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

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 clobetasol 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 sexual health women

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 Association 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 1.

Age distribution at diagnosis of the LS cohort.

Figure 2 shows the presenting symptoms at diagnosis. The majority (91%) had either itch or itch associated with soreness. One hundred and twelve (41%) had perianal involvement. Only five (2%) were documented as having extra-genital disease.

Figure 2.

Presenting symptoms at diagnosis of the LS cohort.

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.

Table 1.

Spread of autoimmune conditions in the LS cohort compared with UK prevalences.

Other AI conditions	Number	%	UK prevalence (%)
Thyroid underactive	54	20	2
Thyroid overactive ^a	2 ^a	1 ^a	0.1 ^a
Alopecia	3	1	0.2
Diabetes (insulin)	3	1	0.5
Vitiligo	4	2	0.5
Pernicious anaemia	4	2	0.2
Women with at least one AI condition	62	23	3

Figures for overactive thyroid dysfunction are in italics as they represent incidence rather than prevalence.

AI: autoimmune: LS: lichen sclerosus.

Two hundred and forty-two women (89%) had standard induction treatment with clobetasol propionate (Dermovate) ointment. Four women, who had been 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.

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		Number	%
On-going symptoms	None/minimal	196	72
	Moderate	65	24
	Severe	12	4
Follow-up	12 months/if required	140	52
	4–6 months	104	38
	1–3 months	28	10
On-going treatments	Clobetasol propionate ointment	233	85
	Applications per month	Mode = 8; range, 0–30
	Ointment preparations	233	85
	Cream preparations	33	12
	No treatment	7	3
Sexual dysfunction	Total reporting	43	16
Surgical procedures	Total procedures (to restore sexual function in 13 women)	16	6
	Repeat surgery	3	1
	Division of adhesions	3	1
	Modified z-plasty	12	4
	Fenton’s	1	0.4
Biopsy	For diagnosis and/or to exclude malignant change	86	32
Cancers	Total cancers diagnosed	9	3
Cancers	Total women with cancers	6	2

LS: lichen sclerosus.

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, five had classic LS clinically, which responded to ultra-potent 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 patients, 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.

Table 3.

Information regarding nine cancers diagnosed in six patients with LS.

Patient	Year of birth	Diagnosis of LS	Symptoms	Vulval SCC	Symptoms	Vulval SCC/VIN recurrence	Symptoms	Cancer/VIN recurrence	Follow-up
1	1937	Biopsy LS in 2001	Long-standing itch and soreness	June 2009 excision biopsy Grade 1 A vulval SCC with non-HPV VIN	Recurrent itch				Six-monthly at GUM
2	1929	Biopsy LS in 1987	Ulcer few weeks	First cancer 2002– moderately differentiated SCC and VIN	Second ulcer few weeks	Second cancer 2005 well differentiated SCC wide excision biopsy	Anal pain	2006 anal cancer abdomino-perineal resection	Four-monthly at GUM
2	1929	Biopsy LS in 1987	Ulcer few weeks	Wide local excision and bilateral node sampling – nodes all clear	Second ulcer few weeks		Anal pain	2006 anal cancer abdomino-perineal resection	Four-monthly at GUM
3	1966	Biopsy LS in 1991	Asymptomatic SCC and VIN detected by surveillance biopsy	March 2013 partial vulvectomy	Asymptomatic VIN detected by surveillance biopsy	Low-grade differentiated VIN. Gynae MDT decision to use imiquimod-VIN cleared	Itch		Three-monthly at GUM
3	1966	Biopsy LS in 1991	Asymptomatic SCC and VIN detected by surveillance biopsy	Well differentiated SCC and VIN 2	Asymptomatic VIN detected by surveillance biopsy		Itch		Three-monthly at GUM
4	1958	Biopsy LS in 2007	Long-standing itch and soreness – defaulted follow-up	March 2011 excision biopsy-differentiated VIN with features of early invasion	Long-standing itch and soreness – defaulted follow-up	September 2013 wide excision and sentinel node biopsy clear plus DXT			Gynae cancer team follow-up
5	1944	Biopsy LS in 1991	Ulcer few weeks	2003 invasive SCC left hemi-vulvectomy and left node dissection – 10 nodes all clear	Second ulcer for 2 weeks	2004 second SCC right anterior vulvectomy with clitoris and right lymph node dissection – 8 nodes all clear	Asymptomatic	2013 VIN 2 excised – no SCC	Three-monthly at GUM
6	1959	Biopsy LS in 1995	Long-standing itch and soreness	2011 left para clitoral area – invasive SCC with high grade VIN and HPV negative – left sentinel node biopsy clear	On-going itch and soreness	VIN recurrence – excised from upper right vulva	On-going itch and soreness		Three-monthly at GUM
6	1959	Biopsy LS in 1995	SCC detected by surveillance biopsy		VIN detected by surveillance biopsy	VIN recurrence – excised from upper right vulva	On-going itch and soreness		Three-monthly at GUM

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

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 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 guidelines^1–3 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.

Footnotes

Ethical Approval

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

Declaration of Conflicting Interests

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The authors received no financial support for the research, authorship, and/or publication of this article.

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