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Vulvar Lichen Sclerosus: Diagnosis and Management
Vulvar Lichen Sclerosus: Diagnosis and Management Nicholas Wedel, BN, MN (NP), and Laura Johnson, RN (NP), MN ABSTRACT
Vulvar lichen sclerosus (VLS) is a chronic, inflammatory skin condition of the vulva that most commonly affects postmenopausal women. Although there is currently no cure, modern treatments can effectively manage VLS once diagnosed. Unfortunately, vulvar conditions are routinely underdiagnosed and undertreated. Untreated VLS can cause significant physical, emotional, and sexual discomfort, which may result in irreversible destruction of the genitalia and can progress to squamous cell carcinoma. Nurse practitioners must be aware of the key features and complications of this condition and must have a good understanding of the diagnostic criteria and treatment strategies for VLS. Keywords: lichen sclerosus, nurse practitioner, squamous cell carcinoma, topical ultrapotent corticosteroids, vulvar biopsy, vulvar conditions Ó 2014 Elsevier, Inc. All rights reserved.
T
he topic of women’s health is currently receiving a great deal of attention in the media, among social groups, and by society as a whole. However, vulvar health is 1 aspect of women’s health that is often not discussed. It is estimated that up to 20% of all women will experience significant vulvar symptoms at some point in their lifetime.1 Still, conditions such as vulvar lichen sclerosus (VLS) are often underdiagnosed and undertreated.2,3 Admittedly, vulvar health may be a difficult topic for patients and practitioners to discuss. However when left untreated, VLS can cause significant physical, emotional, and sexual discomfort and may result in irreversible destruction of the genitalia and the development of squamous cell carcinoma (SCC). In order to minimize complications and improve patient’s quality of life, nurse practitioners (NPs) working in women’s health and in primary care settings must have the skills to recognize and manage conditions such as VLS. This article outlines the key features of VLS and provides the information NPs need to accurately identify, diagnose, and manage this condition. EPIDEMIOLOGY Definition
Lichen sclerosus (LS) is a chronic, inflammatory skin condition that can affect men, women, and children 42
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on any area of the body but most commonly affects the anogenital region of adult women.4 Although there is currently no cure for LS, the condition can be effectively managed when it is recognized and diagnosed appropriately. Prevalence
The true prevalence of VLS is difficult to determine because it often goes unrecognized and is never diagnosed. The most consistent estimates indicate that between 1 in 70 and 1 in 1000 women are affected by VLS, rates that are 10 times higher than those among men.1 The bimodal peaks of LS occur during prepuberty and postmenopause, with an increase to 1 in 30 among postmenopausal women.1,5 Higgins and Cruickshank3 indicated that at least 25% of women seen in dedicated vulva clinics received treatment for VLS. Etiology
Although the definite cause of LS is not currently known, experts have identified strong associations between LS and autoimmune diseases, genetic factors, and low levels of endogenous sex hormones.3,5 Thorstensen and Birenbaum6 found that up to 20% of women with VLS have coexisting autoimmune diseases, particularly thyroid disease, vitiligo, and alopecia areata.4 Other Volume 10, Issue 1, January 2014
Figure 1. Vulvar lichen sclerosus.
research studies have indicated that 44% to 74% of women with LS have tested positive for circulating immunoglobulin autoantibodies.5,6 High rates of VLS among first-degree relatives supports the theory that a genetic component exists in this condition.3 Up to 12% of patients with VLS have a positive family history of the disease.6 Low endogenous estrogen levels have also been linked with the development of VLS. This association is supported by the substantially higher incidence of VLS among postmenopausal women; however, researchers have not yet identified any consistent change in symptoms during menses, pregnancy, or with the use of oral contraceptive medications or hormone replacement therapies.7 KEY FEATURES Symptoms
Patients with VLS may experience an insidious or aggressive onset of symptoms, or they may remain www.npjournal.org
Figure 2. Vulvar lichen sclerosus.
asymptomatic for long periods of time. Vulvar pruritus is the hallmark symptom of VLS and may be mild and intermittent or intense and constant.6 The condition causes skin changes called vulvar fissures, which may occur spontaneously or through physical irritation from scratching and/or sexual intercourse.4,6 Vulvar fissures and subsequent scarring frequently result in dysuria, dyspareunia, pain with defecation, and rectal bleeding.4,6 Examination
VLS causes characteristic color changes to the skin of the vulva. The typical blanched, shiny white, crinkled tissue paper appearance can be present from the mons pubis to the perineal region and is characteristically distributed in a figure-of-8 or keyhole pattern over the labia and perineum (Figures 1 and 2).6,8 Skin changes occur externally only; the vagina and other internal structures are not affected by VLS. Flat, shiny white plaques surrounded by red-, purple-, or violet-colored borders are indicative of The Journal for Nurse Practitioners - JNP
43
Table. Differential Diagnoses for Vulvar Lichen Sclerosus 1. 2. 3. 4. 5.
Vulvar lichen sclerosus Candida vulvovaginitis Squamous cell carcinoma Vulvar intraepithelial neoplasia Genital tract inflammation a. Contact dermatitis b. Atrophic vaginitis c. Vestibular vulvodynia
atrophy and scarring caused by longstanding untreated VLS. Structural changes such as loss of the labia minora, flattening of the labia majora, inversion and trapping of the clitoris, and severe constriction of the introitus occur when VLS is left untreated or is treated ineffectively.2,6,7 Secondary skin lesions are usually present and may include vulvar edema, erythema, excoriation, and fissures. Concurrent infections can increase inflammation to the point of bullae formation. Deep erosions are not typically caused by VLS unless the condition is accompanied by significant scratching or in the presence of vulvar intraepithelial neoplasia (VIN) or SCC.6
Vaginal Swabs
Although vaginal swabs are not routinely required for the diagnosis of VLS, they may be useful in ruling out concurrent conditions, such as bacterial vaginosis, trichomoniasis, candida vulvovaginitis, herpes simplex virus, and other sexually transmitted infections. Vaginal swabs are primarily collected in the presence of abnormal discharge, vulvar erythema, fissuring, or excoriation.6,10 Signs and symptoms such as labial swelling, erythema, and thick white discharge are often easily identified as candida vulvovaginitis. However, practitioners must remain cognizant of possible differential diagnoses, namely VLS, if infection recurs or is unresponsive to antifungal treatment.6 Immunology
Some autoimmune disorders, including thyroid disease, vitiligo, alopecia areata, pernicious anemia, and diabetes mellitus, are common among patients with VLS. Therefore, when clinical signs and/or symptoms suggest an underlying autoimmune disease, additional testing based on the suspected autoimmune disorder is recommended.5,6,8,10 COMPLICATIONS
Differential Diagnoses
The Table presents the differential diagnoses for VLS. DIAGNOSTIC TESTING Vulvar Biopsy
Vulvar biopsy is the gold standard for confirmation of pathologies such as VLS, SCC, and VIN. Biopsy is mandatory if neoplastic changes are suspected, the condition fails to respond to adequate treatment, extragenital LS is present, pigmented areas indicative of VIN or SCC are present, and when second-line therapy is required.2,9,10 Obtaining a biopsy before initiating treatment with topical corticosteroids is recommended because these medications have been found to interfere with biopsy results.6 NPs must understand that biopsy is not always essential or practical when the clinical features are typical. However, because LS is a lifelong chronic condition requiring long-term treatment and follow-up, confirmatory biopsy is recommended before initiating treatment.6 44
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Complications caused by the progression of VLS are troublesome. Affected individuals express frustration, anxiety, and embarrassment with the physical and emotional impact of this condition. The recurrent and often chronic symptoms of genital pruritus, irritation, burning, and pain can interfere with daily function and negatively affect self-image.5,6,11 Irreversible structural changes occur when inflammation is inadequately treated, often leading to sexual dysfunction and relationship difficulties.2,6,7,11 In addition, VLS has been associated with VIN and SCC (Figure 3). Although this relationship is not clearly understood, the widely accepted lifetime risk of developing SCC from VLS is considered to be 3% to 5%; however, approximately two thirds of vulvar cancer cases are found in close proximity to VLS.1,5,10 A diagnosis of vulvar cancer is commonly delayed even though patients may report having vulvar symptoms for many years.1 Health care providers primarily attribute this to poor recognition and diagnosis of vulvar conditions. Volume 10, Issue 1, January 2014
Figure 3. Vulvar lichen sclerosus in the background of squamous cell carcinoma.
PHARMACOLOGIC MANAGEMENT First-line Therapy
Topical ultrapotent corticosteroids. The most widely accepted and recommended gold standard treatment for VLS is the topical application of ultrapotent corticosteroid ointment, particularly clobetasol propionate 0.05%.8,10,12,13 The antiinflammatory properties of clobetasol have been most effective in managing associated symptoms, decreasing inflammation, and preventing progression of the condition and subsequent scarring.6 The mucous membranes of the vulva are relatively steroid resistant, meaning maximal steroid potency is required to achieve an effect.6 Ointments have fewer additives when compared with creams and therefore are less likely to irritate the skin.7 Initial treatment of acute inflammation should be prompt and aggressive. It is recommended that clobetasol propionate 0.05% be applied topically twice per day for 4 weeks followed by once per day application for 3 months and then maintenance dosing.10 Patients are to apply the corticosteroid ointment sparingly to clean, dry, affected areas only and should continue to use a maintenance dose of 1 to 2 applications per week even when asymptomatic.8,10 If symptoms increase with tapering of the treatment, the frequency of application should be increased until the symptoms begin to resolve. The overall length of treatment with clobetasol propionate 0.05% is largely directed by the presence www.npjournal.org
of symptoms and progression of the condition. Because inflammatory recurrences can be chronic and can lead to atrophy and scarring, long-term maintenance therapy is recommended and considered safe.8,10 A volume of 15 to 30 g is typically prescribed; a specialist referral is necessary if more than 30 g is required to control symptoms within a 6-month period.10 Because no randomized controlled trials have compared steroid potency, frequency of application, and duration of treatment, each patient is managed on a case-by-case basis, with individualized plans for symptom control, observation, and follow-up.10 A study published by Gurumurthy et al2 found that 66% of patients who were compliant with the clobetasol regimen experienced complete remission of their symptoms, and an additional 30% experienced a partial response to the treatment. Scarring did not progress in any patients who were compliant with clobetasol. Conversely, there was no improvement in symptoms among 75% of patients who were noncompliant with treatment, and scar progression occurred in 35% of these cases. Although rare, the most commonly reported side effects of long-term topical corticosteroid therapy in the anogenital region include dermal thinning, rebound reactions, striae formation, fungal infections, and systemic absorption resulting in adrenal suppression.4,8,12,13 Bradford and Fischer13 found that all side effects resolved promptly with a decrease in topical corticosteroid potency. Because of the rarity of adverse effects and proven efficacy and safety of treatment, ultrapotent corticosteroids should remain the first line recommendation for VLS. Second-line Therapy
Topical calcineurin inhibitors. Topical calcineurin inhibitors are currently recommended as second-line therapy for VLS. Topical pimecrolimus cream 1% is an immunosuppressant agent that inhibits T-cell activation and thus significantly improves the symptoms of pruritus, burning, and inflammation associated with VLS.5,12 Although calcineurin inhibitors can provide effective relief of symptoms, topical clobetasol is superior to pimecrolimus in terms of decreasing inflammation and improving overall appearance.7,12 Although The Journal for Nurse Practitioners - JNP
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pimecrolimus cream has an acceptable safety profile and does not cause dermal thinning, its use has been associated with an increased risk of SCC because of local immunosuppression.7,12 As a result, calcineurin inhibitors should be prescribed under the supervision of a specialist who can monitor for potential malignancy.2,12 Given the proven efficacy and safety of topical corticosteroid ointments, experts agree that calcineurin inhibitors should be reserved for cases in which corticosteroid therapy is ineffective.10,13 Retinoids. Typically, neither topical nor systemic retinoids have been effective in treating uncomplicated LS.10 Some evidence does suggest that retinoids may be effective for the treatment of hyperkeratosis or scarring from hypertrophic diseases that do not respond to corticosteroids.10 Specialist involvement is recommended for all patients who are considering the use of retinoids for VLS. Sedating agents. Because of the persistent symptoms of pruritus associated with LS, patients often worsen the skin condition by rubbing, itching, and scratching, particularly at night when they are unaware. These complications may be prevented if patients use prescription sedatives as well as overthe-counter oral antihistamines, particularly before going to sleep.6,7 Antihistamines work by inhibiting the activity of H1 receptors, which are responsible for the inflammatory reaction and subsequently pruritus. In addition, the lipophilic properties of antihistamines allow these drugs to cross the blood-brain barrier, leading to a sedating effect. However, the use of antihistamines as a sleep aid is not recommended for greater than 3 consecutive nights because patients will develop a tolerance to the sedative effect. The prolonged use of antihistamines may result in side effects such as daytime drowsiness, dry mouth, dizziness, and memory problems.14 NONPHARMACOLOGIC MANAGEMENT Therapeutics
In addition to pharmacologic treatment, patients must receive education regarding the importance of avoiding skin irritants and using comfort measures. These nonpharmacologic interventions can improve symptom management and improve quality of life. Avoidance of potential skin irritants such as soaps, sanitary pads, excessive scratching, and tight clothing 46
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can decrease local inflammation.6,7 Comfort measures that are nonirritating to the skin such as the application of cool gel packs, use of sitz baths, and daily application of petroleum jelly may provide temporary symptom relief.7,14 Skin moisturizers not containing common allergens such as a propylene glycol and lanolin may also minimize local inflammation. When used daily and applied liberally, petroleum jelly and other moisturizers are effective adjuncts in the treatment of VLS.6,15 Moisturizers increase the water content in the stratum corneum of the skin, which strengthens the weakened skin barrier and reduces subclinical inflammation.14 A study conducted by Simonart et al16 concluded that more than 50% of women who applied a daily moisturizer along with their topical corticosteroids remained asymptomatic for a median time of 58 months. In that study, over two thirds of women discontinued their topical corticosteroid without any effects on vulvar condition over the same period of time. Surgery
Surgery is not indicated for the removal of vulvar tissue affected by uncomplicated VLS because it will not cure the condition. Surgery is typically reserved for cases of VLS with malignancy or severe scarring and adhesions that interfere with urinary and/or sexual function.2,6,10 In the postoperative period, dilators are often warranted to reduce the recurrence of introitus narrowing.8 In the presence of vulvar SCC, an oncologic gynecologist is the most appropriate specialist for surgical management. Mechanical problems related to scarring and adhesions can be effectively managed by a gynecologic surgeon.10 Alternate Therapy
Dermatologists and gynecologists have used several alternative therapies for the treatment of LS with varying degrees of success. Some of these alternative therapies include photodynamic therapy, ultraviolet phototherapy, cryotherapy, and laser vaporization. Although these treatments have improved symptoms for many patients, there is limited evidence to support that alternative treatments slow disease progression or decrease recurrence rates.10 As a result, alternative therapies must be investigated further before they can be recommended for patients with VLS.2,4,10 Volume 10, Issue 1, January 2014
ROLE OF THE NURSE PRACTITIONER Referral
Gynecology. Nurse practitioners must possess the knowledge, skills, and judgment required to identify VLS. Whenever possible, NPs should obtain a vulvar biopsy before prescribing topical corticosteroids. It is recommended that the initial assessment be conducted through consultation with a gynecologist who can better direct management and arrange appropriate follow-up. A gynecology consult is also indicated for diagnostic workup when evaluation, testing, and/or biopsy are inconclusive or when the NP is not comfortable obtaining vulvar biopsy.6,8 In uncomplicated VLS, most sources suggest that patients have 2 follow-up visits with a specialist. The first follow-up should occur after 3 months of treatment to assess the therapeutic response. The second follow-up should occur 6 months later to ensure the patient understands disease progression, potential complications, and future assessment schedules.10 Long-term follow-up with a specialist is only required when troublesome symptoms and/or localized skin thickening are present, patients have had previous SCC or VIN or the presence of VIN uncertainty, when the condition is unresponsive to treatment, when greater than 30 g topical corticosteroid is used in a 6-month period, and when second-line therapy is being used.1,10 Psychiatry. VLS can have significant psychological and sexual impacts on women and their partners. As a result, psychiatric referral should be considered. Depression screening should be performed in a sensitive manner using recognized screening tools to promote patient comfort and enhance patient’s receptiveness to psychiatric consultation. In addition, many experts also encourage the involvement of sex therapists and sexologists who are better qualified to assist patients with sexual health issues.6,10,16 Follow-up
Appropriate follow-up by the NP enhances treatment compliance, maximizes optimal treatment, minimizes condition progression, and promotes aggressive identification and the management of progression and complications.1,7,9,13,14 Frequent follow-up allows the NP to investigate potential www.npjournal.org
reasons for treatment failure, which is most often related to poor compliance, incorrect diagnosis, secondary sensory problems, and irreversible mechanical problems related to scarring.10 Initial follow-up with the NP should be 1 to 3 months after the initial evaluation and is subsequently symptom based. Once the condition stabilizes, annual follow-up is sufficient.1,6,9,10 It is essential that NPs conduct thorough vulvar assessments at each follow-up visit. This assessment involves inspection of the entire vulva for textural and color changes, fissures, excoriation, erosions, ulcerations, lesions, and nodules.6 Biopsy of suspicious tissue is always indicated. The incorporation of vulvar examinations into routine physical examinations and cervical smears for all women may also promote early identification of vulvar conditions. Research has shown that 31% of women with vulvar SCC had more than 3 consults related to vulvar symptoms in the months preceding their diagnosis. Education and Counseling
Because of the chronic and progressive nature of VLS, it is crucial that patients understand the importance of maintenance therapy with topical corticosteroids. Patients should receive education regarding the cautionary labels found on these products because these labels may otherwise deter their compliance with treatment. Although adverse effects have been associated with the long-term use of topical corticosteroids, these effects are very rare.10 Frequent follow-up with NPs ensures the early identification of any adverse effects. In addition, patients must receive education on the proper application of topical corticosteroids, with emphasis placed on thorough hand washing, avoiding contact with sensitive areas of the body such as the eyes, and preventing the exposure of others to the medication.4 Bradford and Fischer13 noted that SCC only occurred in patients with VLS who were noncompliant with their treatment. Therefore, compliance with the treatment regimen is critical. Patients must also be aware that symptoms may improve with intermittent topical corticosteroid treatment, but previous scarring is irreversible.5 To enhance health promotion and disease prevention, all women should be educated on The Journal for Nurse Practitioners - JNP
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performing vulvar self-examinations. Proper monthly self-examinations promote self-awareness and early identification of potential changes.6 Self-examinations are best performed every month between periods. Using a mirror, the patient should closely inspect the mons pubis, labia majora, labia minora, perineum, and anus for any notable changes, including skin color and any new bumps, blisters, lesions, ulcers, warts, or sores. The patient should also feel for swelling, soreness, irritation, or itchiness. In addition to engaging in face-to-face discussions, NPs should provide patients with written instructions describing available treatments, both pharmacologic and nonpharmacologic components of vulvar care and hygiene, irritants to avoid, and the technique for vulvar self-examination.6,9 These written descriptions can effectively enhance the education and awareness of both patients and practitioners. CONCLUSION
Women’s health is a topic of growing concern in today’s society. Still, many vulvar conditions remain underdiagnosed and undertreated by health care providers. Women may feel uncomfortable discussing issues related to their genitalia with their primary care providers. As a result, NPs working in areas of women’s health and primary care settings must take the initiative to regularly discuss vulvar health with their patients. NPs must also include thorough assessments of the vulvar area in routine physical examinations to address any skin changes and chronic or recurrent symptoms. As NPs become more aware of various vulvar conditions and develop the skills required to assess for such conditions, they will be better able to confidently recognize changes, arrange appropriate diagnostic tests and referrals, initiate required treatment, and conduct follow-up as necessary. By engaging their patients in discussions regarding vulvar health promotion and disease prevention strategies, NPs can empower women to take control of their own health and improve their quality of life.
2. Gurumurthy M, Morah N, Gioffre G, Cruickshank ME. The surgical management of complications of vulval lichen sclerosus. Eur J Obstet Gynecol Reprod Biol. 2012;162(1):79-82. 3. Higgins CA, Cruickshank ME. A population-based case-control study of aetiological factors associated with vulval lichen sclerosus. J Obstet Gynaecol. 2012;32(3):271-275. 4. Pugliese JM, Morey AF, Peterson AC. Lichen sclerosus: review of the literature and current recommendations for management. J Urol. 2007;178(6):2268-2276. 5. Chi CC, Kirtschig G, Baldo M, Lewis F, Wang SH, Wojnarowska F. Systematic review and meta-analysis of randomized controlled trials on topical interventions for genital lichen sclerosus. J Am Acad Dermatol. 2012;67(2):305-312. 6. Thorstensen KA, Birenbaum DL. Recognition and management of vulvar dermatologic conditions: lichen sclerosus, lichen planus, and lichen simplex chronicus. J Midwifery Womens Health. 2012;57(3):260-275. 7. Vulvar lichen sclerosus may be treated effectively with topical corticosteroids, but long-term surveillance is required. Drugs Ther Perspect. 2010;26(5):16-19. 8. Edwards QT, Saunders-Goldson S. Lichen sclerosus of the vulva in women: Assessment, diagnosis, and management for the nurse practitioner. J Am Acad Nurse Pract. 2003;15(3):115-119. 9. Jones RW, Scurry J, Neill S, MacLean AB. Guidelines for the follow-up of women with vulvar lichen sclerosus in specialist clinics. Am J Obstet Gynecol. 2008;198(5):496.e1-496.e3. 10. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol. 2010;163(4):672-682. 11. Chi CC, Kirtschig G, Baldo M, Brackenbury F, Lewis F, Wojnarowska F. Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev. 2011;12:CD008240. 12. 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;64(6):e99-e104. 13. Bradford J, Fischer G. Long-term management of vulval lichen sclerosus in adult women. Aust N Z J Obstet Gynaecol. 2010;50(2):148-152. 14. Greaves MW. Antihistamines in dermatology. Skin Pharmacol Physiol. 2005;18(5):220-229. 15. Simonart T, Lahaye M, Simonart JM. Vulvar lichen sclerosus: effect of maintenance treatment with a moisturizer on the course of the disease. Menopause. 2008;15(1):74-77. 16. Van de Nieuwenhof HP, Meeuwis KA, Nieboer TE, Vergeer MC, Massuger LF, De Hullu JA. The effect of vulvar lichen sclerosus on quality of life and sexual functioning. J Psychosom Obstet Gynaecol. 2010;31(4):279-284.
Nicholas J. Wedel, BN, MN (NP), works as a nurse practitioner in the adult emergency department of the Health Sciences Centre in Winnipeg, Manitoba, and can be reached at nwedel@ exchange.hsc.mb.ca. Laura Johnson, RN (NP), MN, is an instructor for the primary care nurse practitioner program at the University of Manitoba and an emergency nurse practitioner at Health Sciences Center in Winnipeg. In compliance with national ethical guidelines, the authors report no relationships with business or industry that would pose a conflict of interest. Acknowledgment The photos in this article were taken by and printed with permission from Dr. Margaret Burnett, FRCSC, associate head, Academic, Department of Obstetrics and Gynecology, University of Manitoba, Canada.
References 1. Eva LJ. Screening and follow up of vulval skin disorders. Best Pract Res Clin Obstet Gynaecol. 2012;26(2):175-188.
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1555-4155/13/$ see front matter © 2014 Elsevier, Inc. All rights reserved. http://dx.doi.org/10.1016/j.nurpra.2013.10.009
Volume 10, Issue 1, January 2014
Vulvar lichen sclerosus (VLS) is a chronic, inflammatory skin condition of the vulva that most commonly affects postmenopausal women. Although there is currently no cure, modern treatments can effectively manage VLS once diagnosed. Unfortunately, vulvar conditions are routinely underdiagnosed and undertreated. Untreated VLS can cause significant physical, emotional, and sexual discomfort, which may result in irreversible destruction of the genitalia and can progress to squamous cell carcinoma. Nurse practitioners must be aware of the key features and complications of this condition and must have a good understanding of the diagnostic criteria and treatment strategies for VLS. Keywords: lichen sclerosus, nurse practitioner, squamous cell carcinoma, topical ultrapotent corticosteroids, vulvar biopsy, vulvar conditions Ó 2014 Elsevier, Inc. All rights reserved.
T
he topic of women’s health is currently receiving a great deal of attention in the media, among social groups, and by society as a whole. However, vulvar health is 1 aspect of women’s health that is often not discussed. It is estimated that up to 20% of all women will experience significant vulvar symptoms at some point in their lifetime.1 Still, conditions such as vulvar lichen sclerosus (VLS) are often underdiagnosed and undertreated.2,3 Admittedly, vulvar health may be a difficult topic for patients and practitioners to discuss. However when left untreated, VLS can cause significant physical, emotional, and sexual discomfort and may result in irreversible destruction of the genitalia and the development of squamous cell carcinoma (SCC). In order to minimize complications and improve patient’s quality of life, nurse practitioners (NPs) working in women’s health and in primary care settings must have the skills to recognize and manage conditions such as VLS. This article outlines the key features of VLS and provides the information NPs need to accurately identify, diagnose, and manage this condition. EPIDEMIOLOGY Definition
Lichen sclerosus (LS) is a chronic, inflammatory skin condition that can affect men, women, and children 42
The Journal for Nurse Practitioners - JNP
on any area of the body but most commonly affects the anogenital region of adult women.4 Although there is currently no cure for LS, the condition can be effectively managed when it is recognized and diagnosed appropriately. Prevalence
The true prevalence of VLS is difficult to determine because it often goes unrecognized and is never diagnosed. The most consistent estimates indicate that between 1 in 70 and 1 in 1000 women are affected by VLS, rates that are 10 times higher than those among men.1 The bimodal peaks of LS occur during prepuberty and postmenopause, with an increase to 1 in 30 among postmenopausal women.1,5 Higgins and Cruickshank3 indicated that at least 25% of women seen in dedicated vulva clinics received treatment for VLS. Etiology
Although the definite cause of LS is not currently known, experts have identified strong associations between LS and autoimmune diseases, genetic factors, and low levels of endogenous sex hormones.3,5 Thorstensen and Birenbaum6 found that up to 20% of women with VLS have coexisting autoimmune diseases, particularly thyroid disease, vitiligo, and alopecia areata.4 Other Volume 10, Issue 1, January 2014
Figure 1. Vulvar lichen sclerosus.
research studies have indicated that 44% to 74% of women with LS have tested positive for circulating immunoglobulin autoantibodies.5,6 High rates of VLS among first-degree relatives supports the theory that a genetic component exists in this condition.3 Up to 12% of patients with VLS have a positive family history of the disease.6 Low endogenous estrogen levels have also been linked with the development of VLS. This association is supported by the substantially higher incidence of VLS among postmenopausal women; however, researchers have not yet identified any consistent change in symptoms during menses, pregnancy, or with the use of oral contraceptive medications or hormone replacement therapies.7 KEY FEATURES Symptoms
Patients with VLS may experience an insidious or aggressive onset of symptoms, or they may remain www.npjournal.org
Figure 2. Vulvar lichen sclerosus.
asymptomatic for long periods of time. Vulvar pruritus is the hallmark symptom of VLS and may be mild and intermittent or intense and constant.6 The condition causes skin changes called vulvar fissures, which may occur spontaneously or through physical irritation from scratching and/or sexual intercourse.4,6 Vulvar fissures and subsequent scarring frequently result in dysuria, dyspareunia, pain with defecation, and rectal bleeding.4,6 Examination
VLS causes characteristic color changes to the skin of the vulva. The typical blanched, shiny white, crinkled tissue paper appearance can be present from the mons pubis to the perineal region and is characteristically distributed in a figure-of-8 or keyhole pattern over the labia and perineum (Figures 1 and 2).6,8 Skin changes occur externally only; the vagina and other internal structures are not affected by VLS. Flat, shiny white plaques surrounded by red-, purple-, or violet-colored borders are indicative of The Journal for Nurse Practitioners - JNP
43
Table. Differential Diagnoses for Vulvar Lichen Sclerosus 1. 2. 3. 4. 5.
Vulvar lichen sclerosus Candida vulvovaginitis Squamous cell carcinoma Vulvar intraepithelial neoplasia Genital tract inflammation a. Contact dermatitis b. Atrophic vaginitis c. Vestibular vulvodynia
atrophy and scarring caused by longstanding untreated VLS. Structural changes such as loss of the labia minora, flattening of the labia majora, inversion and trapping of the clitoris, and severe constriction of the introitus occur when VLS is left untreated or is treated ineffectively.2,6,7 Secondary skin lesions are usually present and may include vulvar edema, erythema, excoriation, and fissures. Concurrent infections can increase inflammation to the point of bullae formation. Deep erosions are not typically caused by VLS unless the condition is accompanied by significant scratching or in the presence of vulvar intraepithelial neoplasia (VIN) or SCC.6
Vaginal Swabs
Although vaginal swabs are not routinely required for the diagnosis of VLS, they may be useful in ruling out concurrent conditions, such as bacterial vaginosis, trichomoniasis, candida vulvovaginitis, herpes simplex virus, and other sexually transmitted infections. Vaginal swabs are primarily collected in the presence of abnormal discharge, vulvar erythema, fissuring, or excoriation.6,10 Signs and symptoms such as labial swelling, erythema, and thick white discharge are often easily identified as candida vulvovaginitis. However, practitioners must remain cognizant of possible differential diagnoses, namely VLS, if infection recurs or is unresponsive to antifungal treatment.6 Immunology
Some autoimmune disorders, including thyroid disease, vitiligo, alopecia areata, pernicious anemia, and diabetes mellitus, are common among patients with VLS. Therefore, when clinical signs and/or symptoms suggest an underlying autoimmune disease, additional testing based on the suspected autoimmune disorder is recommended.5,6,8,10 COMPLICATIONS
Differential Diagnoses
The Table presents the differential diagnoses for VLS. DIAGNOSTIC TESTING Vulvar Biopsy
Vulvar biopsy is the gold standard for confirmation of pathologies such as VLS, SCC, and VIN. Biopsy is mandatory if neoplastic changes are suspected, the condition fails to respond to adequate treatment, extragenital LS is present, pigmented areas indicative of VIN or SCC are present, and when second-line therapy is required.2,9,10 Obtaining a biopsy before initiating treatment with topical corticosteroids is recommended because these medications have been found to interfere with biopsy results.6 NPs must understand that biopsy is not always essential or practical when the clinical features are typical. However, because LS is a lifelong chronic condition requiring long-term treatment and follow-up, confirmatory biopsy is recommended before initiating treatment.6 44
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Complications caused by the progression of VLS are troublesome. Affected individuals express frustration, anxiety, and embarrassment with the physical and emotional impact of this condition. The recurrent and often chronic symptoms of genital pruritus, irritation, burning, and pain can interfere with daily function and negatively affect self-image.5,6,11 Irreversible structural changes occur when inflammation is inadequately treated, often leading to sexual dysfunction and relationship difficulties.2,6,7,11 In addition, VLS has been associated with VIN and SCC (Figure 3). Although this relationship is not clearly understood, the widely accepted lifetime risk of developing SCC from VLS is considered to be 3% to 5%; however, approximately two thirds of vulvar cancer cases are found in close proximity to VLS.1,5,10 A diagnosis of vulvar cancer is commonly delayed even though patients may report having vulvar symptoms for many years.1 Health care providers primarily attribute this to poor recognition and diagnosis of vulvar conditions. Volume 10, Issue 1, January 2014
Figure 3. Vulvar lichen sclerosus in the background of squamous cell carcinoma.
PHARMACOLOGIC MANAGEMENT First-line Therapy
Topical ultrapotent corticosteroids. The most widely accepted and recommended gold standard treatment for VLS is the topical application of ultrapotent corticosteroid ointment, particularly clobetasol propionate 0.05%.8,10,12,13 The antiinflammatory properties of clobetasol have been most effective in managing associated symptoms, decreasing inflammation, and preventing progression of the condition and subsequent scarring.6 The mucous membranes of the vulva are relatively steroid resistant, meaning maximal steroid potency is required to achieve an effect.6 Ointments have fewer additives when compared with creams and therefore are less likely to irritate the skin.7 Initial treatment of acute inflammation should be prompt and aggressive. It is recommended that clobetasol propionate 0.05% be applied topically twice per day for 4 weeks followed by once per day application for 3 months and then maintenance dosing.10 Patients are to apply the corticosteroid ointment sparingly to clean, dry, affected areas only and should continue to use a maintenance dose of 1 to 2 applications per week even when asymptomatic.8,10 If symptoms increase with tapering of the treatment, the frequency of application should be increased until the symptoms begin to resolve. The overall length of treatment with clobetasol propionate 0.05% is largely directed by the presence www.npjournal.org
of symptoms and progression of the condition. Because inflammatory recurrences can be chronic and can lead to atrophy and scarring, long-term maintenance therapy is recommended and considered safe.8,10 A volume of 15 to 30 g is typically prescribed; a specialist referral is necessary if more than 30 g is required to control symptoms within a 6-month period.10 Because no randomized controlled trials have compared steroid potency, frequency of application, and duration of treatment, each patient is managed on a case-by-case basis, with individualized plans for symptom control, observation, and follow-up.10 A study published by Gurumurthy et al2 found that 66% of patients who were compliant with the clobetasol regimen experienced complete remission of their symptoms, and an additional 30% experienced a partial response to the treatment. Scarring did not progress in any patients who were compliant with clobetasol. Conversely, there was no improvement in symptoms among 75% of patients who were noncompliant with treatment, and scar progression occurred in 35% of these cases. Although rare, the most commonly reported side effects of long-term topical corticosteroid therapy in the anogenital region include dermal thinning, rebound reactions, striae formation, fungal infections, and systemic absorption resulting in adrenal suppression.4,8,12,13 Bradford and Fischer13 found that all side effects resolved promptly with a decrease in topical corticosteroid potency. Because of the rarity of adverse effects and proven efficacy and safety of treatment, ultrapotent corticosteroids should remain the first line recommendation for VLS. Second-line Therapy
Topical calcineurin inhibitors. Topical calcineurin inhibitors are currently recommended as second-line therapy for VLS. Topical pimecrolimus cream 1% is an immunosuppressant agent that inhibits T-cell activation and thus significantly improves the symptoms of pruritus, burning, and inflammation associated with VLS.5,12 Although calcineurin inhibitors can provide effective relief of symptoms, topical clobetasol is superior to pimecrolimus in terms of decreasing inflammation and improving overall appearance.7,12 Although The Journal for Nurse Practitioners - JNP
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pimecrolimus cream has an acceptable safety profile and does not cause dermal thinning, its use has been associated with an increased risk of SCC because of local immunosuppression.7,12 As a result, calcineurin inhibitors should be prescribed under the supervision of a specialist who can monitor for potential malignancy.2,12 Given the proven efficacy and safety of topical corticosteroid ointments, experts agree that calcineurin inhibitors should be reserved for cases in which corticosteroid therapy is ineffective.10,13 Retinoids. Typically, neither topical nor systemic retinoids have been effective in treating uncomplicated LS.10 Some evidence does suggest that retinoids may be effective for the treatment of hyperkeratosis or scarring from hypertrophic diseases that do not respond to corticosteroids.10 Specialist involvement is recommended for all patients who are considering the use of retinoids for VLS. Sedating agents. Because of the persistent symptoms of pruritus associated with LS, patients often worsen the skin condition by rubbing, itching, and scratching, particularly at night when they are unaware. These complications may be prevented if patients use prescription sedatives as well as overthe-counter oral antihistamines, particularly before going to sleep.6,7 Antihistamines work by inhibiting the activity of H1 receptors, which are responsible for the inflammatory reaction and subsequently pruritus. In addition, the lipophilic properties of antihistamines allow these drugs to cross the blood-brain barrier, leading to a sedating effect. However, the use of antihistamines as a sleep aid is not recommended for greater than 3 consecutive nights because patients will develop a tolerance to the sedative effect. The prolonged use of antihistamines may result in side effects such as daytime drowsiness, dry mouth, dizziness, and memory problems.14 NONPHARMACOLOGIC MANAGEMENT Therapeutics
In addition to pharmacologic treatment, patients must receive education regarding the importance of avoiding skin irritants and using comfort measures. These nonpharmacologic interventions can improve symptom management and improve quality of life. Avoidance of potential skin irritants such as soaps, sanitary pads, excessive scratching, and tight clothing 46
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can decrease local inflammation.6,7 Comfort measures that are nonirritating to the skin such as the application of cool gel packs, use of sitz baths, and daily application of petroleum jelly may provide temporary symptom relief.7,14 Skin moisturizers not containing common allergens such as a propylene glycol and lanolin may also minimize local inflammation. When used daily and applied liberally, petroleum jelly and other moisturizers are effective adjuncts in the treatment of VLS.6,15 Moisturizers increase the water content in the stratum corneum of the skin, which strengthens the weakened skin barrier and reduces subclinical inflammation.14 A study conducted by Simonart et al16 concluded that more than 50% of women who applied a daily moisturizer along with their topical corticosteroids remained asymptomatic for a median time of 58 months. In that study, over two thirds of women discontinued their topical corticosteroid without any effects on vulvar condition over the same period of time. Surgery
Surgery is not indicated for the removal of vulvar tissue affected by uncomplicated VLS because it will not cure the condition. Surgery is typically reserved for cases of VLS with malignancy or severe scarring and adhesions that interfere with urinary and/or sexual function.2,6,10 In the postoperative period, dilators are often warranted to reduce the recurrence of introitus narrowing.8 In the presence of vulvar SCC, an oncologic gynecologist is the most appropriate specialist for surgical management. Mechanical problems related to scarring and adhesions can be effectively managed by a gynecologic surgeon.10 Alternate Therapy
Dermatologists and gynecologists have used several alternative therapies for the treatment of LS with varying degrees of success. Some of these alternative therapies include photodynamic therapy, ultraviolet phototherapy, cryotherapy, and laser vaporization. Although these treatments have improved symptoms for many patients, there is limited evidence to support that alternative treatments slow disease progression or decrease recurrence rates.10 As a result, alternative therapies must be investigated further before they can be recommended for patients with VLS.2,4,10 Volume 10, Issue 1, January 2014
ROLE OF THE NURSE PRACTITIONER Referral
Gynecology. Nurse practitioners must possess the knowledge, skills, and judgment required to identify VLS. Whenever possible, NPs should obtain a vulvar biopsy before prescribing topical corticosteroids. It is recommended that the initial assessment be conducted through consultation with a gynecologist who can better direct management and arrange appropriate follow-up. A gynecology consult is also indicated for diagnostic workup when evaluation, testing, and/or biopsy are inconclusive or when the NP is not comfortable obtaining vulvar biopsy.6,8 In uncomplicated VLS, most sources suggest that patients have 2 follow-up visits with a specialist. The first follow-up should occur after 3 months of treatment to assess the therapeutic response. The second follow-up should occur 6 months later to ensure the patient understands disease progression, potential complications, and future assessment schedules.10 Long-term follow-up with a specialist is only required when troublesome symptoms and/or localized skin thickening are present, patients have had previous SCC or VIN or the presence of VIN uncertainty, when the condition is unresponsive to treatment, when greater than 30 g topical corticosteroid is used in a 6-month period, and when second-line therapy is being used.1,10 Psychiatry. VLS can have significant psychological and sexual impacts on women and their partners. As a result, psychiatric referral should be considered. Depression screening should be performed in a sensitive manner using recognized screening tools to promote patient comfort and enhance patient’s receptiveness to psychiatric consultation. In addition, many experts also encourage the involvement of sex therapists and sexologists who are better qualified to assist patients with sexual health issues.6,10,16 Follow-up
Appropriate follow-up by the NP enhances treatment compliance, maximizes optimal treatment, minimizes condition progression, and promotes aggressive identification and the management of progression and complications.1,7,9,13,14 Frequent follow-up allows the NP to investigate potential www.npjournal.org
reasons for treatment failure, which is most often related to poor compliance, incorrect diagnosis, secondary sensory problems, and irreversible mechanical problems related to scarring.10 Initial follow-up with the NP should be 1 to 3 months after the initial evaluation and is subsequently symptom based. Once the condition stabilizes, annual follow-up is sufficient.1,6,9,10 It is essential that NPs conduct thorough vulvar assessments at each follow-up visit. This assessment involves inspection of the entire vulva for textural and color changes, fissures, excoriation, erosions, ulcerations, lesions, and nodules.6 Biopsy of suspicious tissue is always indicated. The incorporation of vulvar examinations into routine physical examinations and cervical smears for all women may also promote early identification of vulvar conditions. Research has shown that 31% of women with vulvar SCC had more than 3 consults related to vulvar symptoms in the months preceding their diagnosis. Education and Counseling
Because of the chronic and progressive nature of VLS, it is crucial that patients understand the importance of maintenance therapy with topical corticosteroids. Patients should receive education regarding the cautionary labels found on these products because these labels may otherwise deter their compliance with treatment. Although adverse effects have been associated with the long-term use of topical corticosteroids, these effects are very rare.10 Frequent follow-up with NPs ensures the early identification of any adverse effects. In addition, patients must receive education on the proper application of topical corticosteroids, with emphasis placed on thorough hand washing, avoiding contact with sensitive areas of the body such as the eyes, and preventing the exposure of others to the medication.4 Bradford and Fischer13 noted that SCC only occurred in patients with VLS who were noncompliant with their treatment. Therefore, compliance with the treatment regimen is critical. Patients must also be aware that symptoms may improve with intermittent topical corticosteroid treatment, but previous scarring is irreversible.5 To enhance health promotion and disease prevention, all women should be educated on The Journal for Nurse Practitioners - JNP
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performing vulvar self-examinations. Proper monthly self-examinations promote self-awareness and early identification of potential changes.6 Self-examinations are best performed every month between periods. Using a mirror, the patient should closely inspect the mons pubis, labia majora, labia minora, perineum, and anus for any notable changes, including skin color and any new bumps, blisters, lesions, ulcers, warts, or sores. The patient should also feel for swelling, soreness, irritation, or itchiness. In addition to engaging in face-to-face discussions, NPs should provide patients with written instructions describing available treatments, both pharmacologic and nonpharmacologic components of vulvar care and hygiene, irritants to avoid, and the technique for vulvar self-examination.6,9 These written descriptions can effectively enhance the education and awareness of both patients and practitioners. CONCLUSION
Women’s health is a topic of growing concern in today’s society. Still, many vulvar conditions remain underdiagnosed and undertreated by health care providers. Women may feel uncomfortable discussing issues related to their genitalia with their primary care providers. As a result, NPs working in areas of women’s health and primary care settings must take the initiative to regularly discuss vulvar health with their patients. NPs must also include thorough assessments of the vulvar area in routine physical examinations to address any skin changes and chronic or recurrent symptoms. As NPs become more aware of various vulvar conditions and develop the skills required to assess for such conditions, they will be better able to confidently recognize changes, arrange appropriate diagnostic tests and referrals, initiate required treatment, and conduct follow-up as necessary. By engaging their patients in discussions regarding vulvar health promotion and disease prevention strategies, NPs can empower women to take control of their own health and improve their quality of life.
2. Gurumurthy M, Morah N, Gioffre G, Cruickshank ME. The surgical management of complications of vulval lichen sclerosus. Eur J Obstet Gynecol Reprod Biol. 2012;162(1):79-82. 3. Higgins CA, Cruickshank ME. A population-based case-control study of aetiological factors associated with vulval lichen sclerosus. J Obstet Gynaecol. 2012;32(3):271-275. 4. Pugliese JM, Morey AF, Peterson AC. Lichen sclerosus: review of the literature and current recommendations for management. J Urol. 2007;178(6):2268-2276. 5. Chi CC, Kirtschig G, Baldo M, Lewis F, Wang SH, Wojnarowska F. Systematic review and meta-analysis of randomized controlled trials on topical interventions for genital lichen sclerosus. J Am Acad Dermatol. 2012;67(2):305-312. 6. Thorstensen KA, Birenbaum DL. Recognition and management of vulvar dermatologic conditions: lichen sclerosus, lichen planus, and lichen simplex chronicus. J Midwifery Womens Health. 2012;57(3):260-275. 7. Vulvar lichen sclerosus may be treated effectively with topical corticosteroids, but long-term surveillance is required. Drugs Ther Perspect. 2010;26(5):16-19. 8. Edwards QT, Saunders-Goldson S. Lichen sclerosus of the vulva in women: Assessment, diagnosis, and management for the nurse practitioner. J Am Acad Nurse Pract. 2003;15(3):115-119. 9. Jones RW, Scurry J, Neill S, MacLean AB. Guidelines for the follow-up of women with vulvar lichen sclerosus in specialist clinics. Am J Obstet Gynecol. 2008;198(5):496.e1-496.e3. 10. Neill SM, Lewis FM, Tatnall FM, Cox NH. British Association of Dermatologists’ guidelines for the management of lichen sclerosus 2010. Br J Dermatol. 2010;163(4):672-682. 11. Chi CC, Kirtschig G, Baldo M, Brackenbury F, Lewis F, Wojnarowska F. Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev. 2011;12:CD008240. 12. 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;64(6):e99-e104. 13. Bradford J, Fischer G. Long-term management of vulval lichen sclerosus in adult women. Aust N Z J Obstet Gynaecol. 2010;50(2):148-152. 14. Greaves MW. Antihistamines in dermatology. Skin Pharmacol Physiol. 2005;18(5):220-229. 15. Simonart T, Lahaye M, Simonart JM. Vulvar lichen sclerosus: effect of maintenance treatment with a moisturizer on the course of the disease. Menopause. 2008;15(1):74-77. 16. Van de Nieuwenhof HP, Meeuwis KA, Nieboer TE, Vergeer MC, Massuger LF, De Hullu JA. The effect of vulvar lichen sclerosus on quality of life and sexual functioning. J Psychosom Obstet Gynaecol. 2010;31(4):279-284.
Nicholas J. Wedel, BN, MN (NP), works as a nurse practitioner in the adult emergency department of the Health Sciences Centre in Winnipeg, Manitoba, and can be reached at nwedel@ exchange.hsc.mb.ca. Laura Johnson, RN (NP), MN, is an instructor for the primary care nurse practitioner program at the University of Manitoba and an emergency nurse practitioner at Health Sciences Center in Winnipeg. In compliance with national ethical guidelines, the authors report no relationships with business or industry that would pose a conflict of interest. Acknowledgment The photos in this article were taken by and printed with permission from Dr. Margaret Burnett, FRCSC, associate head, Academic, Department of Obstetrics and Gynecology, University of Manitoba, Canada.
References 1. Eva LJ. Screening and follow up of vulval skin disorders. Best Pract Res Clin Obstet Gynaecol. 2012;26(2):175-188.
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Volume 10, Issue 1, January 2014