BASHH Guidelines

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Vulval lichen planus

(1) Aetiology and natural history

Lichen planus is an inflammatory disorder with manifestations on the skin, genital and oral mucous membranes. More rarely it affects the lacrimal duct, oesophagus and external auditory meatus.  It is an inflammatory condition of unknown pathogenesis.  In some cases, there is overlap between LS and lichen planus.

(2) Clinical features

Symptoms: Itch/irritation,soreness, dyspareunia, urinary symptoms, vaginal discharge . Can be asymptomatic.

Signs: The anogenital lesions of lichen planus maybe divided into three main groups according to their clinical presentation: 

  • Classical: typical papules will be found on the keratinised anogenital skin, with or without striae on the inner aspect of the vulva. Hyperpigmentation frequently follows.  May be asymptomatic.
  • Hypertrophic: Thickened warty plaques  in the perineum and perianal area which may become ulcerated, infected and painful. They  are relatively rare and can be difficult to diagnose. Can mimic malignancy. 
  • Erosive: the most common subtype to cause vulval symptoms. The mucosal surfaces are eroded. At the edges of the erosions, the epithelium is mauve/grey and a pale network (Wickham’s striae) is sometimes seen. 
    Can lead to scarring and complete stenosis. 

Complications: Scarring, including vaginal synaechia. Development of SCC (up to 3%). 

(3) Diagnosis 

Characteristic clinical appearance and histopathology of vulval biopsy

(4) Management

  • Biopsy: is a necessity if the diagnosis is uncertain or coexistent VIN/SCC is suspected. 
  • Investigation for autoimmune disease especially of the thyroid (i.e. T4 and TSH if there is any suspicion of abnormality) (IV, C)
  • Skin swab: to exclude secondary infection especially of excoriated lesions as clinically indicated. 
  • Patch testing: if secondary medicament allergy or contact dermatitis suspected.
  • Treatment: 
    • General advice . Patients should be informed about the condition and given information (written or web-based). Patients should be made aware of the small risk of neoplastic change. They should be advised to contact the doctor if they notice a change in appearance or texture (e.g. ulcer, lump or hardening of skin).
    • Recommended regimen: Ultra-potent topical steroids, e.g. Clobetasol proprionate (Iib, B).  Maintenance treatment may be required and can either be with weaker steroid preparations or less frequent use of potent steroids. .
    • Vaginal corticosteroids: A proprietary preparation containing hydrocortisone (Colifoam), introduced with an applicator, is useful. Prednisolone suppositories may be used in more severe cases (IV, C). 
    • Alternative regimens: An ultra-potent topical steroid with antibacterial and antifungal, e.g. Dermovate NN or generic equivalent (Clobetasol with neomycin and nystatin) or an alternative preparation that combats secondary infection (such as Fucibet cream) may be appropriate if secondary infection is a concern. These should only be used for a short period of time to clear infection (IV, C). 
  • Onward referral critieria: Referral to a multidisciplinary vulval clinic is recommended for erosive disease and any recalcitrant cases, or those in whom systemic therapy is considered. 
  • Systemic treatments: There is no consensus and there is little evidence base for the use of systemic agents. Oral ciclosporin, retinoids, oral steroids and new biological agents may be used but all are potentially toxic and need careful monitoring. They are best supervised by a dermatologist in the context of a specialised clinic (IV, C).
  • Follow-up:
    • At 2–3 months to assess response to treatment. 
    • Erosive lichen planus needs long-term specialised follow-up (IV, C). . Stable disease should be reviewed annually except in well-counselled patients who control their symptoms well. 
    • If review is by the GP this should be communicated to the patient and GP by the clinic. 
    • Patients should be informed that if they notice the development of a lump or change in appearance they should seek medical advice urgently.

Vulval eczema

(1) Aetiology and natural history

  • 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.

(2) Clinical features

  • Symptoms: Vulval itch and soreness.
  • Signs: Erythema, lichenification and excoriation. Fissuring, pallor or hyperpigmentation.
  • Complications: Secondary infection.

(3) Diagnosis 

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

(4) Management

  • Further investigation:
    • Patch testing – standard battery and medicaments (III, B). 
    • Biopsy (IV, C) – only if atypical features (e.g. asymmetric, localised or eroded) or failure to respond to treatment.
  • Treatment:
    • Avoidance of precipitating factor (IV, C). . 
    • 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 for short-term use (to try and avoid the development of contact allergy) if secondary infection suspected. Apply once daily (IV, C).
  • Follow-up: As clinically required. Long-term follow-up and psychological support may be needed.

Vulval lichen simplex

(1) Aetiology and natural history

  • Underlying dermatoses, i.e. atopic dermatitis, allergic contact dermatitis, superficial fungal (tinea and candidiasis) infections.  
  • Systemic conditions causing pruritus, i.e. 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.

(2) Clinical features

  • Symptoms: Vulval itch and soreness.
  • Signs: Lichenification, 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.

(3) Diagnosis

  • Clinical presentation.  
  • History including mental state examination where indicated.  
  • General examination of the skin to look for other signs of psoriasis or lichen simplex elsewhere.

(4) Management

  • Further investigation:
    • Screening for infection (e.g. Staphylococcus aureus, Candida albicans). . Dermatological referral for consideration of patch testing – standard battery and medicaments (III, B). 
    • Ferritin (III, B).
    • Biopsy (IV, C)
  • Treatment: 
    • Avoidance of precipitating factor (IV, C). 
    • Use of emollient soap (some people may have a reaction to aqueous cream when it is used as an emollient. For this reason, it is recommended only as a soap substitute and not an emollient). . 
    • Topical corticosteroid – potent topical steroids are required when treating lichenified areas, e.g. betamethasone or clobetasol for limited periods. A combined preparation containing antifungal and/or antibiotic may be required if secondary infection was suspected. Apply once or twice daily.(IV, C). A graduated reduction in the frequency of application of the topical steroid is helpful, over about 3–4 months
    • A mildly anxiolytic antihistamine such as hydroxyzine or doxepin at night is helpful. 
    • Cognitive behavioural therapy may be helpful if there are co-existing mental health issues.
  • Follow-up: 
    • Mild disease – as clinically required. 
    • Severe disease (i.e. when using potent topical steroids) – one month then as required.

Vulval psoriasis

(1) Aetiology and natural history

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.

(2) Clinical features

  • Symptoms: vulval itch, soreness and burning sensation.
  • Signs: Well-demarcated brightly erythematous plaques, often symmetrical, frequently affects natal cleft. Usually lacks scaling due to maceration. Fissuring. Involvement of other sites, e.g. scalp, umbilicus.
  • Complications: May be worsened due to Koebner effect by irritation from urine, tight-fitting clothes or sexual intercourse.

(3) Diagnosis  

  • Clinical presentation. 
  • General examination of the skin and nails to look for other signs of psoriasis.

(4) Management

  • Further investigation: 
    • 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 steroids such as clobetasol propionate 0.05% may be used. A combined preparation containing antifungal and/or antibiotic may be required if secondary infection was suspected (e.g. Trimovate). This should be for short-term use only to try and avoid the development of contact allergy (IV, C).  
    • Weak coal-tar preparations – may be used alone or combined or alternated with topical steroids. However, these preparations can cause irritation and folliculitis (IV, C).  
    • Vitamin D analogues such as Talcalcitol – alone or in combination with corticosteroid; however, their usefulness may be limited by causing irritation (IV, C).
  • Onward referral: Referral to a multidisciplinary vulval clinic is recommended for unresponsive or recalcitrant cases, or those in whom systemic therapy is considered.  
  • Systemic treatments: if required for severe and extensive psoriasis may help genital lesions but not recommended for isolated genital psoriasis.
  • Follow-up: 
    • Mild disease – as clinically required.
    • Severe disease – (i.e. when using potent topical steroids) one month then as required

Vulval intraepithelial neoplasia

(1) Aetiology

This is a vulval skin condition which may become cancerous if left untreated. It is confirmed by histological diagnosis and can be defined as low grade or high grade. Low-grade change is usually associated with human papillomavirus (HPV) and may resolve.  A second type, generally not HPV related, occurs in conjunction with LS or lichen planus (known as differentiated type). The risk of progression to SCC is much greater.

(2) Clinical features

  • Symptoms: lumps, erosions, burning and itch/irritation, pain or may be asymptomatic.
  • Signs: Clinical appearance is very 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.
  • Complications: Development of SCC, Recurrence is common and progression to cancer can occur following previous treatment.  Psychosexual consequences have also been described (especially following surgical treatment).

(3) Diagnosis

  • Biopsy. Multiple biopsies may be required as there is a risk of missing invasive disease.  

(4) Management

  • Further investigation: 
    • Ensure that cervical cytology remains up-to-date – there is an association with cervical intraepithelial neoplasia (CIN)) (IV, C). 
    • All patients with VIN should be referred for up-todate colposcopy to exclude CIN and VIN. 
    • For perianal lesions, referral for anoscopy is recommended (IV, C).
  • Treatment:
    • Local excision (III,B)
    • Imiquimod cream 5%. (not licensed in pregnancy).This is an unlicensed indication. (Ib, A)
    • Vulvectomy .  Recurrence may occur and function and cosmesis will be impaired (IV, C).
  • Alternative regimens:
    • Local destruction.  A variety of techniques have been evaluated, including carbon dioxide laser and ultrasonic surgical aspiration, photodynamic therapy, cryotherapy and laser. (IIa, B). The recurrence rates at follow-up tend to be higher than for excision, but cosmesis is usually good.
    • 5 fluorouracil cream – may lead to resolution of some lesions but the results are variable and side effects are common. (not licensed in pregnancy).This is an unlicensed indication (IV, C).
    • Supervision – some lesions will spontaneously regress.  There is  a risk of progression (IV, C).
  • Onward referral: Cases of VIN should be assessed in a multidisciplinary vulval clinic, or have input from gynaecology regarding assessment for surgical excision.
  • Follow-up: Close follow-up is mandatory. Although resolution may occur, VIN III particularly has a significant rate of progression.

Vulval pain

(A) Localised provoked vulvodynia (vestibulodynia)

(1) Aetiology and natural history

Likely to be multifactorial, a history of vulvovaginal candidiasis, usually recurrent, is the most commonly reported feature. 

(2) Clinical features 

  • Symptoms: Vulval pain – frequently felt at the introitus at penetration during sexual intercourse or on insertion of tampons. There is usually a long history.
  • Signs: Focal tenderness elicited by gentle application of a cotton wool tip bud at the introitus or around the clitoris. 
  • Complications: Sexual dysfunction and psychological morbidity.

(3) Diagnosis

Clinical diagnosis made on history and examination.

(4) Management

  • Further investigation:   After exclusion of other treatable causes no further investigation is required. Biopsy should be performed if there is any suspicion of an underlying dermatosis.
  • Treatment:
    • Avoidance of irritating factors.  
    • Use of emollient soap substitute. 
    • Topical local anaesthetics, e.g. 5% lidocaine ointment or 2% lidocaine gel should be used with caution as irritation may be caused. The application should be made 15–20 min prior to penetrative sex and washing off the lidocaine just before sex or the use of condom by the partner can reduce the risk of transfer resulting in penile numbness. Oral contact should be avoided (IV, C).  
    • Physical therapies. Pelvic floor muscle biofeedback(III, B), vaginal transcutaneous electrical nerve stimulation (Ib, A), vaginal trainers (III, B), cognitive behaviour therapy (III, C)  
    • Psychosexual counselling.
  • Alternative regimens: 
    • Pain modifiers – the benefit of drugs such as tricyclic antidepressants, gabapentin and pregabalin is not clear. Amitriptyline may be beneficial in some women (IV, C). 
    • Surgery – modified vestibulectomy may be considered in cases where other measures have been unsuccessful. Patients who have responded to topical lidocaine prior to sex have a better outcome (III, B).
  • Follow-up:  As clinically required. Long-term follow-up and psychological support may be needed.

 

(B) Unprovoked vulvodynia

(1) Aetiology and natural history

The aetiology is unknown and the condition is best managed as a chronic pain syndrome.

(2) Clinical features

  • Symptoms: Pain that is longstanding and unexplained. May be associated with urinary symptoms such as interstitial cystitis. Can also be associated with irritable bowel syndrome and fibromyalgia.
  • Signs: The vulva appears normal.
  • Complications: Sexual dysfunction and psychological morbidity.

(3) Diagnosis

Clinical diagnosis made on history and examination having excluded other causes

(4) Management

  • Further investigation: After exclusion of other treatable causes no further investigation is required.  Biopsy should be performed if any suspicion of alternative diagnosis.
  • Treatment:
    • Use of emollient soap substitute.
    • Pain modifiers – tricyclic antidepressants are well established in chronic pain management. Few studies have specifically examined the effect in vulvodynia; however, amitriptyline is frequently first-line treatment; dosage should be increased by small increments starting at 10 mg up to 100 mg daily according to the patient’s response (III, B). Note: a recent randomised study has not confirmed the beneficial effect of amitriptyline (Ib, A)
    • If unresponsive or unable to tolerate the side effects, gabapentin (III, B) or pregabalin may be used (IIb, B).
  • Alternative regimens: 
    • Topical local anaesthetic, e.g. 5% lidocaine ointment or 2% lidocaine gel. Irritation is a common side effect (IV, C). 
    • Cognitive behavioural therapy and psychotherapy (IIb, B). 
    • Acupuncture (IIb, C).  
    • Physiotherapy if evidence of a weak pelvic floor.
    • Treatment-resistant unprovoked vulvodynia may require referral to a pain clinic.
  • Follow-up: As clinically required.

 

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