Management of Vaginal Intraepithelial Neoplasia and Vulva Skin disorder   

Management of Vaginal Intraepithelial Neoplasia and Vulva Skin disorder

Dr Sunday Ajayi DFRSH MRCOG Consultant Gynaecologist Furness General Hospital

Dr Funmi Odusoga DFRSH MRCOG Consultant Gynaecologist Royal Bolton hospital

Table of Content

1. General Advice for all Vulva conditions

2. Vulval intraepithelial neoplasia

3. Vulva Lichen Sclerosis

4. Lichen Planus

5. Lichen simplex

6. Vulval Psoriasis

7. Vulval Eczema

1.General Advice for all Vulva conditions

Avoid contact with soap, shampoo and bubble bath

• Simple emollients can be used as a soap substitute and general moisturiser
• Avoid tight fitting garments which may irritate the area
• Avoid use of spermicidally lubricated condoms
• Explain diagnosis, sequelae and offer accurate information leaflet
• Exclude sexually transmitted infections
• Always assess for sexual dysfunction

2.Vulval intraepithelial neoplasia

• Premalignant disease of the vulva- Vulva intraepithelial neoplasia

Aetiology

• HPV 16 is a cause
• A second type, generally not HPV related occurs in conjunction with lichen sclerosus or lichen planus (known as differentiated type)
• The risk of progression to squamous cell carcinoma is much greater with the differentiated type of VIN and needs specialised management.
• VIN is commoner in immunocompromised women.
• Smoking is also a risk factor.
• Affects mainly the labia minora and the perineum.
• It may also extend to the perianal and anal mucosa

Presentation

Symptoms

• Lumps
•  Burning and itch/irritation
• Asymptomatic
• Pain
• May be asymptomatic.

Signs

• Appearance is variable
• Raised white, erythematous or pigmented lesions occur and these may be warty, moist or eroded (pigmented lesions were previously known as Bowenoid papulosis)
• Multifocal lesions are common

Diagnosis

• colposcopy & vulva biopsy
• Multiple biopsy may be required to avoid missing invasive disease

Complications

• Squamous cell carcinoma 9-18.5% of women with VIN
• Recurrence is common and progression to cancer can still occur following treatment
• Psychosexual problems after surgery

Classified

• VIN 1-3
• Invasive potential of VIN3 is low (<10%), takes 20-30yrs to progress to invasion and spontaneous regression up to 40%
• Low grade VIN may be observed

Management

• Ensure cervical cytology is up to date
• Refer for colposcopy to rule out Cervical intraepithelial neoplasia
• Refer for anoscopy if there is perianal lesion

Treatment Regimen

• Local excision
• Imiquimod 5%
• Vulvectomy-Recurrence may still occur

Pregnancy and breastfeeding

Imiquimod and 5-Flourouracil are not licensed in pregnancy

Alternative regimen

• Local destruction with laser, photodynamic therapy, cryotherapy
• 5 Fluorouracil cream -results are variable and side effects common
• Supervision of partial thickness lesions which may regress spontaneously

Follow up

• Close FU is mandatory though regression may occur

 

3.Vulva Lichen Sclerosis

• Lichen sclerosis is an inflammatory dermatosis of unknown aetiology

Aetiology

• Auto-immune factors may be contributory.
• There is increased frequency of other autoimmune conditions in this patient

Symptoms

• Itching, soreness, dyspareunia if introital narrowing, urinary symptoms, constipation if there is peri-anal involvement
• Rarely asymptomatic

Signs

• pale white atrophic areas affecting the vulva,
• purpura (ecchymosis) is common,
• Fissuring,
• Erosion (blistering is rare)
• hyperkeratosis,
•  loss of architecture- loss of labial minora, midline fusion, clitoris may be buried under clitoral hood

Complications

• Squamous cell carcinoma <5%
• Clitoral Pseudocyst
• Sexual dysfunction
• Dysaesthesia

Diagnosis

• Has a characteristic clinical appearance
• Histology of vulval biopsy: thinned epidermis with sub-epidermal hyalinization and deeper inflammatory infiltrate
• Histology can be difficult in early disease

Management

• Biopsy is mandatory if diagnosis is uncertain or VIN or squamous cell carcinoma is suspect
• Do Thyroid function test
• Skin swab is useful to exclude co-existing infection
• Patch testing should be done only if medication allergy is suspected (Discuss with dermatologist)

Treatment

Give general advice on condition

• Inform about small risk of neoplastic change
• Advise to inform doctor if they notice changes in appearance of vulva lesions or if symptoms change

Medication

Ultrapotent topical steroids e.g. Clobetasol propionate (Dermovate)

• Use daily for 1month
• Then alternate days for 1 month
• Then Twice weekly for 1month
• Then as required depending on symptoms
• 30grams should last 3 months

Alternative regimen

• Clobetasol with neomycin and nystatin if secondary infection is suspected

Unlicensed treatments

• Topical calcineurin inhibitors (Tacrolimus 0.1%)-not to be used as first line- Cases of malignancy have been reported
• Oral retinoid (Acitretin)-Only to be used by dermatologist experienced with this agent. It is severely teratogenic. Pregnancy must be avoided for 2 years after completing treatment
• UVA1 phototherapy

Pregnancy and breastfeeding

• Topical steroids are safe
• Topical calcineurin inhibitors are contraindicated
• Retinoids are absolutely contraindicated

Referrals

• Non responders to routine steroid treatment should be referred to specialised vulva clinic.
• Patients who develop VIN or squamous cell carcinoma should be referred to a specialised vulva clinic
• Surgery should only be used in patients with coexisting VIN, SCC or fusion
• Disease tend to reoccur around the scar

Follow up

• See after 3 months to assess response to initial treatment
• Review stable disease annually

4.Lichen Planus

• Lichen planus is an inflammatory disorder with manifestations on the skin, genital and oral mucous membrane
• Pathogenesis is unknown
• Sometimes linked to Lichen sclerosis
• More rarely it affects the lacrimal duct, oesophagus and external auditory meatus

Clinical features

Symptoms

• Itch, irritation, Soreness, Dyspareunia, Urinary symptoms, Vaginal discharge
• Can be asymptomatic

Signs-Anogenital lesions can be divided into 3

• Classical-Typical papules found on keratinised anogenital skin with or without striae on the inner aspect of vulvae
• Hypertrophic- Affects perineum and perianal area, presents as thickened warty plaque which may become ulcerated and painful. They are rare and can mimic malignancy
• Erosive- The lesions consist of friable telangiectasia with patchy erythema which are responsible for the common symptoms of postcoital bleeding, dyspareunia and a variable discharge which is often serosanguinous. Important to recognise early as can lead to scarring and stenosis

Complications

• Scarring, including vaginal synechiae,
• Squamous cell carcinoma around 3%

Diagnosis

• Characteristic clinical appearance, involvement of vagina excludes lichen sclerosus
• Histology of vulva biopsy

Management

• Biopsy is important if diagnosis is uncertain or there is suspicion of small cell carcinoma or vulva intraepithelial neoplasia
• Do Thyroid function test
• Skin swab to exclude secondary infection
• Patch testing if allergy to medication is suspected

Treatment

• Inform patient of small risk of neoplastic changes
• Offer ultrapotent steroid clobetasol propionate
• Can give clobetasol propionate with neomycin and nystatin
• Refer to multidisciplinary specialist vulva clinic in erosive disease
• In the vulvovaginal–gingival syndrome- Oral ciclosporin may be considered
• Retinoids can be very helpful in hypertrophic cases
• Oral steroids-prednisolone 40 mg/day, tapered off over a few weeks; for severe flares.
• Basiliximab is effective in patients with oral and cutaneous disease use with caution and monitoring by dermatologist with experience in its use

Follow up

• 2-3months to assess response to initial treatment
• Then follow up annually
• Patient should inform doctor of changes in appearance

5.Lichen simplex

Classified into 4 groups

Underlying dermatoses	atopic dermatitis, allergic contact dermatitis, superficial fungal (tinea and candidiasis) infections
Systemic conditions causing pruritus	renal failure, obstructive biliary disease (primary biliary cirrhosis and primary sclerosing cholangitis), Hodgkin’s lymphoma, hyper- or hypothyroidism, and polycythaemia rubra vera
Environmental factors	heat, sweat, rubbing of clothing, and other irritants such as harsh skincare products
Psychiatric disorders	anxiety, depression, obsessive-compulsive disorder, and dissociative experiences are often associated with the condition. Emotional tensions in predisposed people (i.e., those with an underlying predisposition for atopic dermatitis, asthma, and allergic rhinitis) can induce itch and thus begin the chronic itch-scratch cycle

 

Clinical Features

 Symptoms

• Vulval itch and Soreness

Signs

• Lichenification i.e. thickened, slightly scaly, pale or earthy-coloured skin with accentuated markings, maybe more marked on the side opposite the dominant hand.
• Erosions and fissuring.
• Excoriations as a result of scratching may be seen
• The pubic hair is often lost in the area of scratching

Complications

• Secondary infection

Management

• Do swab to rule out infection e.g. candida and staphylococcus aureus
• Check Ferritin
• Patch test
• Biopsy

Treatment

• Use emollient soap
• Avoid triggers
• Topical corticosteroid e.g. clobetasol propionate or betamethasone for limited period
• anxiolytic antihistamine such as hydroxyzine or doxepin
• Cognitive behavioural therapy if there is mental health issue
• Follow up severe disease 1 month after treatment then as required

6.Vulval Psoriasis

Aetiology

• Psoriasis is a chronic inflammatory epidermal skin disease affecting approximately 2% of the general population.
• Genital psoriasis may present as part of plaque or flexural psoriasis or, rarely, as the only area affected.

Clinical Features

Symptoms

Vulval itch, Soreness, Burning sensation

Signs

• Well demarcated brightly erythematous plaques often symmetrical
• Frequently affects natal cleft
• Usually lacks scaling due to maceration
• Fissuring

Complications

• May be worsened due to Koebner effect by irritation from urine, tightfitting clothes or sexual intercourse.

 Management

• Skin punch biopsy if the diagnosis is in doubt

Treatment

• Avoidance of irritating factors
• Use of emollient soap substitute
• Topical corticosteroid – weak to moderate steroids are preferred but if insufficient to induce a response then intensive short-term potent steroid such as clobetasol propionate 0.05% may be used. A combined preparation containing antifungal and/or antibiotic may be required if secondary infection suspected e.g. Trimovate
• Coal-tar preparations – may be used alone or combined or alternated with topical steroids
• Vitamin D analogues such as Talcalcitol – alone or in combination with corticosteroid
• Referral to a multidisciplinary vulval clinic is recommended for unresponsive or recalcitrant cases, or those in whom systemic therapy is considered.
• Follow-up Mild disease – as clinically required, Severe disease – (i.e. when using potent topical steroids) 1 month then as required.

7.Vulva Eczema

Aetiology Eczematous and lichenified diseases, as classified by ISSVD includes:

• Atopic: the ‘allergic’ type often seen in people who also have hay fever or asthma.
• Allergic contact: due to skin contact to a substance to which the individual is sensitive.
• Irritant contact: due to skin contact with irritating chemicals, powders, cleaning agents, etc.

Clinical Features

• Symptoms: Vulval itch, Soreness
• Signs: Erythema, Lichenification and excoriation, Fissuring
• Complications: Secondary infection

 Diagnosis: Clinical presentation (as above).

• General examination of the skin to look for other signs of dermatitis

Management

• Further Investigation, Patch testing standard battery and medication
• Biopsy – only if atypical features or failure to respond to treatment

Treatment

• Avoid precipitating factor
• Use of emollient soap substitute (aqueous cream should not be applied as a moisturiser due to the risk of irritant effects,
•  Hydramol can be a suitable alternative
•  Topical corticosteroid – the choice of preparation will depend on severity, 1% Hydrocortisone ointment in milder cases, or betamethasone valerate 0.025% or clobetasol propionate 0.05% for limited periods if severe or lichenified.
•   A combined preparation containing antifungal and/or antibiotic may be required if secondary infection suspected. Apply once daily.

Follow-up -As clinically required

• Long-term follow up and psychological support may be needed

References

BASHH 2014 UK national Guideline on the Management of Vulval conditions