Vulval Disease

Lecture framework for obstetrics and gynaecology core trainees

Introduction• These presentations were prepared by Caroline

Owen Consultant Dermatologist and David Nunns Consultant Gynaecologist on behalf of the BSSVD education group.

• They are designed as a framework, to cover the vulval disease component of the core curriculum for obstetrics and gynaecology trainees, as set out by the RCOG.

• The clinical images have been omitted for patient consent issues, and speakers are encouraged to insert their own pictures where indicated.

• The lectures are intended only as a guide and resource.

Lecture one - Objectives

• Assessment of vulval patient– History, examination, investigations

• Treatment principles– Emollients and topical steroids

• Overview of most common vulval dermatoses– Eczema, psoriasis, candidiasis, lichen

sclerosus, lichen planus

StratOG guidance on appropriate practitioners and level of care for vulval conditions

Practitioner Roles and responsibilities Suggested conditions

GP Patient assessment (history, examination, swabs)

Uncomplicated vulvo-vaginal infections and follow-up of vulval conditions e.g. lichen sclerosus

General gynaecology clinics

Patient assessment (history, examination, swabs, biopsy)Treatment for common & uncommon conditionsFollow-upReferral for supra-specialist care

Skin disease (e.g. lichen sclerosus)VulvodyniaUnifocal VINComplicated infections(e.g. resistant to treatment)

Supra-specialist care(Vulval clinic)

Assessment and management of uncommon and rare skin disease

Uncommon conditionsVulval dermatoses (e.g. Lichen planus)Multifocal VINAny patient with symptoms that do not respond to basic measures e.g. Vulvodynia

Gynaecological cancer teamLevel 4 care

Patient assessment and treatment of premalignant and malignant vulval diseaseLiaison with the extended cancer team

Vulval cancer

VIN (all types including Paget's disease)

Who sees vulval disease?

• GP• Dermatology• Gynaecology• GUM• Urology We all need to get good at it – there is plenty

out there….

Vulval disease

1. Assessment of the patient with a vulval problem

2. Treatment principles

3. Specific vulval dermatoses– Eczema (irritant contact dermatitis, allergic

contact dermatitis, lichen simplex)– Psoriasis, recurrent candidiasis– Lichen sclerosus, lichen planus

Assessment of patient with vulval problem

• PC

• HPC

• PMH

• DH

• FH

• SH

Good start but ………

Vulval clinic – history taking

• Need time & box of tissues

• Have often had many appointments, investigations, procedures already

• Confused, wary, distressed

• Relationships may be under pressure

• May be struggling to conceive

• May not have spoken to anyone else

Picture of end stage LS

Vulval clinic – history taking

• Timescales

• Interventions that have helped or not

• Ask about sex

• Ask about urinary continence

• All topical applications

• Hygiene/washing routine

• Previous swabs, biopsies, investigations

Vulval clinic - examination• Good light• Whole skin (including mouth)• Be systematic –

• mons pubis• crural folds• labia majora • labia minora• clitoris • introitus• fourchette• perianal area

Anatomy

Picture of a normal vulval

Terminology

• Erythema• Macule – flat• Papule – raised <0.5cm• Nodule – > 0.5cm• Vesicle – blister < 0.5cm• Bulla – blister > 0.5cm• Ecchymosis, purpura, petichiae –

bleeding/bruise

Terminology cont:• Erosion – loss of superficial epidermis• Ulcer – loss of epidermis +/- dermis• Glazed erythema – red, shiny skin but

intact epidermis• Excoriation – scratch • Fissuring – splits/cuts• Lichenification – thickening• Atrophy – thinning, wrinkling • Fusion – scarring, loss of vulval

architecture

2 images of LS, one adult, one child

Vulval clinic - investigations

• Consider GUM screen/referral

• Viral and bacterial swabs (candida very common without obvious clinical signs)

• Patch testing (if suspect allergic contact dermatitis)

• Clinical photograph

• Biopsy

Vulval biopsy• As outpatient• Local anaesthetic• 4mm punch biopsy (usually)• 5’0 vicryl rapide• Site – NOT eroded or ulcerated area• Incisional/punch biopsy for rashes, excision for

lesions• Must document site and all clinical information

with differential for pathologists• If performing excision be confident of required

margins

04/19/23

4 mm punch biopsy

Picture of erosive LP to demonstrate site of biopsy

Treatment principles 1• Complex patients need multidisciplinary

team– Dermatology– GUM– Urogynaecology– Pathology– Physiotherapy– Psychosexual counselling – GP– Patient support groups

Treatment principles 2

• Emollients emollients emollients

• Topical steroids

• Lubricants

• Dilators (Amielle comfort or Fenmax)

Emollients Emollients Emollients

• Moisturisers

• Vital active treatment

• Repairs skin’s barrier

• Prevents penetration by allergens and irritants and bacteria

• Reduces itch and makes skin feel more comfortable

• Soap substitute & leave on moisturiser

Emollients

• Lotions – light, spread easily, cooling but not very moisturising

• Creams– Heavier than lotions but not as moisturising as

ointments

• Ointments– Do not contain any water, thick and can be difficult

and greasy to apply but very good at moisturising

Emollients

• Light– E45– Double Base– Ung Merck– Diprobase cream

• Greasy– Epaderm– Hydromol ointment– Emulsifying ointment– 50/50 white soft paraffin/liquid paraffin

– Aqueous, too light – use only as soap substitute (need to wash off)

THE BEST EMOLLIENT IS

THE ONE THE PATIENT

WILL USE

Topical steroids

• Very effective

• Very safe

• Underuse a MUCH greater problem than overuse

Topical Steroids

• Steroids are produced naturally by the body

• Anti-inflammatory

• Allow skin a chance to repair

• Side effects very rare, steroid atrophy extremely rare

Topical steroids

• Can use on broken skin

• Can use longer than 7 days

• Ignore the word ‘sparingly’

• Can use potent and superpotent steroids on vulva (and often need to)

• Better to reduce frequency than go up and down ‘steroid ladder’

Topical Steroids - guidelines

• Don’t use more than twice daily

• Must use with regular emollients

• Stop using them once completely clear but continue with moisturisers

• Start again if necessary

• Use mirror to demonstrate correct site

Vulval diseaseDermatoses

• General dermatological dermatoses Eczema psoriasis

• InfectionsCandidiasisSTIs

• Specific vulval dermatoses Lichen sclerosus, lichen planus

Lesions• Benign

Bartholin cyst Epithelial (sebaceous) cyst Angiokeratoma

• Malignant VIN/SCC BCC/melanoma

Vulvodynia• Localised/ provoked or unprovoked

Vulval Eczema

• Very common• Look for signs of eczema elsewhere• Defect in barrier function of skin• Often atopic • Always itchy• Often worse at night• Eczema = dermatitis • Irritant contact dermatitis / allergic contact

dermatitis

Image of vulval eczema

2 images of vulval eczema demonstrating excoriations and fissures

Vulval eczema with fissuring in crural folds

Vulval eczema - treatment

• Emollients emollients emollients

• Avoid soap

• Loose cotton underwear

• Topical corticosteroids

• Consider irritants and allergens (wetwipes)

• Pre-disposes to candidiasis (impaired barrier function) swab to check

• Irritant contact dermatitis – chapped, damaged skin, can happen to anyone– Water, abrasives– Soap, shampoo– Wool/synthetic clothing– Cold weather

• Allergic contact dermatitis – more common in those who already have eczema– Caused by a true allergic reaction to a specific substance

(allergen)– Previous contact (often prolonged) with substance is needed

to start the allergic reaction– Lasts forever– Diagnosed on patch testing– Consider if previously controlled eczema flares or start to

react to topical treatments

2 images of vulval eczema and lichen simplex

Images of lichen simplex on vulva and leg

Image of Perianal eczema

Vulval psoriasis

• Appearances often deceptive

• Look for signs of psoriasis elsewhere

• May have family history

• Often sore

• Can be psychologically disabling

4 images of psoriasis – plaque, flexural, vulval

Vulval psoriasis - treatment• Explain diagnosis

• Loose cotton clothing

• Emollients

• Refer to dermatology (options are topical steroids/topical Vitamin D analogues/Immunomodulators/combination therapies– Trimovate/Alphosyl HC/ Curatoderm/Protopic

• May need systemic therapy

Vulvovaginal candidiasis

• Common• Difficult to diagnose clinically• Pain, itch, dyspareunia, swelling• Take a swab• Albicans in 80 -92%• Non-albicans (e.g. glabrata) in the rest• Often associated with eczema• Recurrent if >6 episodes in one year

Image of typical vulval candidiasis

2 images of dry fragile vulva with satellite/ perifollicular superficial peeling often seen in VVC

Vulvovaginal candidiasis - treatment

• Emollients – long term• Topical steroid at night during acute phase• Oral fluconazole – as stat treatment AND

then maintenance therapy (usually weekly)• Relapse very common if treatment stops• Consider stopping OCP/HRT (related to ^

oestrogen)• No need to treat asymptomatic partners

Maintenance fluconazole therapy for recurrent vulvovaginal candidiasis. Sobel et al NEJM 2004 351:876-883

Before and after treatment pictures of VVC showing significant swelling of vulva before therapy

Lichen sclerosus

• Prevalence 1:300 – 1:1000• Very often associated with urinary

incontinence• Any age but particularly peri or post

menopause and prepuberty• Unknown aetiology

– ? Circulating autoantibodies to BMZ proteins e.g. ECM1

– Clin exp derm 2004;29(5)499-504

Lichen sclerosus

Symptoms:

• Intense itching

• Pain

• Dyspareunia

Lichen sclerosus

Signs:

• Pallor• Atrophy• Excoriations• Erosions and purpura• Hyperkeratosis• Loss of vulval architecture/scarring

4 slides of LS including perianal, and extragenital disease

Lichen sclerosus treatment

• Explain diagnosis (not infectious, not cancerous)

• Control rather than cure

• Information leaflet, patient support group

• Emollients

• Dermovate

Lichen sclerosus treatment

Super-potent topical steroid e.g. Dermovate• Once daily for 1 month• Alternate days for 1 month• Twice weekly for 1 month • then as required, if relapse occurs return to

frequency that controlled symptoms

BJD 2010;163(4):672-682

Lichen sclerosus treatment

• Reassure re steroid side effects• 30g over 3 months to control disease• 30g over 6 months safe as maintenance

treatment• Teach self examination and advise to seek help

if any non-healing erosions/lumps

4 slides of LS, some with advanced disease

LS –treatment failure• Treatment non-compliance Fear of steroidsPoor understanding of anatomy

• Incorrect diagnosis Biopsy

• Incontinence• Complicated LS Lichen planus overlap

• Additional diagnosisVulvodyniasquamous cell carcinoma

Treatment applied to wrong site

Image

Image of VIN/SCC with LS

Vulval lichen planus

Symptoms:

• Pain

• Itching

• Discharge

• Bleeding

• Dyspareunia

Images of oral and vulval lichen planus

Vulval lichen planus

Signs:

• Erythematous flat-topped papules on keratinised skin

• Fine reticulate white pattern on mucosal surfaces

• Erosions in more severe disease• Scarring• Discharge• Vaginal stenosis

4 slides of lichen planus

Vulval lichen planus - treatment

• Explain diagnosis (not infectious, not cancerous)

• Control rather than cure

• Information leaflet, patient support group

• Emollients

• Topical steroids

Vulval lichen planus - treatment

• Patients should be referred to dermatology• Erosive disease very resistant to treatment: Superpotent topical steroids Prednisolone pessaries Oral steroids Hydroxychloroquine, Methotrexate, Mycophenolate

mofetil.

• Surgery – last resort, in conjunction with steroids and dilators to prevent restenosis

Summary

• Assessment of vulval patient– History, examination, investigations

• Treatment principles– Emollients and topical steroids

• Overview of most common vulval dermatoses– Eczema, psoriasis, candidiasis, lichen

sclerosus, lichen planus

Lecture two - Objectives

• Assessment and treatment of women with vulvodynia

• Assessment and treatment of women with premalignant disease (VIN)

• Knowledge of the team approach to women with vulval disease and role of the general gynaecologist

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Assessment and treatment of women with vulvodynia

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VULVODYNIA

• Vulval discomfort, most often described as a burning pain, occurring in the absence of visible findings or a specific, clinically identifiable, neurological disorder

• A chronic pain syndrome• Unprovoked or provoked pain• Localised or generalised

– Hemivulvodynia– Clitorodynia– Vestibulodynia (aka vestibulitis)

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Assessment of women with vulval pain

• Pain– Site– Radiation– Relieving/aggravating factors– Severity of pain-subjective/objective– Impact on function? (Work, play)

– Other pain issues – sexual pain?– Back problems? Coccyx injuries?

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Clinical examination

• Often normal appearances• Allodynia (touch sensitivity) may be seen (Q

tip swab test)• Important not to overlook subtle skin disease

eg small fissures, vulval eczema

Vulvodynia – additional points

• Patient experience is often poor– Delay in the diagnosis/focus on medical

treatments– Often misdiagnosis or inappropriate diagnosis

• Stress, anxiety and sexual issues are often overlooked in gynaecology clinics

Management of women with vulvodynia

4 ‘P’s

• Patient education and reassurance

• Pain modifying drugs

• Physical treatments

• Psychological and psychosexual therapy support

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Patient education and reassurance

• Give a diagnosis and written information• Explain chronic pain pathway mechanisms• Explain what it is not! Eg cancer , STDs, impact

on fertility• Refer to patient support organisations eg

Vulval Pain Society

Pain modifying drugs

• Tricyclic antidepressants– Ami or nortryptyline– Escalate dose/warn of often shortlived side effects

• Gabapentin/pregabalin

• 70% response rate• Important to judge benefit/SE of treatment

Physical treatments

• Pelvic floor hypertonicity is common in pain

• Desensitisation = make less sensitive!– Digital massage– Vaginal trainers/dilators– Pelvic floor exercises– Use of a simple vibrator– Biofeedback

BehavioursAvoid intimacy

Becomes withdrawn emotionallyPush self to make up for it

Thoughts Physical sensationsI’m less of a woman Muscle tension = Further painI better not lead my partner on HeadachesHe might find someone else Irritable bowel symptomsI’ll try & make up for it in other ways Sinking feeling in stomach

EmotionsFear / Anxiety

StressGuilt

Depression

Psychological and psychosexual therapy support

Psychological and psychosexual therapy support

• Sexual dysfunction (esp vaginismus) is near universal with provoked pain

• Patients will benefit from psychosexual therapy if there is sexual dysfunction (eg vaginismus, low libido, poor arousal)

• Stress/anxiety will ‘fuel’ pain• Discuss strategies to reduce this eg lifestyle,

counselling, CBT.

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Vulvodynia - Role of the gynaecologist

• Assessment and make a diagnosis

• Education and reassurance

• Discussion of chronic pain pathways

• Start basic treatment

• Encourage self management

• Triage to vulval team depending on patients needs

• Think 3Ps

Assessment and treatment of women with VIN

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VIN – ISSVD 2005 Classification• Usual type – warty, undifferentiated • Combines VIN2/3. No VIN1.

Associated with high risk HPV.

• Differentiated type Associated with vulval cancer, lichen sclerosus, squamous hyperplasia

84

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VIN – Clinical Features

• Itch, sore or a lump• Gross appearance – white/warty, red,

pigmented, ulcer• Unifocal or multifocal• Can affect any vulval structure and

perineum. 80% of lesions are on the labia• 10-15% asymptomatic

85

VIN made easy! ‘Uncomplicated’ VIN

• Unifocal disease• Less than 1cm in diameter• Site amenable to primary

closure if surgery considered

• GENERAL GYNAECOLOGY MANAGEMENT IF CONFIDENT

‘Complicated’ VIN

• Multifocal disease• Large areas greater than 1cm• Difficult site – eg clitorus or

perineum (surgery might compromise function)

• Immunosuppressed patients• Difficult lesions to assess eg

indurated lesions• VIN associated with LS• REFER TO VULVAL SERVICE

Picture of unifocal VIN

Picture of multifocal VIN

Making a diagnosis

• Full history – smoking? immunosuppression?

• Examination of genital tract with good light (include perianal area)

• Punch biopsy(ies)

• Check cervix / last smear

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Risk of invasive disease

• Exact risk unknown• 15% of cases of VIN associated with

invasive disease • 3% in treated patients• 30-50% in untreated patients• 1% rate of invasive disease in surgical

specimens

04/19/23

Management objectives

• Exclude invasive disease

• Symptoms relief

• Preservation of function

• Sustained remission

• Reduce the risk of malignant progression

Treatment options

• Surgery– Aim for complete excision of lesion with a clear

margin with primary closure– Advantages

• High cure rates• Good symptom relief

– Disadvantages• Close/incomplete margins have a higher recurrence

rate• Not recommended for multifocal disease• Can produce disfigurement if difficult site (eg perineum

or clitoral hood) or large areas (>2cm)

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Other treatment options

•Topical agents – imiquimoid (70% response rate, but needs vulval service supervision)

•Conservative management - eg in pregnancy, young women

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VIN- Role of the gynaecologist

• Assessment and make a diagnosis

• Education and reassurance

• Uncomplicated VIN – surgical management

• Complicated VIN refer to vulval team

A team approach for managing vulval disease

• A ‘vulval service’ is defined as an multidisciplinary team of health professionals interested in vulval disorders– ‘Vulval’ team in vulval clinic– Dermatology and gynaecology– Psychosexual counselling– Physiotherapy – Pain management– Clinical psychology– Plastic surgery

• Self management important• Management is provided at all levels of care (eg GP and

hospital)• Referral to a vulval clinic depending on the needs of the

patients