Dr. Leslie Ann Sadownik (biography, no disclosures)
Lichen sclerosus (LS) is a chronic skin disorder with a remitting and relapsing clinical course. Women commonly present with severe vulvar itch and an urge to scratch the skin. The recommended treatment is a course of topical steroids. Most women will improve with treatment. However, some will report, “the steroids did not help”.
Janet is a 53-year-old woman who presents with a 2-year history of distressing vulvar itch. At the beginning she thought she had yeast. She tried over the counter anti-yeast and anti-itch medications. The itch became so bad at night that she had trouble sleeping. She and her partner have not been able to have sex for over a year because it is too uncomfortable. She recently saw her family doctor who noticed whitening of her vulvar skin and suspected lichen sclerosus. She tried using a steroid cream. While, it seemed to help initially as soon as she stopped, the itch returned. She wonders if there is a treatment that will cure her vulvar itch?
Data that answers these questions
It is helpful to approach patients with “unresponsive” lichen sclerosus by asking yourself a series of questions.
Question 1: Is the clinical diagnosis correct?
The diagnosis is usually clinical. LS causes the affected skin to become atrophic (white, thin, crinkled). There is often a symmetrical “figure of 8” inflamed and/or atrophic skin pattern around the vulva and anus. Sometimes, the skin changes are isolated to the clitoris, perineum, or perianal areas (versus the whole vulva). Women with vulvar LS rarely have LS elsewhere on the body.
The fragile skin may crack or tear. Erosions, fissures, purpura and ecchymoses are common. The tears cause discomfort during urination, bowel movements or sex. Early anatomical changes include: loss of the inter-labial folds, flattening, and/or loss of the labia minora. Advanced changes include: clitoral phimosis and labia adhesions resulting in introital stenosis. LS does not involve the skin above the hymenal ring (vagina or cervix).
A skin biopsy may confirm the diagnosis if the pathology reports the classic histological features of LS (thin epidermis, loss of rete ridges, hyperkeratosis and a band-like lymphocytic inflammatory infiltrate). However, patient may still have clinical disease even if the biopsy reports non-specific changes – a “normal” biopsy does not rule out Lichen Sclerosus.
The differential diagnosis for LS includes: irritant/contact dermatitis, lichen planus, lichen simplex chronicus, vitiligo, mucous membrane pemphigoid, psoriasis, vulvar intra-epithelial neoplasia, paget’s disease, and or urogenital atrophy. There is an overlap between the clinical presentation of LS and lichen planus (LP). Both conditions can cause vulvar itch, whitening of the vulvar skin, and progressive anatomical changes. LP is more difficult to treat and often involves the vestibular and vaginal skin resulting in scarring and or adhesions in the vagina. Women with vulvovaginal LP often have LP elsewhere on the body. For example, in the oral cavity one can find Wickham’s striae, inflammation (gingivitis), erosions and or ulcers.
Question 2: Is the treatment appropriate?
The standard therapy is a course of a super-potent (eg. Clobetasol) or potent (eg. Mometasone Fourate) topical steroid. Moderate or mild potency steroids are preferred for pregnant woman.1 There is no evidence to support hormonal therapy (eg. Testosterone).
Question 3: Are the treatment goals appropriate?
The first goal is to reduce the itch. This should be achieved within weeks of starting therapy. The second goal is to improve the integrity of the skin. Fissures and erosions should heal: patients should be able to resume daily and sexual activities. The whitening of the skin may persist in some individuals but the skin texture should improve. Resolution of all whitening is not an explicit goal of therapy. The third goal is to preserve the vulvar architecture and prevent further changes. Topical therapy will not correct significant anatomical changes.
Question 4: Is the patient adherent to treatment recommendations?
Lee et al. reported that less than 2/3 of patients (67%) comply with therapy recommendations.2 Factors that may affect patient adherence to topical steroids include: non aesthetic nature of the topical medication (i.e. greasy), time-consuming and complicated regimens, ambiguous dosing instructions (i.e “apply sparingly”), side effects, misbeliefs about the disease and treatment regimen, and cost and affordability. These factors should be explored at each follow up visit.
For women with severe disease (inflammation, erosions, severe symptoms) a follow up visit at 2-4 weeks after staring therapy is recommended. For most women, the first follow up visit can be 2-3 months after starting therapy. Ask the woman to bring her medication to this appointment. Review the amount of medication used over the time period. It is recommended that patients with a “figure of 8” involvement of the genitilia use 1 Fingertip Unit (FTU) per application. One FTU is the amount of ointment expressed from a tube with a 5 mm nozzle, applied from the distal skin crease of the index finger to the tip- approximately 0.4 g. Thus, a 30-g jar will usually last three months of acute treatment – see Table 1 at the bottom of the article for an example of a treatment regiment.
If the patient appears to be using more or less of the medication, review her application technique. She should apply the medication directly with her fingertip (not a cotton swab) and spread in a film over the vulvar (medial labia majora/inter-labial folds, both sides of the labia minora, and the perineum) and, if involved, perianal skin. As the skin condition often involves changes in the entire vulvar area, it is easier to have patients get into a routine of systematically treating this area rather than only treating “white” or “symptomatic” areas. Simply wash hands with soap and water after application of the medication. In general, ointments should be prescribed initially (they are more potent and contain less potential irritants). Patients may be subsequently switched to creams if they prefer a cream base.
If the disease is stable over time, reduce the potency of the prescribed steroid (from potent to medium to low) at subsequent follow up visits. The majority of women will relapse if they reduce the frequency of the steroid application to less than twice per week or completely stop treatment. Since most patients stop therapy intermittently, patients need explicit instructions on how to manage flare-ups. “Restart daily application for 1-2 weeks until symptoms resolve. If symptoms do not resolve, and or increase, stop the medication and see a doctor. You should not be on daily therapy for more than 4 weeks. Once symptoms improve go back to regular 2-3 times per week application.”
Explore what dosing regimen is most convenient for patients. For example, when starting note that once daily application of steroid (am or pm) is as effective as twice daily. For “twice weekly” maintenance, a simple routine is to use medication on weekends and then use emollients on weekdays. Give the patient realistic guidelines on how much medication to use over time – a 30 gram jar will last 3 months of initial treatment and 6 months of maintenance treatment.
Patients should be educated that the skin disease, LS, is thinning the skin – the topical steroid is in fact stopping that process, and when applied correctly will not thin the vulvar skin. Care, of course, should be taken to avoid spreading the steroid to unaffected nearby skin (eg. thighs). Most women are disappointed to hear that LS cannot be cured. Women should be reassured that that regular use of topical steroid medication will result in better symptom control and potentially reduce the risk of squamous cell carcinoma.3 If used appropriately, long term use of topical super-potent or potent steroid is also safe and does not cause steroid induced atrophy or increased risk of HPV/HSV or candidiasis infections.4
Question 5: Is there a secondary diagnosis?
A secondary diagnosis is common. Consider any and all of the following: irritant/contact dermatitis, superinfection (candidiasis, HSV, bacterial), high grade squamous dysplasia/cancer, hypoestrogenism, steroid induced atrophy or rebound steroid dermatitis, vulvodynia. Review the patient’s daily skin care routine. Many women continue to use potential irritants (eg. bar soap, panty liners), or engage in harmful behavior (eg. frequent washing of the skin). Patients may be allergic to a component of the topical steroid. It may be helpful to discontinue all topical medications for 1 month and then re-assess. For patients who suffer from recurrent: candidiasis, herpes simplex virus or urinary tract infections reduce the potency of the steroid, and or add on prophylactic therapy (e.g. antiviral coverage). Consider VIN or cancer for persistent erosions, fissures, ulcers or plaques – biopsy any persistent skin lesions. Many women will develop vulvar LS in the menopausal years. If women are reporting persistent dryness, burning and dyspareunia consider adding local vaginal estrogen therapy. If there is objective improvement but patients report unchanged symptoms consider a diagnosis of vulvodynia.
Question 6: Is there an alternative treatment for this patient?
Alternative treatments include; higher potency topical steroids, intralesional/intramuscular steroids, and or topical calcineurin inhibitors. Topical tacrolimus 0.1% ointment can be an effective treatment for steroid non-responsive LS. The medication is costly and patients often report significant burning upon application. The standard dose is 0.1% applied BID for 6 weeks however I have found that reducing the dose and/or frequency of application is often better tolerated by patients starting therapy, for example, tacrolimus 0.03% applied daily and then increased as tolerated. Less commonly reported treatments for LS include: topical and systemic retinoids, phototherapy and photodynamic therapy. Current evidence is weak for the use of: adipose-derived stem cells, platelet rich plasma, or laser as treatment for vulvar LS and should not be recommended at this time.5,6 Surgical intervention is limited to excising pre-malignant/malignant lesions and/or correct anatomical defects. A referral to a specialist in vulvar skin disorders is often warranted when a patient, despite adherence to standard topical steroid therapy, has persistent symptoms and or signs of LS.
What I recommend (practice tip)
Take Home Message: Most treatment “failures” of vulvar lichen sclerosus are due to patients using an inadequate amount of topical steroid medication (“applying sparingly”) on an irregular basis (“only when I need it”). Many women with chronic vulvar diseases will have a secondary diagnosis that is contributing to persistent symptoms.
Frequently Asked Questions and Answers:
- Is a skin biopsy necessary to diagnose Lichen Sclerosus? No, the diagnosis is usually clinical; but early on in the disease the findings may be very subtle. Women must be off all topical steroids for 3 weeks prior to taking a skin biopsy.
- How long do you need to follow women with Lichen Sclerosus? Once the condition is stable annual follow up is recommended.
- What is the risk of squamous cell carcinoma? The Incidence of squamous cell carcinoma in vulvar lichen sclerosus is estimated to be between 0.3-4.9%.3
- Chi C, Wang S, Kirtschig G. Safety of topical corticosteroids in pregnancy. JAMA Dermatol. 2016;152(8):934-935. DOI: 10.1001/jamadermatol.2016.1009. (Request with CPSBC or view with UBC)
- Lee A, Bradford J, Fischer G. Long-term management of adult vulvar lichen sclerosus: a prospective cohort study of 507 women. JAMA Dermatol. 2015;151(10):1061-1067. DOI: 10.1001/jamadermatol.2015.0643. (Request with CPSBC or view with UBC)
- Fistarol SK, Itin PH. Diagnosis and treatment of lichen sclerosus: an update. Am J Clin Dermatol. 2013;14(1):27-47. DOI: 10.1007/s40257-012-0006-4. (View)
- Kai A, Lewis F. Long-term use of an ultrapotent topical steroid for the treatment of vulval lichen sclerosus is safe. J Obstet Gynaecol. 2016;36(2):276-277. DOI: 10.3109/01443615.2015.1049252 (Request with CPSBC or view with UBC)
- Eshtiaghi P. Fact or fiction? Adipose-derived stem cells and platelet-rich plasma for the treatment of vulvar lichen sclerosus. J Low Genit Tract Dis. 2019;23(1):65-70. DOI: 10.1097/LGT.0000000000000440. (View with CPSBC or UBC)
- Digesu GA. The energy based devices for vaginal “rejuvenation,” urinary incontinence, vaginal cosmetic procedures, and other vulvo-vaginal disorders: An international multidisciplinary expert panel opinion. Neurourol Urodyn. 2019. DOI: 10.1002/nau.23927. (Request with CPSBC or view with UBC)
Handout for patients: http://bcvulvarhealth.ca/wp-content/uploads/2017/06/bccvh_lichen_sclerosus.pdf
|Example of a
for lichen sclerosus
|You have been prescribed: topical mometasone fourate 0.1%|
Once at day (morning or night), apply a thin layer of the ointment to the
affected skin areas, not forgetting the skin around the anus if this is involved.
|Second month:||Apply on alternate nights. Use an emollient on days “off”.|
Apply twice a week (eg. Mondays and Thursdays or on weekends Saturdays & Sundays).
Follow up with your doctor after you have finished 3 months of treatment and then once per year.