

Symptoms

Clinical features

Management

Recurrences

Progression

Follow up

When to refer

Practice points

Further reading

Useful Web Site for Patient Information

21 Vulval Squamous Cell Carcinoma

Introduction

Epidemiology

Aetiology / histology

Symptoms and Clinical Features

Management

Surgery

Complications

Radiotherapy

Follow up

Prognosis

When to refer

Practice points

Further reading

Useful Web Site for Patient Information

22 Other Vulval Cancers

Basal Cell Carcinoma

Malignant melanoma

Other Malignant Tumours and the Vulva

23 Vulvodynia

Definition

Epidemiology

Pathophysiology

Psychological Aspects of Vulvodynia

Histology

Symptoms

Signs

Diagnosis

Management

Further reading

Useful Web Sites for Patient Information

24 Psychosexual Aspects of Vulval Disease

Clinical Psychological Assessment

Further reading

25 Benign Lesions

Epidermoid cysts

Comedones

Syringomata

Hidradenoma Papilliferum

Lymphangioma / Lymphangiectasia

Further reading

Index

End User License Agreement

List of Tables

Chapter 04

Table 4.1 Glossary of cell types and structures.

Table 4.2 Glossary of histopathological terms.

Table 4.3 Special histological stains.

Table 4.4 Immunohistochemical stains.

Chapter 06

Table 6.1 Classification of topical treatment.

Table 6.2 Grading of topical steroids (European).

Chapter 07

Table 7.1 Causes of vulval pruritis.

Table 7.2 Causes of vulval soreness.

Table 7.3 Causes of vulval pain.

Table 7.4 Causes of dyspareunia.

Table 7.5 Causes of discharge.

Table 7.6 Causes of change in appearance without symptoms.

Chapter 08

Table 8.1 Terminology of vulval lesions.

Table 8.2 Describing vulval lesions.

Table 8.3 Common presentations of vulval disorders.

Chapter 09

Table 9.1 Common causes of contact dermatitis on the vulva.

Table 9.2 Common causes of irritant eczema/dermatitis on the vulva.

Chapter 13

Table 13.1 Other sites that can be affected by erosive LP.

Chapter 14

Table 14.1 Hurley staging system for hidradenitis supparativa.

Chapter 15

Table 15.1 Causes of diffuse pigmentation.

Table 15.2 Types of acanthosis nigricans.

Table 15.3 Pigmented lesions on the vulva.

Chapter 16

Table 16.1 Other dermatoses.

Table 16.2 Clinical features and immunofluorescence patterns in auto‐immune bullous disease.

Table 16.3 Diagnostic criteria for Behcet’s syndrome.

Chapter 17

Table 17.1 Major sexually transmitted infections.

Chapter 18

Table 18.1 Nonsexually transmitted vulval infections.

Table 18.2 Staphylococcal and streptococcal infections.

Chapter 19

Table 19.1 Squamous vulval intraepithelial neoplasia (VIN) terminology.

Chapter 20

Table 20.1 Immunocytochemistry in EMPD.

Chapter 23

Table 23.1 Treatments used.

List of Illustrations

Chapter 01

Figure 1.1 The vulva.

Figure 1.2 Normal vulva (a) – outer and (b) – inner vulva.

Figure 1.3 The clitoris.

Figure 1.4 Hart’s line.

Figure 1.5 Bifid labium minus.

Figure 1.6 Fordyce spots.

Figure 1.7 Vestibular papillomatosis.

Figure 1.8 Angiokeratomata.

Figure 1.9 Vulval varicosities.

Chapter 02

Figure 2.1 A lamp with good light and magnification is vital.

Figure 2.2 Plastic speculum.

Chapter 03

Figure 3.1 The area to be biopsied is cleaned with antiseptic.

Figure 3.2 Local anaesthetic is infiltrated.

Figure 3.3 Punch biopsy.

Figure 3.4 The punch biopsy is inserted and rotated.

Figure 3.5 A core of tissue is obtained and cut off at the base.

Figure 3.6 Cold knife biopsy – ellipse is cut.

Figure 3.7 Cold knife biopsy – the ellipse is then excised to a depth of at least 5 mm.

Figure 3.8 The surgical wound that results.

Figure 3.9 Haemostasis is achieved by suturing.

Figure 3.10 The final appearance.

Figure 3.11 The tissue is then immediately put into fixative.

Chapter 04

Figure 4.1 Histopathological features of the normal vulva.

Chapter 05

Figure 5.1 Bacterial swab.

Figure 5.2 (a) Viral swab and transport medium. (b) The swab is taken and then the tip is snapped off. (c) The tip of the swab is left in the transport medium.

Figure 5.3 ‘Banana’ scalpel used to obtain skin scrapings.

Figure 5.4 The scaly edge of the lesion is gently scraped on to paper.

Figure 5.5 Dark paper used to collect skin scrapings.

Figure 5.6 Allergens at suitable concentration are placed on Finn chambers.

Figure 5.7 Finn chambers taped on back and marked.

Figure 5.8 Positive patch test reaction – eczematous papule at site of application.

Chapter 06

Figure 6.1 Cream, ointment and gel, illustrating differences between the preparations.

Figure 6.2 Emulsifying ointment used as a soap substitute.

Figure 6.3 In warm water, the emulsifying ointment is made into an emulsion to wash in.

Figure 6.4 Striae on the thighs after excessive use of a potent topical steroid.

Figure 6.5 Telangiectasia on outer labia majora at incorrect site of application of topical steroids for lichen sclerosus.

Figure 6.6 Tinea incognito – papules and folliculitic lesions after the application of a topical steroid on a fungal infection.

Figure 6.7 Steroid foam preparation that can be inserted into the vagina to treat inflammatory disease.

Figure 6.8 Potassium permanganate – dissolve a tablet in warm water to produce a pale purple liquid.

Figure 6.9 Gauze is soaked in the solution and this can be applied to the vulva for 10 minutes.

Figure 6.10 Barrier preparation applied to the skin showing the resistance to water.

Figure 6.11 Punctate erosions seen with the use of imiquimod.

Chapter 08

Figure 8.1 (a) Diagnostic algorithm for vulval ulceration. (b) Diagnostic algorithm for vulval oedema.

Chapter 09

Figure 9.1 Chronic eczema showing inflammation and dryness.

Figure 9.2 Acute allergic contact dermatitis.

Figure 9.3 Allergic contact dermatitis with extension to perianal area and thighs.

Figure 9.4 Erosions – allergic contact dermatitis to depilatory cream.

Figure 9.5 Irritant dermatitis secondary to urinary incontinence.

Chapter 10

Figure 10.1 Typical psoriatic plaque on elbow showing silver scales.

Figure 10.2 Psoriasis affecting scalp, hairline and ears.

Figure 10.3 Onycholysis and pitting of the nails

Figure 10.4 Vulval psoriasis – confluent plaques with well defined edge.

Figure 10.5 Scaling on lesions on mons pubis.

Figure 10.6 Umbilical psoriasis.

Figure 10.7 Psoriasis with excoriation and scaling.

Figure 10.8 Psoriasis affecting the labia majora, mons pubis and extending into inguinal folds.

Figure 10.9 Fissuring in inguinal folds.

Chapter 11

Figure 11.1 Gross thickening of skin on inner and outer surfaces of the labia majora.

Figure 11.2 Lichenification of labia majora and minora.

Figure 11.3 Lichenification of labia majora with accentuation of the skin markings and rugose appearance.

Figure 11.4 Perianal lichen simplex.

Chapter 12

Figure 12.1 Histology of LS.

Figure 12.2 Atrophic extragenital LS.

Figure 12.3 Sclerotic extragenital LS.

Figure 12.4 Ecchymosis in extragenital LS.

Figure 12.5 Symmetrical white plaques on labia majora.

Figure 12.6 Perianal LS.

Figure 12.7 Ecchymosis (purpura) can be widespread in some patients.

Figure 12.8 Sclerotic plaques on labia minora.

Figure 12.9 Postinflammatory hyperpigmentation in LS.

Figure 12.10 Hyperpigmentation in LS.

Figure 12.11 Pre‐pubertal LS.

Figure 12.12 Fusion of labia minora reducing introitus.

Figure 12.13 Sealing clitoral hood and burial of clitoris.

Figure 12.14 Lichen sclerosus on prolapsed vaginal mucosa.

Figure 12.15 Acanthotic disease.

Figure 12.16 Lichen sclerosus and psoriasis can coexist.

Figure 12.17 Squamous cell carcinoma developing on LS.

Chapter 13

Figure 13.1 Typical histological features of LP.

Figure 13.2 Plaque of lichen planus with Wickham’s striae on the surface.

Figure 13.3 Postinflammatory hyperpigmentation in inguinal fold.

Figure 13.4 Lichen planus affecting gingival margins.

Figure 13.5 Classic type of vulval LP with Wickham’s striae.

Figure 13.6 Hypertrophic LP affecting labia majora.

Figure 13.7 Erosive LP.

Figure 13.8 Scarring in erosive LP.

Figure 13.9 Scarring around lacrimal duct in lichen planus.

Figure 13.10 Squamous cell carcinoma in LP – eroded tumour left labium majus.

Chapter 14

Figure 14.1 Hidradenitis suppurativa – vulval involvement.

Figure 14.2 Bridged comedones.

Figure 14.3 Oedema in ano‐genital Crohn’s disease may be the only feature.

Figure 14.4 Lymphangiectasia are a frequent feature.

Figure 14.5 ‘Knife‐cut’ fissures.

Figure 14.6 Fissuring in gluteal cleft.

Figure 14.7 Fissuring in abdominal scar.

Chapter 15

Figure 15.1 Linea nigra.

Figure 15.2 Postinflammatory hyperpigmentation after eczema.

Figure 15.3 Acanthosis nigricans in axilla.

Figure 15.4 Thickened velvety surface seen in acanthosis nigricans.

Figure 15.5 Melanosis –irregular pigmentation can be seen in the vestibule.

Figure 15.6 Melanosis showing very dark pigmentation.

Figure 15.7 Histological features of melanosis – increased melanin and some pigment in the dermis.

Figure 15.8 Benign mucosal naevus.

Figure 15.9 Benign compound naevus.

Figure 15.10 Atypical genital naevus.

Figure 15.11 Seborrhoeic keratosis.

Figure 15.12 Vitiligo (patient also has lichen sclerosus).

Chapter 16

Figure 16.1 Hailey‐Hailey disease – pruritic papules on labium majus.

Figure 16.2 Bullous pemphigoid – tense bullae.

Figure 16.3 Bullous pemphigoid: direct immunofluorescence shows a strong linear deposition of IgG (shown) and C3 both along the basement membrane zone and the roof of the subepidermal blister

Figure 16.4 Pemphigus: Direct immunofluorescence shows a positive intraepidermal intercellular deposition of IgG (shown) and C3. Please notice the intraepidermal acantholytic blister

Figure 16.5 Mucous membrane pemphigoid – scarring and erosions.

Figure 16.6 Mucous membrane pemphigoid – desquamative gingivitis.

Figure 16.7 Pemphigus vulgaris of the vulva.

Figure 16.8 Stevens‐Johnson syndrome – vulval involvement.

Figure 16.9 Toxic epidermal necrolysis – full thickness epidermal loss.

Figure 16.10 Healing aphthous ulcer lower right labium majus.

Figure 16.11 Behcet’s disease – deeper scarring ulcers.

Figure 16.12 Lipschutz ulcer.

Figure 16.13 Genital graft‐versus‐host disease – appearances may be indistinguishable from erosive lichen planus.

Figure 16.14 Zoon’s vulvitis.

Figure 16.15 Langerhans cell histiocytosis of the vulva.

Chapter 17

Figure 17.1 Lymphogranuloma venereum: sinuses.

Figure 17.2 Primary chancre of syphilis on right inner labium majus.

Figure 17.3 Herpes simplex infection – multiple painful vesicles.

Figure 17.4 Confluent vesicles in HSV.

Figure 17.5 Hypertrophic HSV infection in immunosuppressed.

Figure 17.6 Warts – papillomatous lesions.

Figure 17.7 Warts – confluent plaques.

Figure 17.8 Molluscum contagiosum – umbilicated papules.

Figure 17.9 Scabies – crusted lesions in the finger spaces in severe infection.

Chapter 18

Figure 18.1 Erythrasma.

Figure 18.2 Scaly edge to the plaques.

Figure 18.3 Folliculitis.

Figure 18.4 Bartholin’s abscess.

Figure 18.5 Staphylococcal infection (

S. aureus

) with superficial desquamation.

Figure 18.6 Vulval cellulitis with erythema and oedema involving the right labium majus and extending to mons pubis.

Figure 18.7 Acute candidiasis.

Figure 18.8 Satellite lesions spreading to thighs.

Figure 18.9 Tinea cruris – extending lesions with central sparing.

Figure 18.10 Extensive infection caused by inappropriate use of topical steroids (tinea incognito).

Chapter 19

Figure 19.1 Undifferentiated VIN with, on the left, the warty type with the condylomatous appearance and the basaloid type with large numbers of relative uniform undifferentiated cells, on the right, often coexisting in one lesion.

Figure 19.2 Differentiated VIN with atypical mitosis in the basal layer, basal cellular atypia, dyskeratosis, prominent nucleoli and elongation and anastomosis of the rete ridges.

Figure 19.3 Typical multifocal uVIN with white, red and pigmented lesions.

Figure 19.4 Extensive multifocal uVIN with perianal involvement.

Figure 19.5 Undifferentiated VIN with localized and small lesions.

Figure 19.6 Multifocal uVIN lesions forming confluent plaques.

Figure 19.7 Undifferentiated VIN with only pigmented papules, also in the perianal region.

Figure 19.8 Differentiated VIN presenting with multifocal erosive lesions in the fourchette on the background of LS.

Figure 19.9 (a) Undifferentiated VIN and (b) after treatment with imiquimod.

Figure 19.10 (a) Vulval SCC. (b) Labium majus, which developed in a region with dVIN on the background of LS.

Figure 19.11 Multifocal uVIN with progression to vulval SCC on the perineum.

Figure 19.12 Vulval melanoma

in situ.

Chapter 20

Figure 20.1 Clusters of Paget’s cells with abundant clear cytoplasm and round nuclei in the basal layer.

Figure 20.2 Extramammary Paget’s disease affecting the right labium majus.

Figure 20.3 Extramammary Paget’s disease with extensive sharply demarcated erythema with areas of hyperkeratosis, erosions and moist skin.

Figure 20.4 Extension of EMPD to the perianal area.

Figure 20.5 (a) Wide local excision for primary vulval EMPD and a recurrence in the same patient – primary vulval EMPD (courtesy of J. J. Hage, MD, PhD, plastic surgeon). (b) After the wide local excision. (c) Defect closed by fasciocutaneous infragluteal propeller flap. (d) Recurrence of vulval EMPD on the left side after 14 months’ follow up. (e) Recurrence excised by wide local excision. (f) Defect closed by pedicled pudendal thigh island flap. (g) After 3 months’ follow up.

Chapter 21

Figure 21.1 Verrucous squamous cell carcinoma.

Figure 21.2 Plastic reconstruction is often necessary to prevent wound complications. Psychosexual sequalae might still be significant (courtesy of J. J. Hage, MD, PhD, plastic surgeon).

Chapter 22

Figure 22.1 Typical appearances of BCC – pearly papule.

Figure 22.2 Vulval BCC – small nodule on outer labium majus.

Figure 22.3 Melanoma right labium minus.

Figure 22.4 Extensive nodular malignant melanoma.

Figure 22.5 Variation in colour with amelanotic areas.

Chapter 23

Figure 23.1 Subsets of vulvodynia.

Chapter 25

Figure 25.1 Multiple epidermoid cysts.

Figure 25.2 Large epidermoid cyst on left labium majus in a patient with lichen planus.

Figure 25.3 Open comedone.

Figure 25.4 Hidradenoma papilliferum.

Figure 25.5 Lymphangiectasia in Crohn’s disease.

Guide

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A Practical Guide to Vulval Disease

Diagnosis and Management

Fiona Lewis

Wexham Park Hospital

Wexham Street

Slough, UK

St John’s Institute of Dermatology

Guy’s & St Thomas’ Hospital

London, UK

Fabrizio Bogliatto

Chivasso Civic Hospital Turin

Turin, Italy

Marc van Beurden

Netherlands Cancer Institute

Amsterdam, Netherlands

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Library of Congress Cataloging‐in‐Publication Data

Names: Lewis, Fiona, 1963– author. | Bogliatto, Fabrizio, author. | Beurden, Marc van, author.Title: Practical guide to vulval disease : diagnosis and management / Fiona Lewis, Fabrizio Bogliatto, Marc van Beurden.Description: Chichester, West Sussex ; Hoboken, NJ : John Wiley & Sons, Inc., 2017. | Includes bibliographical references and index.Identifiers: LCCN 2016040293 (print) | LCCN 2016040776 (ebook) | ISBN 9781119146056 (cloth) | ISBN 9781119146063 (pdf) | ISBN 9781119146070 (epub)Subjects: | MESH: Vulvar Diseases | Vulva–pathologyClassification: LCC RG261 (print) | LCC RG261 (ebook) | NLM WP 200 | DDC 618.1/6–dc23LC record available at https://lccn.loc.gov/2016040293

A catalogue record for this book is available from the British Library.

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Cover image: Pazhyna/Gettyimages

Acknowledgements

We would like to thank all those who have helped us with the preparation of this book – particularly our trainees who inspired us to write it and our colleagues for their encouragement.

Our thanks to Dr Catherine Stefanato for supplying the immunofluorescence figures, Figures 16.3 and 16.4, and to Dr Eduardo Calonje, Marie‐Laure Jullie and Kristina Semkova for help with the photography of histology slides. We are also indebted to Dr Joris Hage for providing the clinical photographs in Figures 20.5a and 21.2.

We are grateful to our patients who have allowed us to use their clinical photographs and to our medical photography colleagues for their patience, advice and technical skills.

1The Normal Vulva

The vulva is a complex organ, due to its embryologic derivation from the three germ layers belonging to the embryonic disc:

ectoderm (squamous epithelium);

mesoderm (connective epithelium);

endoderm (vulval vestibule).

This embryological derivation is responsible for the different variants in morphology that occur during the development of the vulva.

A correct and thorough knowledge of the ‘normal’ vulva is vital for several reasons. Firstly, it is important in order to recognize some of the normal anatomical variants in order to differentiate them from pathological features. This will prevent unnecessary excision and treatment of normal areas. Secondly, it leads to a more specific and logical approach in treating vulval disorders. In some conditions, the normal anatomy of the vulva is altered and this can give diagnostic clues. It is important to note that the ‘normal’ vulva modifies itself during a woman’s lifetime, depending on age, obstetrical and gynaecological history.

Normal vulval anatomy

The vulva may be considered as the combination of the mucosal, cutaneous, muscular and connective tissue structures that compose the lower part of the female genital tract. The peculiarity of this localization means that the vulva is in close association with urological structures (urethra and bladder), gynaecological structures (vagina), and intestinal structures (rectum and anus).

The borders of the vulva are: mons pubis anteriorly, perineal body posteriorly, genital crural folds laterally and hymen medially (Figure 1.1). In this triangular‐shaped region, with naked‐eye examination, five distinct structures clearly appear: the labia majora, the clitoris, the vestibule, the labia minora and the hymen (Figure 1.2 a, b).

Figure 1.1 The vulva.

Figure 1.2 Normal vulva (a) – outer and (b) – inner vulva.

There is usually a limited description of the internal structures of the vulva in gynaecological and dermatological textbooks. These structures reach the plane of the perineal fascia (or urogenital membrane) under the skin. A knowledge of the anatomy of these structures and planes then encompasses the clitoral body, the minor vestibular bulbs and glands, the urethral opening and the paraurethral glands, which are all part of the vulva. A good understanding of the anatomy, together with its embryological development, allows a comprehensive approach to vulval morphology and correct surgical dissection if required.

The labia majora are two cutaneous folds, even and symmetrical, arising from the lateral portions of the mons pubis and extending to the posterior triangle of the perineum. Laterally they terminate on the genito‐crural fold, and medially continue to the external aspects of the labia minora, forming the interlabial sulci. On the outer surface, they are covered by hair‐bearing skin. The hair follicles are lost on the inner surface but many sebaceous glands remain.

The labia minora are two thin structures that are connected anteriorly to form the clitoral hood and, below the clitoral body, form the frenulum. Posteriorly the labia minora unite to define the fourchette The epithelium starting from the internal side of the fourchette to the hymen is called the navicular fossa. The labia minora do not have hair follicles but they are covered by numerous sebaceous glands and sweat glands.

The clitoris develops from an outgrowth in the embryo called the genital tubercle. It contains trabeculated erectile tissue, similar to the male penis, and is composed of the body (the shaft and the glans) and the crura. The glans is covered by the clitoral hood, formed by the fusion of the anterior portions of the labia minora. The body of the clitoris continues in each crus (singular form of ‘crura’), attached to the corresponding ischial ramus, beneath the descending pubic rami. Hence only about 30% of the clitoris is visible (Figure 1.3).

Figure 1.3 The clitoris.

The vestibule is the space between the hymenal ring and the internal aspect of labia minora. Its boundaries are the clitoris anteriorly, the fourchette posteriorly and the ‘Hart’s line’ laterally, which runs down the internal side of the labia minora. It represents the junction between the mucosal epithelium and the keratinized skin of the vestibule (Figure 1.4). Some authors define the lateral extension of the vestibule as the free edge of labia minora, therefore including the two types of epithelium (mucosa and skin).

Figure 1.4 Hart’s line.

Several structures open into the vestibule. The urethral opening is clearly seen with the paraurethral Skene’s glands laterally. The ducts of the Bartholin’s glands and the lesser vestibular glands open into the lower third of the vaginal introitus.

The bulb of the vestibule is located deeply and, as aggregations of erectile tissue, this may be considered as an internal part of the clitoris.

The hymen is an elastic ring‐shaped structure, covered by mucosal epithelium that separates the vagina from the vulval vestibule. After the first penetrative sexual intercourse it can be torn apart, leaving one or more scars on its surface. Very rarely the hymen may be septate or cribriform.

The mons pubis lies in front of and above the upper part of the symphysis pubis. A thick cushion of subcutaneous fat is covered by hair‐bearing keratinized epithelium.

The vulva obtains its blood supply from the internal pudendal artery and drains via the external pudendal vein. The nerve supply is from branches of the perineal nerve but the clitoris is supplied by the dorsal nerve of the clitoris, a branch of the pudendal nerve. Lymphatic drainage is to the inguinal and internal iliac nodes.

Normal vulval and vaginal flora

The vagina is colonized by several strains of bacteria. At puberty, lactobacilli increase and the glycogen metabolized by them produces lactic acid, giving a normal vaginal acidic pH of 4.5 or less. A change in the normal discharge can occur if levels of Candida albicans or Streptococcus agalactiae (beta haemolytic streptococcus) increase but this does not necessarily require any treatment.

Further reading

Andrikopoulou, M., Michala, L. and Creighton, S. M. (2013) The normal vulva in medical textbooks.

Journal of Obstetrics and Gynaecology

33

, 648–650.

Lloyd, J., Crouch, N. S., Minto, C. L.

et al.

(2005) Female genital appearance: ‘normality’ unfolds.

British Journal of Obstetrics and Gynaecology

112

, 643–646.

Neill, S. M. and Lewis, F. M. (2009) Basics of vulval embryology, anatomy and physiology, in

Ridley’s The Vulva

, 3rd edn (eds S. M. Neill and F. M. Lewis). Wiley‐Blackwell, London, pp. 13–33.

Normal anatomical variants

The shape and morphology of the vulva depend on the appearance of all the structures involved. The differences in the developmental process and integration into the whole of each structure render the vulva a unique organ. For this reason it is usual to find some variants; that should be considered normal. However, these can cause great worry to a woman when she first looks at her vulva. In addition, the explosion in cosmetic surgery for the external genitalia in recent years in order to reach a ‘perfect vulva’ has greatly increased the focus of attention on vulval appearance. As a consequence, aesthetic vulval surgery is performed, modifying structures that are normal, without any pathological reason.

Common normal vulval variants are considered here.

Agenesis of the labia minora.

This is a normal finding in women but should not be confused with labial adhesion. The latter condition is an acquired disease, more frequent in girls under 2 years due to several predisposing factors such as oestrogen deprivation, inadequate personal care, local irritants, infections or previous trauma. This situation may mimic labial reabsorption in lichen sclerosus but generally resolves spontaneously.

Asymmetry of the labia minora.

There is great variability in the size and shape of the labia minora. In one study, the length and width of the labia minora were examined in 50 women aged from 18 to 50. The length varied from 20 to 100 mm and the width from 7 to 50 mm. Sometimes a duplication of the labia minora may occur, without any pathological consequence (

Figure 1.5

). The edge of the labia minora may become rugose and the rim is often pigmented.

Sebaceous glands.

The vulva is rich in sebaceous glands (Fordyce spots) that can appear as little yellow spots spread on the vestibule and labia minora (

Figure 1.6

). In some cases, hypertrophic and inflamed sebaceous glands may upset the normal surface anatomy of the labia. This condition is known as Fox–Fordyce disease.

Vestibular papillomatosis.

Often misdiagnosed and treated as HPV condylomata, this condition is characterized by papillary growths of the vestibular mucosa located within Hart’s line. On naked eye examination, they are finger‐like projections and each has a solitary base (

Figure 1.7

).

Vestibular erythema.

Located in the vestibule at the opening of the Bartholin glands, this physiological erythema is found, in observational studies, in more than 40% of asymptomatic women. Previously associated with localized provoked vestibular pain, it is now to be considered a normal variant.

Paraurethral cysts and vestibular cysts

.