Transcript British Society for the Study of Vulval Disease

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 conditions
Follow-up
Referral for supra-specialist care
Skin disease (e.g. lichen sclerosus)
Vulvodynia
Unifocal VIN
Complicated infections
(e.g. resistant to treatment)
Supra-specialist care
(Vulval clinic)
Assessment and management of
uncommon and rare skin disease
Uncommon conditions
Vulval dermatoses (e.g. Lichen
planus)
Multifocal VIN
Any patient with symptoms that do not
respond to basic measures e.g.
Vulvodynia
Gynaecological
cancer team
Level 4 care
Patient assessment and treatment
of premalignant and malignant
vulval disease
Liaison 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
4 mm punch biopsy
3/31/2016
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 disease
Dermatoses
• General dermatological dermatoses
 Eczema psoriasis
• Infections
 Candidiasis
 STIs
• 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/combinatio
n 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 steroids
Poor understanding of anatomy
• Incorrect diagnosis
Biopsy
• Incontinence
• Complicated LS
Lichen planus overlap
• Additional diagnosis
Vulvodynia
squamous 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
3/31/2016
Assessment and treatment of
women with vulvodynia
3/31/2016
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)
3/31/2016
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?
3/31/2016
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
3/31/2016
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
Psychological and psychosexual therapy support
Behaviours
Avoid intimacy
Becomes withdrawn emotionally
Push self to make up for it
Thoughts
I’m less of a woman
I better not lead my partner on
He might find someone else
I’ll try & make up for it in other ways
Physical sensations
Muscle tension = Further pain
Headaches
Irritable bowel symptoms
Sinking feeling in stomach
Emotions
Fear / Anxiety
Stress
Guilt
Depression
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.
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
3/31/2016
Assessment and treatment of
women with VIN
3/31/2016
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
3/31/2016
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
3/31/2016
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
3/31/2016
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
3/31/2016
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)
3/31/2016
Other treatment options
•Topical agents – imiquimoid (70% response
rate, but needs vulval service supervision)
•Conservative management - eg in
pregnancy, young women
3/31/2016
VIN- Role of the gynaecologist
• Assessment and make a diagnosis
• Education and reassurance
• Uncomplicated VIN – surgical
management
• Complicated VIN refer to vulval team
3/31/2016
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