reading

Cover
Title Page
Acknowledgements
1 The Normal Vulva
1. Normal vulval anatomy
2. Normal vulval and vaginal flora
3. Further reading
4. Normal anatomical variants
5. Normal changes over the lifetime
6. Further reading
2 Taking a History and Examination
1. Taking a history
2. Examination of the vulva
3. Further reading
3 How to Take a Vulval Biopsy and the Importance of Clinico‐Pathological Correlation
1. Incisional biopsy
2. Fixation of the biopsy
3. Further reading
4 Basic Histology of the Vulva
1. Special histological stains
2. Immunohistochemical stains
3. Other tests
4. Further reading
5 Investigations in Vulval Disease
1. Investigations for infection
2. Investigations for allergy
3. Investigations for inflammatory disease
4. Imaging investigations
5. Useful web sites for patient information
6. Further reading
6 Topical Treatment in Vulval Disease
1. Introduction
2. General principles
3. Topical treatments used for vulval disease
4. Practice points
5. Further reading
6. Useful web sites for patient information
7 Symptoms in Vulval Disease
1. Pruritis (Itch)
2. Causes of vulval pruritis
3. Soreness
4. Pain
5. Dyspareunia
6. Discharge
7. No symptoms
8. Useful web site for patient information
8 Signs in Vulval Disease
1. Differential Diagnosis Based on Appearance
2. Algorithms
3. Further reading
9 Eczema, Allergy and the Vulva
1. Seborrhoeic eczema
2. Allergic Contact Eczema / Dermatitis
3. Clinical features
4. Irritant Eczema / Dermatitis
5. Urticaria
10 Psoriasis
1. Introduction
2. Incidence
3. Clinical features
4. Basic management
5. When to refer
6. Practice points
7. Further reading
8. Useful Web Sites for Patient Information
11 Lichen Simplex
1. Introduction
2. Epidemiology
3. Incidence
4. Pathophysiology
5. Histological features
6. Symptoms
7. Clinical features
8. Basic management
9. When to refer
10. Practice points
11. Further reading
12. Useful Web Sites for Patient Information
12 Lichen Sclerosus
1. Introduction
2. Incidence
3. Pathophysiology
4. Histological features
5. Symptoms
6. Clinical features
7. Associated disease
8. Risk of malignancy
9. Basic Management
10. Follow Up
11. When to Refer
12. Practice points
13. Further reading
14. Useful Web Sites for Patient Information
13 Lichen Planus
1. Introduction
2. Epidemiology
3. Incidence
4. Pathophysiology
5. Histological Features
6. Clinical Features of Lichen Planus
7. Vulval Lichen Planus
8. Risk of Malignancy
9. Basic management
10. When to refer
11. Practice points
12. Further reading
13. Useful Web Sites for Patient Information
14 Hidradenitis Suppurativa and Crohn's Disease
1. Hidradenitis Suppurativa
2. Crohn’s disease
15 Disorders of Pigmentation on the Vulva
1. Introduction
2. Postinflammatory Pigmentation
3. Post‐Traumatic Pigmentation
4. Acanthosis Nigricans
5. Melanosis
6. Pigmented lesions
7. Lentigines
8. Benign naevi
9. Atypical Genital Naevi
10. Seborrhoeic Keratoses
11. Pigmented Basal Cell Carcinoma (see Chapter 22)
12. Hypopigmentation
13. Vitiligo
14. When to Refer
15. Practice points
16. Further reading
17. Useful Web Sites for Patient Information
16 Other Dermatoses
1. Genetic disorders
2. Auto‐Immune Bullous Disease
3. Drug Eruptions
4. Manifestations of Underlying Disease
5. Inflammatory ulcers
6. Others
17 Vulval Infection – Sexually Transmitted
1. Normal flora
2. Trichomoniasis
3. Chlamydia
4. Lymphogranuloma Venereum
5. Gonorrhoea
6. Syphilis
7. Chancroid
8. Donovanosis (Granuloma Inguinale)
9. Herpes Simplex Infection
10. Human Papillomavirus Infection
11. Molluscum contagiosum
12. Scabies
13. Pubic lice
18 Vulval Infection – Nonsexually Transmitted
1. Bacterial Infections
2. Bacterial Vaginosis
3. Erythrasma
4. Staphylococcal and Streptococcal Infections
5. Folliculitis
6. Bartholin abscess
7. Other Staphylococcal Infections
8. Cellulitis
9. Other Streptococcal Infections
10. Vulvovaginal Candidiasis
11. Tinea cruris
12. Viral infections
19 Vulval Intraepithelial Neoplasia
1. Introduction
2. Epidemiology
3. Aetiology
4. Prevention
5. Histological features
6. Symptoms
7. Clinical features
8. Management
9. Progression
10. Melanoma in situ
11. When to refer
12. Practice points
13. Further reading
14. Useful Web Site for Patient Information
20 Extramammary Paget’s Disease
1. Introduction
2. Epidemiology
3. Histological features
4. Classification
5. Symptoms
6. Clinical features
7. Management
8. Recurrences
9. Progression
10. Follow up
11. When to refer
12. Practice points
13. Further reading
14. Useful Web Site for Patient Information
21 Vulval Squamous Cell Carcinoma
1. Introduction
2. Epidemiology
3. Aetiology / histology
4. Symptoms and Clinical Features
5. Management
6. Surgery
7. Complications
8. Radiotherapy
9. Follow up
10. Prognosis
11. When to refer
12. Practice points
13. Further reading
14. Useful Web Site for Patient Information
22 Other Vulval Cancers
1. Basal Cell Carcinoma
2. Malignant melanoma
3. Other Malignant Tumours and the Vulva
23 Vulvodynia
1. Definition
2. Epidemiology
3. Pathophysiology
4. Psychological Aspects of Vulvodynia
5. Histology
6. Symptoms
7. Signs
8. Diagnosis
9. Management
10. Further reading
11. Useful Web Sites for Patient Information
24 Psychosexual Aspects of Vulval Disease
1. Clinical Psychological Assessment
2. Further reading
25 Benign Lesions
1. Epidermoid cysts
2. Comedones
3. Syringomata
4. Hidradenoma Papilliferum
5. Lymphangioma / Lymphangiectasia
6. Further reading
Index
End User License Agreement

List of Tables

Chapter 04
1. Table 4.1 Glossary of cell types and structures.
2. Table 4.2 Glossary of histopathological terms.
3. Table 4.3 Special histological stains.
4. Table 4.4 Immunohistochemical stains.
Chapter 06
1. Table 6.1 Classification of topical treatment.
2. Table 6.2 Grading of topical steroids (European).
Chapter 07
1. Table 7.1 Causes of vulval pruritis.
2. Table 7.2 Causes of vulval soreness.
3. Table 7.3 Causes of vulval pain.
4. Table 7.4 Causes of dyspareunia.
5. Table 7.5 Causes of discharge.
6. Table 7.6 Causes of change in appearance without symptoms.
Chapter 08
1. Table 8.1 Terminology of vulval lesions.
2. Table 8.2 Describing vulval lesions.
3. Table 8.3 Common presentations of vulval disorders.
Chapter 09
1. Table 9.1 Common causes of contact dermatitis on the vulva.
2. Table 9.2 Common causes of irritant eczema/dermatitis on the vulva.
Chapter 13
1. Table 13.1 Other sites that can be affected by erosive LP.
Chapter 14
1. Table 14.1 Hurley staging system for hidradenitis supparativa.
Chapter 15
1. Table 15.1 Causes of diffuse pigmentation.
2. Table 15.2 Types of acanthosis nigricans.
3. Table 15.3 Pigmented lesions on the vulva.
Chapter 16
1. Table 16.1 Other dermatoses.
2. Table 16.2 Clinical features and immunofluorescence patterns in auto‐immune bullous disease.
3. Table 16.3 Diagnostic criteria for Behcet’s syndrome.
Chapter 17
1. Table 17.1 Major sexually transmitted infections.
Chapter 18
1. Table 18.1 Nonsexually transmitted vulval infections.
2. Table 18.2 Staphylococcal and streptococcal infections.
Chapter 19
1. Table 19.1 Squamous vulval intraepithelial neoplasia (VIN) terminology.
Chapter 20
1. Table 20.1 Immunocytochemistry in EMPD.
Chapter 23
1. Table 23.1 Treatments used.

List of Illustrations

Chapter 01
1. Figure 1.1 The vulva.
2. Figure 1.2 Normal vulva (a) – outer and (b) – inner vulva.
3. Figure 1.3 The clitoris.
4. Figure 1.4 Hart’s line.
5. Figure 1.5 Bifid labium minus.
6. Figure 1.6 Fordyce spots.
7. Figure 1.7 Vestibular papillomatosis.
8. Figure 1.8 Angiokeratomata.
9. Figure 1.9 Vulval varicosities.
Chapter 02
1. Figure 2.1 A lamp with good light and magnification is vital.
2. Figure 2.2 Plastic speculum.
Chapter 03
1. Figure 3.1 The area to be biopsied is cleaned with antiseptic.
2. Figure 3.2 Local anaesthetic is infiltrated.
3. Figure 3.3 Punch biopsy.
4. Figure 3.4 The punch biopsy is inserted and rotated.
5. Figure 3.5 A core of tissue is obtained and cut off at the base.
6. Figure 3.6 Cold knife biopsy – ellipse is cut.
7. Figure 3.7 Cold knife biopsy – the ellipse is then excised to a depth of at least 5 mm.
8. Figure 3.8 The surgical wound that results.
9. Figure 3.9 Haemostasis is achieved by suturing.
10. Figure 3.10 The final appearance.
11. Figure 3.11 The tissue is then immediately put into fixative.
Chapter 04
1. Figure 4.1 Histopathological features of the normal vulva.
Chapter 05
1. Figure 5.1 Bacterial swab.
2. 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.
3. Figure 5.3 ‘Banana’ scalpel used to obtain skin scrapings.
4. Figure 5.4 The scaly edge of the lesion is gently scraped on to paper.
5. Figure 5.5 Dark paper used to collect skin scrapings.
6. Figure 5.6 Allergens at suitable concentration are placed on Finn chambers.
7. Figure 5.7 Finn chambers taped on back and marked.
8. Figure 5.8 Positive patch test reaction – eczematous papule at site of application.
Chapter 06
1. Figure 6.1 Cream, ointment and gel, illustrating differences between the preparations.
2. Figure 6.2 Emulsifying ointment used as a soap substitute.
3. Figure 6.3 In warm water, the emulsifying ointment is made into an emulsion to wash in.
4. Figure 6.4 Striae on the thighs after excessive use of a potent topical steroid.
5. Figure 6.5 Telangiectasia on outer labia majora at incorrect site of application of topical steroids for lichen sclerosus.
6. Figure 6.6 Tinea incognito – papules and folliculitic lesions after the application of a topical steroid on a fungal infection.
7. Figure 6.7 Steroid foam preparation that can be inserted into the vagina to treat inflammatory disease.
8. Figure 6.8 Potassium permanganate – dissolve a tablet in warm water to produce a pale purple liquid.
9. Figure 6.9 Gauze is soaked in the solution and this can be applied to the vulva for 10 minutes.
10. Figure 6.10 Barrier preparation applied to the skin showing the resistance to water.
11. Figure 6.11 Punctate erosions seen with the use of imiquimod.
Chapter 08
1. Figure 8.1 (a) Diagnostic algorithm for vulval ulceration. (b) Diagnostic algorithm for vulval oedema.
Chapter 09
1. Figure 9.1 Chronic eczema showing inflammation and dryness.
2. Figure 9.2 Acute allergic contact dermatitis.
3. Figure 9.3 Allergic contact dermatitis with extension to perianal area and thighs.
4. Figure 9.4 Erosions – allergic contact dermatitis to depilatory cream.
5. Figure 9.5 Irritant dermatitis secondary to urinary incontinence.
Chapter 10
1. Figure 10.1 Typical psoriatic plaque on elbow showing silver scales.
2. Figure 10.2 Psoriasis affecting scalp, hairline and ears.
3. Figure 10.3 Onycholysis and pitting of the nails
4. Figure 10.4 Vulval psoriasis – confluent plaques with well defined edge.
5. Figure 10.5 Scaling on lesions on mons pubis.
6. Figure 10.6 Umbilical psoriasis.
7. Figure 10.7 Psoriasis with excoriation and scaling.
8. Figure 10.8 Psoriasis affecting the labia majora, mons pubis and extending into inguinal folds.
9. Figure 10.9 Fissuring in inguinal folds.
Chapter 11
1. Figure 11.1 Gross thickening of skin on inner and outer surfaces of the labia majora.
2. Figure 11.2 Lichenification of labia majora and minora.
3. Figure 11.3 Lichenification of labia majora with accentuation of the skin markings and rugose appearance.
4. Figure 11.4 Perianal lichen simplex.
Chapter 12
1. Figure 12.1 Histology of LS.
2. Figure 12.2 Atrophic extragenital LS.
3. Figure 12.3 Sclerotic extragenital LS.
4. Figure 12.4 Ecchymosis in extragenital LS.
5. Figure 12.5 Symmetrical white plaques on labia majora.
6. Figure 12.6 Perianal LS.
7. Figure 12.7 Ecchymosis (purpura) can be widespread in some patients.
8. Figure 12.8 Sclerotic plaques on labia minora.
9. Figure 12.9 Postinflammatory hyperpigmentation in LS.
10. Figure 12.10 Hyperpigmentation in LS.
11. Figure 12.11 Pre‐pubertal LS.
12. Figure 12.12 Fusion of labia minora reducing introitus.
13. Figure 12.13 Sealing clitoral hood and burial of clitoris.
14. Figure 12.14 Lichen sclerosus on prolapsed vaginal mucosa.
15. Figure 12.15 Acanthotic disease.
16. Figure 12.16 Lichen sclerosus and psoriasis can coexist.
17. Figure 12.17 Squamous cell carcinoma developing on LS.
Chapter 13
1. Figure 13.1 Typical histological features of LP.
2. Figure 13.2 Plaque of lichen planus with Wickham’s striae on the surface.
3. Figure 13.3 Postinflammatory hyperpigmentation in inguinal fold.
4. Figure 13.4 Lichen planus affecting gingival margins.
5. Figure 13.5 Classic type of vulval LP with Wickham’s striae.
6. Figure 13.6 Hypertrophic LP affecting labia majora.
7. Figure 13.7 Erosive LP.
8. Figure 13.8 Scarring in erosive LP.
9. Figure 13.9 Scarring around lacrimal duct in lichen planus.
10. Figure 13.10 Squamous cell carcinoma in LP – eroded tumour left labium majus.
Chapter 14
1. Figure 14.1 Hidradenitis suppurativa – vulval involvement.
2. Figure 14.2 Bridged comedones.
3. Figure 14.3 Oedema in ano‐genital Crohn’s disease may be the only feature.
4. Figure 14.4 Lymphangiectasia are a frequent feature.
5. Figure 14.5 ‘Knife‐cut’ fissures.
6. Figure 14.6 Fissuring in gluteal cleft.
7. Figure 14.7 Fissuring in abdominal scar.
Chapter 15
1. Figure 15.1 Linea nigra.
2. Figure 15.2 Postinflammatory hyperpigmentation after eczema.
3. Figure 15.3 Acanthosis nigricans in axilla.
4. Figure 15.4 Thickened velvety surface seen in acanthosis nigricans.
5. Figure 15.5 Melanosis –irregular pigmentation can be seen in the vestibule.
6. Figure 15.6 Melanosis showing very dark pigmentation.
7. Figure 15.7 Histological features of melanosis – increased melanin and some pigment in the dermis.
8. Figure 15.8 Benign mucosal naevus.
9. Figure 15.9 Benign compound naevus.
10. Figure 15.10 Atypical genital naevus.
11. Figure 15.11 Seborrhoeic keratosis.
12. Figure 15.12 Vitiligo (patient also has lichen sclerosus).
Chapter 16
1. Figure 16.1 Hailey‐Hailey disease – pruritic papules on labium majus.
2. Figure 16.2 Bullous pemphigoid – tense bullae.
3. 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
4. Figure 16.4 Pemphigus: Direct immunofluorescence shows a positive intraepidermal intercellular deposition of IgG (shown) and C3. Please notice the intraepidermal acantholytic blister
5. Figure 16.5 Mucous membrane pemphigoid – scarring and erosions.
6. Figure 16.6 Mucous membrane pemphigoid – desquamative gingivitis.
7. Figure 16.7 Pemphigus vulgaris of the vulva.
8. Figure 16.8 Stevens‐Johnson syndrome – vulval involvement.
9. Figure 16.9 Toxic epidermal necrolysis – full thickness epidermal loss.
10. Figure 16.10 Healing aphthous ulcer lower right labium majus.
11. Figure 16.11 Behcet’s disease – deeper scarring ulcers.
12. Figure 16.12 Lipschutz ulcer.
13. Figure 16.13 Genital graft‐versus‐host disease – appearances may be indistinguishable from erosive lichen planus.
14. Figure 16.14 Zoon’s vulvitis.
15. Figure 16.15 Langerhans cell histiocytosis of the vulva.
Chapter 17
1. Figure 17.1 Lymphogranuloma venereum: sinuses.
2. Figure 17.2 Primary chancre of syphilis on right inner labium majus.
3. Figure 17.3 Herpes simplex infection – multiple painful vesicles.
4. Figure 17.4 Confluent vesicles in HSV.
5. Figure 17.5 Hypertrophic HSV infection in immunosuppressed.
6. Figure 17.6 Warts – papillomatous lesions.
7. Figure 17.7 Warts – confluent plaques.
8. Figure 17.8 Molluscum contagiosum – umbilicated papules.
9. Figure 17.9 Scabies – crusted lesions in the finger spaces in severe infection.
Chapter 18
1. Figure 18.1 Erythrasma.
2. Figure 18.2 Scaly edge to the plaques.
3. Figure 18.3 Folliculitis.
4. Figure 18.4 Bartholin’s abscess.
5. Figure 18.5 Staphylococcal infection (S. aureus) with superficial desquamation.
6. Figure 18.6 Vulval cellulitis with erythema and oedema involving the right labium majus and extending to mons pubis.
7. Figure 18.7 Acute candidiasis.
8. Figure 18.8 Satellite lesions spreading to thighs.
9. Figure 18.9 Tinea cruris – extending lesions with central sparing.
10. Figure 18.10 Extensive infection caused by inappropriate use of topical steroids (tinea incognito).
Chapter 19
1. 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.
2. 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.
3. Figure 19.3 Typical multifocal uVIN with white, red and pigmented lesions.
4. Figure 19.4 Extensive multifocal uVIN with perianal involvement.
5. Figure 19.5 Undifferentiated VIN with localized and small lesions.
6. Figure 19.6 Multifocal uVIN lesions forming confluent plaques.
7. Figure 19.7 Undifferentiated VIN with only pigmented papules, also in the perianal region.
8. Figure 19.8 Differentiated VIN presenting with multifocal erosive lesions in the fourchette on the background of LS.
9. Figure 19.9 (a) Undifferentiated VIN and (b) after treatment with imiquimod.
10. Figure 19.10 (a) Vulval SCC. (b) Labium majus, which developed in a region with dVIN on the background of LS.
11. Figure 19.11 Multifocal uVIN with progression to vulval SCC on the perineum.
12. Figure 19.12 Vulval melanoma in situ.
Chapter 20
1. Figure 20.1 Clusters of Paget’s cells with abundant clear cytoplasm and round nuclei in the basal layer.
2. Figure 20.2 Extramammary Paget’s disease affecting the right labium majus.
3. Figure 20.3 Extramammary Paget’s disease with extensive sharply demarcated erythema with areas of hyperkeratosis, erosions and moist skin.
4. Figure 20.4 Extension of EMPD to the perianal area.
5. 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
1. Figure 21.1 Verrucous squamous cell carcinoma.
2. 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
1. Figure 22.1 Typical appearances of BCC – pearly papule.
2. Figure 22.2 Vulval BCC – small nodule on outer labium majus.
3. Figure 22.3 Melanoma right labium minus.
4. Figure 22.4 Extensive nodular malignant melanoma.
5. Figure 22.5 Variation in colour with amelanotic areas.
Chapter 23
1. Figure 23.1 Subsets of vulvodynia.
Chapter 25
1. Figure 25.1 Multiple epidermoid cysts.
2. Figure 25.2 Large epidermoid cyst on left labium majus in a patient with lichen planus.
3. Figure 25.3 Open comedone.
4. Figure 25.4 Hidradenoma papilliferum.
5. Figure 25.5 Lymphangiectasia in Crohn’s disease.

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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–pathology
Classification: LCC RG261 (print) | LCC RG261 (ebook) | NLM WP 200 | DDC 618.1/6–dc23
LC 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

A Practical Guide to Vulval Disease

Diagnosis and Management

Acknowledgements