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Int J STD AIDS OnlineFirst, published on June 4, 2014 as doi:10.1177/0956462414538419

Audit report

The management of lichen sclerosus in a genito-urinary medicine setting: a 12-month retrospective case-notes review

International Journal of STD & AIDS 0(0) 1–5 ! The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0956462414538419 std.sagepub.com

Rachel Challenor

Abstract A retrospective case-notes review was undertaken of all women with lichen sclerosus seen during a 12-month period to review their characteristics and care. A total of 273 case-notes were reviewed. The mean age was 61 years (range, 14– 94), and the mean duration of symptoms was 6.4 years (range, 1–50). The mean age at diagnosis was 55 years (range, 7– 92). Sixty-two (23%) had at least one other autoimmune condition. Autoimmune conditions were seven times more frequent overall compared with United Kingdom prevalences. On-going symptoms were reported as none/minimal in 196 (72%), moderate in 65 (24%) and severe in 12 (4%). A total of 233 women (85%) had on-going treatment with clobestasol propionate (Dermovate) ointment with a mode of eight applications per month (range, 0–30). Forty-three women (16%) reported sexual dysfunction and 13 (5%) had needed at least one surgical procedure to restore sexual function. Eighty-six (32%) had undergone at least one biopsy. Nine squamous cell cancers (3%) had been diagnosed in six women (2%). These patients were managed in line with all current guidance. It is surprising that there is still no evidence to direct long-term management.

Keywords Lichen sclerosus, autoimmune, case-notes review, vulval cancer Date received: 5 April 2014; accepted: 9 May 2014

Introduction In 2014, the British Association for Sexual Health and HIV (BASHH) produced an updated United Kingdom (UK) National Guideline on the Management of Vulval Conditions.1 This prompted a decision to review the characteristics and care of the cohort of women with lichen sclerosus (LS) attending our clinic, which meant that there was a need to reconsider the other guidelines already in place for the management of LS, namely those produced by the British Society of Dermatologists (BAD)2 and the Royal College of Obstetricians and Gynaecologists (RCOG).3

Methods This was a retrospective case-notes review of all women seen by the author over a 12-month period, from 1 December 2012 to 30 November 2013. Information was extracted from the notes regarding demographics, symptoms both at diagnosis and on-going, co-existing auto-immune conditions, treatment, follow-up

intervals, sexual dysfunction and malignant change. Data were analysed in Excel.

Results A total of 273 women with LS were seen in the 12-month period. The mean age was 61 years (range, 14–94), and the mean duration of symptoms was 6.4 years (range, 1–50). The mean age at diagnosis was 55 years (range, 7–92). Figure 1 shows the age distribution at diagnosis. Fifty-two percent was diagnosed between the ages of 51–70 years. Figure 2 shows the presenting symptoms at diagnosis. The majority (91%) had either itch or itch associated with soreness. One hundred and twelve (41%)

GUM Department, Derriford Hospital, Plymouth, UK Corresponding author: Rachel Challenor, GUM Department, Derriford Hospital, Plymouth, PL6 8DH, UK. Email: [email protected]

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80 70 60 50 40 30 20 10 0 0-10y

11-20y

21-30y

31-40y

41-50y

51-60y

61-70y

71-80y

81y onwards

Figure 1. Age distribution at diagnosis of the LS cohort.

Table 1. Spread of AI conditions in the LS cohort compared with UK prevalences.

itch/itch & sore

Other AI conditions

Number

%

UK prevalence (%)

Thyroid underactive Thyroid overactivea Alopecia Diabetes (insulin) Vitiligo Pernicious anaemia Women with at least one AI condition

54 2a 3 3 4 4 62

20 1a 1 1 2 2 23

2 0.1 a 0.2 0.5 0.5 0.2 3

sore

white skin

sex dysfunction

none 0

50

100

150

200

250

300

Figure 2. Presenting symptoms at diagnosis of the LS cohort.

had perianal involvement. Only five (2%) were documented as having extra-genital disease. Sixty-two (23%) had at least one other autoimmune (AI) condition. Five had two other AI conditions, and two women had three other AI conditions. The majority (20%) had underactive thyroid dysfunction and were taking thyroxine. AI conditions were seven times more frequent overall in the LS cohort4 compared with UK prevalences with a range of 2–10 times more frequent.5–9 Table 1 shows the spread of AI conditions in the LS cohort compared with UK prevalences. Figures for overactive thyroid dysfunction are in italics as they represent incidence rather than prevalence. Two women were taking carbimazole at the most recent visit. Some of the other 54 women currently taking thyroxine may have originally had an overactive thyroid, which subsequently became underactive, but that could not be determined from the notes. Two hundred and forty-two women (89%) had standard induction treatment with clobetasol propionate (Dermovate) ointment. Four women, who had been

a Figures for overactive thyroid dysfunction are in italics as they represent incidence rather than prevalence. AI: autoimmune: LS: lichen sclerosus.

diagnosed in the early 1990s, were given 2% testosterone in yellow soft paraffin as their initial treatment. The remainder, 27 women, were referred by their General Practitioner to the vulval clinic already using a variety of other moderately potent/ultra potent topical steroid preparations and had experienced amelioration or complete resolution of their symptoms. Table 2 shows on-going symptoms, frequency of follow-up, on-going treatments, reported sexual dysfunction, past surgical procedures and frequency of malignant change in the LS cohort. Eighty-six women have had at least one biopsy. Of these, 80 had an initial diagnostic biopsy: 78 had histological confirmation of LS – the other two had classic LS clinically, but the biopsies showed, respectively, chronic inflammation in one and lichen planus in the other. For the six who did not have an initial biopsy,

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Table 2. On-going symptoms, frequency of follow-up, on-going treatments, reported sexual dysfunction, past surgical procedures and frequency of malignant change in the LS cohort. Parameter On-going symptoms

Follow-up

On-going treatments

Sexual dysfunction Surgical procedures

Biopsy Cancers

None/minimal Moderate Severe 12 months/if required 4–6 months 1–3 months Clobetasol propionate ointment Applications per month Ointment preparations Cream preparations No treatment Total reporting Total procedures (to restore sexual function in 13 women) Repeat surgery Division of adhesions Modified z-plasty Fenton’s For diagnosis and/or to exclude malignant change Total cancers diagnosed Total women with cancers

Number

%

196 65 12 140 104 28 233 Mode ¼ 8; range, 0–30 233 33 7 43 16 3 3 12 1 86 9 6

72 24 4 52 38 10 85

85 12 3 16 6 1 1 4 0.4 32 3 2

LS: lichen sclerosus.

five had classic LS clinically, which responded to ultrapotent steroid treatment. In one, the diagnosis was unclear, but was confirmed on the deferred biopsy. The other five biopsies were undertaken to exclude carcinoma/to diagnose new pigmentation/to diagnose hyperkeratotic areas. In two LS, only was confirmed. The other three had biopsies showing benign melanosis, wart and wart, respectively. Sixteen have had at least one repeat biopsy – eight have had one more biopsy, three have had another two biopsies, four have had another three and one has had another four biopsies. These have all been undertaken to diagnose or exclude carcinoma/vulval intra-epithelial neoplasia (VIN) because of hyperkeratosis, raised warty lesions, erosions and/or hyperpigmentation. Table 3 gives information regarding the duration of presenting symptoms of the nine squamous cell cancers diagnosed in six women and information on subsequent treatment and follow-up. Thirty-four women were newly diagnosed in 2013. Of these, 100% had both oral and written information on general care of the vulva with the use of emollients and the avoidance of sensitizers plus information about their diagnosis of LS.

Discussion Guidelines say that complete remission occurs. The BAD says remission occurs in approximately 60%.2 The RCOG says that it occurs in 50–90%,3 and BASHH1 does not put a figure to it. In this cohort, a total of 72% had none or only minimal symptoms, which is very much in keeping with the guidance. One hundred and twelve (41%) had perianal involvement. The BAD says that perianal lesions occur in women in 30%2 whereas it is rarely, if ever, seen in male patients.2 A study of 350 women in 1988 showed that the perianal skin was involved in 44%.10 However, only five (2%) were documented as having extra-genital disease. This is much lower than the 10%, which has been reported.10–12 The difference is more likely to be a failure of documentation rather than a true reflection of the prevalence of extra-genital LS in this cohort (as the quality of data in a retrospective audit is often a reflection of the quality of record keeping). Fifty-two percent had annual follow-up or only if needed, and mostly frequency of follow-up was related to severity of symptoms. However, there was a

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1944

1959

5

6

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SCC detected by surveillance biopsy

Long-standing itch and soreness

Ulcer few weeks

Long-standing itch and soreness – defaulted follow-up

Asymptomatic SCC and VIN detected by surveillance biopsy

Ulcer few weeks

Long-standing itch and soreness

Symptoms

2011 left para clitoral area – invasive SCC with high grade VIN and HPV negative – left sentinel node biopsy clear

2003 invasive SCC left hemi-vulvectomy and left node dissection – 10 nodes all clear

March 2011 excision biopsy-differentiated VIN with features of early invasion

Well differentiated SCC and VIN 2

Wide local excision and bilateral node sampling – nodes all clear March 2013 partial vulvectomy

First cancer 2002– moderately differentiated SCC and VIN

June 2009 excision biopsy Grade 1 A vulval SCC with non-HPV VIN

Vulval SCC

On-going itch and soreness

VIN recurrence – excised from upper right vulva On-going itch and soreness VIN detected by surveillance biopsy

Asymptomatic

Itch

2004 second SCC right anterior vulvectomy with clitoris and right lymph node dissection – 8 nodes all clear

Low-grade differentiated VIN Gynae MDT decision to use imiquimodVIN cleared September 2013 wide excision and sentinel node biopsy clear plus DXT

Second ulcer for 2 weeks

Long-standing itch and soreness – defaulted followup

Asymptomatic VIN detected by surveillance biopsy

Anal pain

2013 VIN 2 excised – no SCC

2006 anal cancer abdomino-perineal resection

Three monthly at GUM

Three monthly at GUM

Gynae cancer team follow-up

Three monthly at GUM

Four monthly at GUM

Second cancer 2005 well differentiated SCC wide excision biopsy

Follow-up

Second ulcer few weeks

Cancer/VIN recurrence

Six monthly at GUM

Symptoms

Recurrent itch

Symptoms

Vulval SCC/VIN recurrence

4

Biopsy LS in 1995

Biopsy LS in 1991

Biopsy LS in 2007

Biopsy LS in 1991

Biopsy LS in 1987

Biopsy LS in 2001

Diagnosis of LS

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DXT: radiotherapy; GUM: genitourinary medicine; SCC: squamous cell cancer; VIN: vulval intraepithelial neoplasia.

1958

2

4

1929

1

1966

1937

Patient

3

Year of birth

Table 3. Information regarding nine cancers diagnosed in six patients with LS.

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mismatch, where some with minimal symptoms were being seen too often. One of the lessons learned from this review has been to work actively towards discharge back to primary care13 or 12-month follow-up for all those with stable disease and to ensure information is given to every woman about when to seek prompt medical advice. Nine squamous cell cancers (3%) had been diagnosed in six women (2%). The guidelines1–3 say that the lifetime risk of developing invasive cancer in women with LS is of the order of 2–4%, exactly as seen in this cohort (whereas the lifetime risk of developing vulval cancer in women in the UK is 1 in 293 or 0.3%).14 Five women remain at very high risk of malignant change. Three have had two separate primary vulval cancers already, and two women have had one cancer followed by the appearance of VIN. These women are reviewed every 3 months. Interestingly, two of the cancers and each of the two VIN occurrences were detected by surveillance and not by the patients re-attending during the interval between appointments. As a result of this extensive case-note review, lessons have been learned, and practice has changed by: . Being more proactive in asking about sexual dysfunction; . Assessing more consistently the quantity of ultra potent steroid being used; . Re-considering the use of topical calcineurin inhibitors (as per guidelines although there are concerns about the development of malignancy with calcineurin inhibitors, and therefore these agents should not be used as first line).1 . Actively discharging back to primary care or extending the review interval to 12 months for all women with stable disease and ensuring every woman understands when to seek prompt medical review. In conclusion, these patients were managed in line with all current guidance.1–3 It is surprising that there is still no evidence to direct long-term management. Conflict of interest The author declares that there is no conflict of interest.

Ethical Approval This was a case note review and no ethical approval was required.

Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

References 1. http://www.bashh.org/documents/UK%20national%20 guideline%20for%20the%20management%20of%20 vulval%20conditions%202014.pdf (accessed 30 March 2014) 2014 UK National Guideline on the Management of Vulval Conditions (accessed 30 March 2014). 2. Neill SM, Lewis FM, Tatnall FM, et al. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol 2010; 163: 672–682. 3. http://www.rcog.org.uk/womens-health/clinical-guidance/ management-vulval-skin-disorders-green-top-58 (accessed 30 March 2014) The Management of Vulval Skin Disorders Green-top Guideline 58. 4. http://www.rightdiagnosis.com/a/ai/stats-country.htm (accessed 30 March 2014) Statistics by country for autoimmune disease. 5. http://www.british-thyroid-association.org/info-for-patients/ (accessed 30 March 2014) 6. http://www.patient.co.uk/doctor/Alopecia.htm (accessed 30 March 2014). 7. https://www.diabetes.org.uk/Documents/Reports/Diabetesin-the-UK-2012.pdf (accessed 30 March 2014) Diabetes in the UK 2012. 8. http://cks.nice.org.uk/vitiligo#!backgroundsub:1 (accessed 30 March 2014) Prevalence of Vitiligo. 9. http://www.nhs.uk/conditions/anaemia-vitamin-b12-andfolate-deficiency/Pages/Introduction.aspx (accessed 30 March 2014) Anaemia, vitamin B12 or folate deficiency. 10. Thomas RHM, Ridley CM, McGibbon DH, et al. Lichen sclerosus et atrophicus and autoimmunity – a study of 350 women. Br J Dermatol 1988; 118: 41–46. 11. http://www.uptodate.com/contents/extragenital-lichensclerosus (accessed 25 April 2014). 12. http://dermnetnz.org/immune/lichen-sclerosus.html (accessed 25 April 2014). 13. http://www.bssvd.org/images/stories/documents/VULVAL% 20LICHEN%20SCLEROSUS%20fu%20guidance%20 for%20gps.pdf (accessed 3 April 2014). 14. http://www.patient.co.uk/doctor/vulval-cancer (accessed 30 March 2014).

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The management of lichen sclerosus in a genitourinary medicine setting: a 12-month retrospective case-notes review.

A retrospective case-notes review was undertaken of all women with lichen sclerosus seen during a 12-month period to review their characteristics and ...
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