How to deal with lichen sclerosus Presented at the FIGO world congress, on behalf of the ISSVD Rome, 2012

1. 9 October 2012
PedroVieira Baptista
Portugal

2. No conflicts of interest to declare

3. PVBaptistaPVBaptista
 Lichen
"(…) symbiotic relationship between the
photosynthetic (green alga or
cyanobacterium; photobiont) and fungal
(mycobiont) partnership (…)”
 Lichenification
"(…) classical term used to describe a
thickening of the skin and a more
pronounced reticulate (…)”
“(…) is characterized clinically by a
palpable thickening of the tissue and
increased prominence of skin markings.
Scale may or may not be detectable (…)”
Esteves J, Baptista P et al, Tratado de Dermatologia, 1992
Lynch PJ, Moyal-Barracco M, Scurry J, Stockdale C. Dermatological
Disorders: An Approach to Clinical Diagnosis, JLGTD, 16,(4), 2012
Piercey-Normore MD, Deduke C. Fungal farmers or algal escorts: lichen
adaptation from the algal perspective. Mol Ecol. 2011 Sep; 20(18):3708-10

4.  Lichen sclerosus
 ISSVD 2006
 Lichenoid pattern or
 Dermal homogenization/sclerosis pattern
 ISSVD 2011
 White lesions (4) with patches and plaques (B)
 Older terms:
 Kraurosis vulvae (1885)
 Leucoplakia (1897)
 Vulvar dystrophy
 Lichen sclerosus et atrophicus
 Etc.
Introduction
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5.  Bimodal distribution
 Pre-pubertal
 Post-menopausal
Epidemiology
0
5
10
15
20
25
30
35
40
Symptoms Diagnosis
Mean age±SD (years)
Symptoms 53±16,3
Diagnosis 59±15,3
n=226
Vieira-Baptista P, 2012, unpublished data

6.  Incidence
 Male:Female 1:6-10
 1:70-1.000 (Friedrich EG Jr , 1976, Wallace HJ, 1971 Burrows LJ et al, 2010)
 1:30 in older women (Jones RW at al, 2008)
 Geographical variation?
 Rare inAfrica vs underdiagnosed
 Vulvar cancer less frequent in Asia than in Europe
 Probably due to less frequency of LS rather than HPV infection
 Africans, Caribbeans and Asians underrepresented among LS patient in
a London clinic
Epidemiology
Jacyk WK, Isaac F. Lichen Sclerosus et Atrophicus in Nigerians. Jour Nat Med Assoc, 71 (4),
1979
MacLean AB, Chan M, Ramos K. Why isThere a GeographicVariation in Lichen
Sclerosus andVulval Cancer? XXIWorld Condgress of the ISSVD, Paris 2011
Real prevalence unknown!
Up to 50% assymptomatic

7.  A role for alimentary factors?
 25% of symptomatic patients referred worsening with
specific food
Epidemiology
n %
Worsening with food
Pork
Acidic fruit
Fried food
Spicy food
Vegetables
Others
43
22
14
13
11
10
6
25%
51%
33%
30%
26%
23%
14%
Vieira-Baptista P, 2012, unpublished data

8.  Symptoms
Presentation
n=192
n=3415%
8%
13%
28%
30%
75%
0 20 40 60 80 100 120 140 160 180
Asymptomatic
Discharge
Pain
Dysuria
Burning
Pruritus
Vieira-Baptista P, 2012, unpublished data

9.  Signs
Presentation
PVBaptistaPVBaptistaPVBaptista
4%
7%
10%
12%
13%
15%
17%
19%
23%
23%
33%
34%
42%
47%
65%
75%
0 20 40 60 80 100 120 140 160 180
Pseudocysts
Isomorfic phenomenon
Ecchymosis
Oedema
Hyperpigmentation
Erosions
Stenosis
Perianal
Erythema
Figure of 8
Fissures
Hyperqueratosis
Cigarette paper
Hypopigmentation
Phymosis/paraphymosis
Atrophy
Vieira-Baptista P, 2012, unpublished data

10. Autoimmunedisease
Birembaum
DL et al, 2007
Simpkin S et
al, 2007
Cooper SM et al,
2008
Vieira Baptista P,
unpublished, 2012
Autoimmune disease - - 28,4% ** 23%
Thyroid disease 29,9% - 16,3% ** 25%
Alopecia areata - - 2,6% 4%
Vitiligo - - 10,5% 1%
Psoriasis - 17% - 5%
Rheumatoid arthritis - - 1,5% 4%
Crohn’s disease - - - 2%
Lupus - - - 1%
Auto-antibodies - - 21% -
Antithyroid - - 9% 25%
** Statistically significant

11.  Autoimmune disease prevalence in the family 21-
56%
 Higher than in LS patients!
 Data supports the screening for autoimmune
disease, specially thyroid disease.
Autoimmunedisease
Harrington CI, Dunsmore IR. An investigation into the incidence of auto-immune disorders in
patients with lichen sclerosus and atrophicus. Br J Dermatol May 1981;104(5):563-6
Powell J Wojnarowska FWinsey S et al. Lichen sclerosus premenarche: autoimmunity and
immunogenetics. Br J Dermatol 2000 Mar; 142:481-4

12. Sexual (dys)function
n=226
With intercourse:
125
With
dyspareunia: 64
Without
dyspareunia: 59
Apareunia: 2
Without
intercourse: 73
Unknown:
28
63% have intercourse
51% of these with dyspareunia
<2% witn apareunia
Vieira-Baptista P, 2012, unpublished data

13. Diagnosis
Clinical history Examination DIAGNOSIS+
?
Biopsy
Symptoms
Medication
Worsening factors
Associated conditions
Family history
Vulvar examination
Speculum examination
Skin, nails, mouth

14.  When to perform it?
 Differentiate LS from lichen planus
 Thickened epidermis/erosions/ulcerations/erythema (Jones RW et al, 2004)
 Lack of response to the treatment
 Rule outVIN/carcinoma
 Where to perform it?
 Transition from affected to normal skin
 Areas of ecchymosis or fine crinkling
 Pitfalls of biopsy
 Treatment with topical steroids changes the typical histological appearance
 Biopsy taken in wrong places
 Insufficient clinical data given to the pathologist
 Pathologist without experience
Biopsy

15.  LS associated with vulvar squamous cell carcinoma
 5% risk – possibly overestimated
 LS increases the 246-300x the relative risk of SCC
 Extra genital lesions not associate with malignancy
 60% of vulvar SCC develop in a background of LS
 Incidence of vulvar cancer rising in the last years
 Weaker association with:
 Melanoma (Friedman RJ et al, 1984)
 Basal cell carcinoma(MeyrickThomas RH et al, 1985)
 Verrucous carcinoma (Brisgotti M et al, 1989)
LSandmalignancy
Renaud-Vilmer C, Cavelier-Balloy, B, Porcher R.Vulvar Lichen Sclerosus: Effect of Long-termTopical
Application of a Potent Steroid on the Course of the Disease. Arch Dermatol,Vol 140, June 2004
MacLean AB; Jones RW, Scurry J, Neill S.Vulvar Cancer and the Need for Awareness of Precursor
Lesions.J LowGenitTract Dis. 2009 Apr;13(2):115-7

16. LSandmalignancy
Differentiated VIN
Usual typeVIN
VULVAR CANCER
HPV
Lichen sclerosus
WalkdenV, ChiaY, Wojnarowska F. The association of squamous cell carcinoma and lichen sclerosus;
implications for follow up. J Obstet Gynecol 1997; 17: 551–3
Vilmer C, Cavelier-Balloy B, Nogues C et al. Analysis of alterations adjacent to invasive vulvar cancer and
their relationship with the associated carcinoma: a study of 67 cases. Eur J GynecolOncol 1998; 19: 25–31.
60%
40%

17.  Objectives:
1. Stop/control symptoms
2. Prevent anatomic distortion
3. Avoid progression to malignancy?
Management

18.  Intimate hygiene
 Water
 Reduce frequency
 Clothes
 Skirts or loose pants
 Natural fabrics
 Underpants
 White
 Natural fabrics
 Not to use it at night
 Tampons rather than sanitary napkins
 Avoid panty liners
 Induce amenorrhea
 Control/correct urinary incontinence and
other irritant factors
 Avoid scratching
Management
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19.  There is no consensus on the schemes to be used in
terms of duration or frequency
 Testosterone, dihydrotestosterone and progesterone –
without interest!
 Ultrapotent topical corticosteroid are the first choice
 Cobetasol propionate 0,05%
 Betamethasone valerate 0,1%
 Ointment rather than cream
 Less sensitizing
 More occlusive
 Better tolerated if fissures or erosions
Management
Chi CC, Kirtschig G, Baldo M, Brackenbury F, Lewis F, Wojnarowska F. Topical interventions for genital lichen
sclerosus.Cochrane Database Syst Rev. 2011 Dec 7;(12):CD008240

20.  Initial management:
 30 g of ointment should be enough
 Maintenance therapy:
 Ultrapotent corticosteroids
 Lowest dose that controls symptoms
 No more than 2-3 times/week
 Spend less than 60 g of ointment/year (Edwards L et al, 2011; Neill SM et al, 2002)
 Lower potency corticosteroids
Management
Twice a weekEveryother dayEvery day
Month 3Month 2Month 1

21.  Calcineurin inhibitors (pimecrolimus, tacrolimus)
 Pimecrolimus vs clobetasol
 Identical efficacy in the control of itching and burning
 Less efficacy in terms of improvement of the skin
 Less efficacy controlling inflammation
 No difference in terms of adverse effects
 Second line treatment
 Non-responders to corticosteroids
 Intolerance to corticosteroids
 Reactivation of HPV and HSV infections?
 Carcinogenesis?
Management
Goldstein AT, Creasey A, Pfau R, Phillips D, Burrows LJ. A double-blind, randomized controlled trial
of clobetasol versus pimecrolimus in patients with vulvar lichen sclerosus. J Am Acad Dermatol. 2011 Jun;64(6):e99-104

22.  Visits
 Ideally, first visit should be one month after starting treatment (no lately than
3 months)
 Visit at 6 months after first scheme of treatment
 Visits at least once a year
 Vulvar cancer rare in diagnosed and treated patients with LS
 Progression fromVIN to cancer occurs very quickly
 Ideally, patients should be assessed every 3 months!
Management
• Response to treatment
• Correct application of the treatment
• Superimposed infection
• Corticosteroid atrophy
• Identify suspect lesions

23.  Others
 Antihistamines (control itch, sedative effect)
 Antidepressants/antiepileptic drugs (pain)
 Topical anaesthetics (ex. lidocaine)
 Topical estrogens
 Lubricants and emollients
 Treat secondary infections (mostly fungal infections)
Management

24.  What to do with asymptomatic patients?
 Some of them might have inactive disease (active
disease in childhood?)
 If hyperkeratosis, ecchymosis , fissures or progressive
atrophy should be treated
Management
Neill SM,Tatnall FM, Cox NA. Guidelines for the management of lichen
sclerosus. British Journal of Dermatology 2002; 147: 640–649

25.  Other options
 Oral retinoids (Bousema MT et al, 1994)
 Triamcinolone (subcutaneous/ointment) (Mazdisnian F et al, 1999; LeFevre C, 2011)
 Cryotherapy (Kastner U et al, 2003)
 Photodynamic therapy/Psoralen plus UVA (Kroft EB et al, 2008)
 Laser (AynaudO et al, 2010)
 Potassium para-aminobenzoate (Penneys NS et al, 1984)
 Antimalarials (Wakelin SH et al, 1994)
 Antibiotics (Shelley WB et al, 2006)
Management

26.  Who should manage these patients?
 Primary care?
 Simple cases
 Gynaecologist/Dermatologist with training in vulvar disease
1. Patients requiring potent corticosteroids more than 3x/week
2. Patients requiring more than 30 g of corticosteroids ointment
in 6 months
3. VIN
4. (Hyperkeratosis or thickened skin – for biopsy)
Management
MacLean AB, Jones RW, Scurry J, Neill S. Vulvar Cancer and the Need for Awareness of Precursor
Lesions. Journal of Lower GenitalTract Disease,Volume 13, Number 2, 2009, 115Y117

27. Management
 Surgery
 Benign conditions
 Pseudocysts: total or subtotal circumcision
 Phymosis/paraphymosis: circumcision; hydrodissection
 Vulvar synecheae: lyses
 Stenosis: perineotomy, vulvoperineoplasty
 Improvement of dyspareunia in 90% of cases
 Malignant and pre-malignant conditions
Rouzier R et al. Perineoplasty for the treatment of introital stenosis related to
vulvar lichen sclerosus. Am J Obstet Gynecol. 2002 Jan;186(1):49-52
GoldsteinAT et al. Surgical treatment of clitoral phimosis caused by
lichen sclerosus. Am J ObstetGynecol. 2007 Feb;196(2):126.e1-4

28.  Surgery - hydrodissection
Management
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Phymosis with apareunia

29.  Treatment failure?
1. Non-compliance
2. Correct diagnosis, but associated /superimposed
conditions
 Candidosis, contact dermatitis, psoriasis, etc.
3. Secondary sensory condition
4. Anatomical distortion
Management