Vulval Lichen Planus



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Aetiology

Using the latest ISSVD terminology, there are two premalignant vulvar lesions, which can lead to a squamous cell carcinoma of the vulva, namely HSIL and DVIN. These are completely different entities with respect to aetiology, malignant potential and treatment. HSIL is caused by a persistent infection with high risk Human Papilloma Virus (HPV). The incidence of HSIL is approximately 5 per 100.000 women per year and is increasing [130], with the highest peak between 35-49 years [131]. A reason for the increased incidence may be the increase of anogenital HPV infections and/or a better diagnosis by the more liberal use of vulvar biopsy. Risk factors are smoking and an immuno-compromised state.

DVIN is associated with lichen sclerosus and lichen planus and has no relation with HPV. DVIN occurs mainly in elderly women, and comprises less than 5% of VIN lesions. The malignant potential of DVIN is higher than that of HSIL [132,133]. The aetiology of DVIN is not clear.
Symptoms and signs





HSIL

DVIN

Symptoms

Itching, burning, irritation

Pain


Psychosexual complaints

Asymptomatic
Symptoms are often due to the underlying lichen sclerosus or lichen planus


Signs


Clinical appearance is very variable

Plaques, whitish, erythematous, or pigmented

Multifocal


Difficult to distinguish from lichen sclerosus lesions

Grey-white or red lesion

Roughened surface or ulceration

More often unifocal than HSIL


Complications

HSIL and DVIN

  • Development of vulvar squamous cell carcinoma

  • High rate of recurrence after treatment

  • Psychosexual complaints


Diagnosis

HSIL and DVIN is often a multifocal disease. It is important to take a biopsy of all lesions (mapping).


Investigation

HSIL

Biopsy


  • Histopathological characteristics: disorganisation of squamaous epithelium, cytological atypia,

high nuclear/cytoplasmic ratio, mitotic figures
DVIN

Biopsy: histopathology is difficult



  • Histopathological characteristics: hyperplasia, hyperkeratosis, parakeratosis, elongation and anastomosis of rete ridges, basal cell atypia, prominent nucleoli, atypical mitosis in basal layer, dyskeratosis, hypermaturation of rete ridges


Management

HSIL

Surgical treatment has been the first choice of treatment, but recurrence rates are high and there is a negative effect on quality of life and sexual function. A new treatment modality is the application of imiquimod cream, an immune response modifier with indirect antiviral and antitumour properties [134].



  • Surgical cold knife excision

  • Laser CO2 therapy

  • Loop electrosurgical procedure (LEEP)

  • Imiquimod cream

  • Follow up without treatment (spontaneous regression)


DVIN

  • Surgical cold knife excision


Follow up

Close follow-up is mandatory, life long



HSIL

  • every 6-12 months, with annual cervical smear


DVIN

  • depends on underlying disease, but at least every 6 months


Vaccination

Several types of therapeutic HPV vaccines have been developed showing different rates of clinical success. Today, therapeutic vaccines are not yet available for routine clinical use.


Prophylactic HPV vaccination was introduced in 2007 with the goal of reducing the incidence of cervical (pre)malignancies and to reduce other HPV related lesions like HSIL. The quadrivalent HPV 6/11/16/18 vaccine shows prevention against HPV 16 and 18 related high grade lesions of vulva and vagina, in women who were HPV 16 or 18 negative before vaccination [135].

Auditable Outcome Measures

Patients should be given a written explanation of their condition: Target 100%

Biopsy should be performed in patients not responding to an initial course of treatment and /or if raised lesions develop: Target 100%


Websites with useful patient information

British Association of Dermatologists



http://www.bad.org.uk/shared/get-file.ashx?id=180&itemtype=document

http://www.bad.org.uk/shared/get-file.ashx?id=113&itemtype=document

International Society for the Study of Vulvo-vaginal Disease



http://issvd.org/wordpress/wp-content/uploads/2014/09/ContactDermatitis-2013-final.pdf

British Contact Dermatitis Society Patient Information on specific allergens



http://www.cutaneousallergy.org/downloads/patient/standard/

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Appendix 1



Levels of Evidence

Ia. Evidence obtained from meta-analysis of randomised controlled trials.

Ib. Evidence obtained from at least one randomised controlled trial.

IIa. Evidence obtained from at least one well designed study without randomisation.

IIb. Evidence obtained from at least one other type of well designed quasi-experimental study.

III. Evidence obtained from well designed non experimental descriptive studies such as comparative studies, correlation studies, and case control studies.

IV. Evidence obtained from expert committee reports or opinions and/or clinical experience of respected authorities.
Grading of Recommendations

A (Evidence levels Ia, Ib)

Requires at least one randomised control trial as part of the body of literature of over all good quality and consistency addressing the specific recommendation.

B (Evidence levels IIa, IIb, III)

Requires availability of well conducted clinical studies but no randomised clinical trials on the topic of recommendation.

C (Evidence IV)

Requires evidence from expert committee reports or opinions and/or clinical experience of respected authorities. Indicates absence of directly applicable studies of good quality.

Appendices:

Search strategy:

Searched libraries: MEDLINE, MEDLINE process, Embase, Cochrane library. Sexually transmitted diseases guidelines produced by the British Association for Sexual Health and HIV (www.bashh.org) were also reviewed.

Search up to March 2015 with no date limitation. The search strategy comprised the following terms in the title or abstract: Vulval lichen sclerosus, Vulval lichen planus, Vulval eczema, Vulval lichen simplex, Vulval psoriasis, Vulval intraepithelial neoplasia, Vulval pain syndromes/vulvodynia.


European STI Guidelines Editorial Board

Dr Keith Radcliffe, UK – Editor-in-Chief

Dr Marco Cusini, Italy (appointed 2010)

Dr Gilbert Donders, Belgium (app. 2012)

Prof Mikhail Gomberg, Russia (app. 2010)

Dr Michel Janier, France (app. 2006)

Dr Jorgen Skov Jensen, Denmark (app. 2006)

Prof Harald Moi, Norway (app. 2007)

Dr Raj Patel, UK (app. 2006)

Prof Jonathan Ross, UK (app. 2006)

Dr Jackie Sherrard, UK (app. 2009)

Dr Magnus Unemo, Sweden (app. 2009)

Dr Willem van der Meijden, Netherlands (app. 2006)

Dr Andrew Winter, UK, (app.

Dr Deniz Gökengin, Turkey, (app.

Dr Simon Barton (UK) – UEMS representative, UK (app. 2010)

Dr Lali Khotenashvili – WHO European Office representative, Georgia (app. 2007)

Prof Mario Poljak – ESCMID representative, Slovenia (app. 2013)

Prof George-Sorin Tiplica, - EADV representative, Romania (app. 2012) - EDF representative (app. 2012)

Gianfranco Spiteri – ECDC representative, Sweden, (app.



List of contributing organisations

This guideline has been produced on behalf of the following organisations: the European Branch of the International Union against Sexually Transmitted Infections (IUSTI Europe); the European Academy of Dermatology and Venereology (EADV); the European Dermatology Forum (EDF); the Union of European Medical Specialists (UEMS). The European Centre for Disease Prevention and Control (ECDC) and the European Office of the World Health Organisation (WHO-Europe) also contributed to its development.



Declarations of Interest

None declared




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