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Original Investigation |

Long-term Management of Adult Vulvar Lichen Sclerosus A Prospective Cohort Study of 507 Women

Andrew Lee, MBBS1; Jennifer Bradford, MBBS2; Gayle Fischer, MD1
[+] Author Affiliations
1Sydney Medical School Northern, The University of Sydney, New South Wales, Australia
2School of Medicine, University of Western Sydney, New South Wales, Australia
JAMA Dermatol. 2015;151(10):1061-1067. doi:10.1001/jamadermatol.2015.0643.
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Importance  Adult vulvar lichen sclerosis (VLS) may be complicated by loss of vulvar structure and vulvar carcinoma. There is a lack of evidence as to the ideal method to maintain long-term remission and prevent complications.

Objectives  To determine whether long-term preventive topical corticosteroid (TCS) treatment of VLS, with a target outcome of induction and maintenance of normal skin texture and color, reduces the risk of vulvar carcinoma, relieves symptoms, improves function, and preserves vulvar architecture, and to evaluate the adverse effects of treatment.

Design, Setting, and Participants  A prospective longitudinal cohort study was conducted in 507 women with biopsy-proved VLS from January 2, 2008, through September 26, 2014, in the private practice of a dermatologist and a gynecologist in Sydney, Australia.

Interventions  Preventive treatment using TCSs of various potencies, adjusted to meet a target outcome of normal skin color and texture, with regular long-term follow-up by a dermatologist or gynecologist.

Main Outcomes and Measures  Symptoms or signs of VLS, scarring, development of malignant neoplasms, and adverse effects.

Results  The mean age at presentation was 55.4 years (range, 18-86 years); duration of symptoms at presentation, 5.0 years (range, 0.1-40.0 years); and duration of follow-up, 4.7 years (range, 2.0-6.8 years). Remission was induced with a potent TCS, followed by regular preventive TCS treatment of a potency titrated to achieve the target outcome. Patients were followed up at least annually. A total of 150 patients (29.6%) did not carry out the advised treatment and were considered partially compliant. A total of 357 patients (70.4%) adhered to treatment instructions and were considered compliant. Biopsy-proved squamous cell carcinoma or vulvar intraepithelial neoplasia occurred during follow-up in 0 of the compliant patients vs 7 (4.7%) of the partially compliant patients (P < .001). Suppression of symptoms occurred in 333 (93.3%) compliant patients vs 87 (58.0%) partially compliant patients (P < .001). Adhesions and scarring occurred during follow-up in 12 (3.4%) compliant patients and 60 (40.0%) partially compliant patients (P < .001). Reversible TCS-induced cutaneous atrophy occurred in 4 (1.1%) compliant patients and 3 (2.0%) partially compliant patients.

Conclusions and Relevance  This prospective, single-center, longitudinal cohort study of adult patients with VLS suggests that individualized preventive TCS regimens that achieve objective normality of skin color and texture and are used by compliant patients who attend regular long-term follow-up visits may modify the course of the disease. There was a significant difference in symptom control, scarring, and occurrence of vulvar carcinoma between compliant and partially compliant patients. The adverse effects of TCSs were minimal.

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Figure 1.
Severity Guide to Treatment of Vulvar Lichen Sclerosus

Grading of hyperkeratosis in vulvar lichen sclerosus used to determine corticosteroid treatment potency.

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Figure 2.
Suggested Long-term Topical Corticosteroid (TCS) Management of Vulvar Lichen Sclerosus

Treatment guideline based on the degree of hyperkeratosis of vulvar lichen sclerosus.

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A satisfactory long term-management of vulvar lichen sclerosus: a goal to score
Posted on July 15, 2015
Alessandro Borghi, Monica Corazza, Annarosa Virgili
Dipartimento di Scienze Mediche, Sezione di Dermatologia e Malattie Infettive, Università degli Studi di Ferrara, Italia
Conflict of Interest: None Declared
To the Editor
We read with great interest the article by Lee and colleagues “Long-term Management of Adult Vulvar Lichen Sclerosus. A Prospective Cohort Study of 507 Women”.1 This prospective cohort study on a large sample of patients affected with vulvar lichen sclerosus (VLS) faces the need for a suitable strategy of long-term management of this chronic-relapsing disease. In fact, while the efficacy of topical corticosteroids (TCs) in the treatment of active VLS is well-documented,2,3 there is currently surprisingly little evidence on the long-term control of the disease after effective treatment with TCs. Lee and colleagues proposed individualized TC regimens, using molecules of various potencies initially chosen according to the severity of hyperkeratosis; once disease and symptom suppression had been achieved, long-term maintenance treatment was initiated, with gradual reduction of TC potency titrated to the clinical response. The main target outcomes were: achieving normal skin color and texture, preserving vulvar architecture and reducing the risk of vulvar squamous neoplasia without significant adverse effects.
We agree with the authors that regular, long-term TC regimens may be more effective in achieving satisfactory disease management than their use on an ‘as needed’ basis. In accordance with the authors, we also consider that restoration and then preservation of the vulvar objective features are treatment endpoints as relevant as symptom control. Moreover, in our clinical practice we schedule protracted patients’ follow up as well.
As regards VLS treatment regimens, we would like to share our former experience with proactive treatment as a maintenance strategy.4,5 This involves the intensive application of TC therapy until both active signs and symptoms are cleared. It is then followed by twice-weekly, long-term application of the same drug to the previously affected areas. In previous randomized trials, we found that twice-weekly proactive application of ultra-potent or potent TCs over 52 weeks was an effective and safe therapy option both in maintaining VLS sign and symptom improvement, obtained after a 12-week active treatment, and in preventing relapse.4,5 No adverse events directly connected to the therapies were observed. In addition, almost all the enrolled patients judged the intermittent regimen as convenient. This possibly explains the very high adherence to treatment observed among our patients. Based on our results, we believe that twice-weekly TC application could represent a reliable treatment in the challenging, long-term management of VLS.
In conclusion, studies like that by Lee and colleagues are a welcome contribution to the literature. Comparing continuative and proactive maintenance treatments would be a very important means for providing indication on the most appropriate regimen in terms of efficacy, tolerability, convenience and prevention of cancer development.

References


1. Lee A, Bradford J, Fischer G. Long-term Management of Adult Vulvar Lichen Sclerosus: A Prospective Cohort Study of 507 Women. JAMA Dermatol. 2015 [Epub ahead of print]
2. Virgili A, Borghi A, Toni G, Minghetti S, Corazza M. First randomized trial on clobetasol propionate and mometasone furoate in the treatment of vulvar lichen sclerosus: results of efficacy and tolerability. Br J Dermatol. 2014;171(2):388-96.
3. Chi CC, Kirtschig G, Baldo M, et al. Topical interventions for genital lichen sclerosus. Cochrane Database Syst Rev 2011 Dec 7; 12: CD008240.
4. Virgili A, Minghetti S, Borghi A, Corazza M. Proactive maintenance therapy with a topical corticosteroid for vulvar lichen sclerosus: preliminary results of a randomized study. Br J Dermatol. 2013; 168(6):1316-24.
5. Corazza M, Borghi A, Minghetti S, Toni G, Virgili A. Clobetasol propionate vs. mometasone furoate in 1-year proactive maintenance therapy of vulvar lichen sclerosus: results from a comparative trial. J Eur Acad Dermatol Venereol. 2015 [Epub ahead of print]

Long-Term Treatment of Adult Vulvar Lichen Sclerosus: Does compliance prevent squamous cell carcinoma? A still unanswered question
Posted on November 17, 2015
Micheline Moyal-Barracco, Giao Do-Pham, Laurence Le Cleach
Department of Dermatology, Hôpital Tarnier-Cochin, APHP, 89, rue d’Assas, 75006 Paris, France (Moyal-Barracco); Department of Dermatology, Hôpital Henri-Mondor, APHP, 51, avenue du Maré
Conflict of Interest: None Declared
We read with interest the paper by Lee and colleagues1 and the accompanying editorial by Cooper SM et al2 concerning the long-term management of vulvar lichen sclerosus (VLS) in adults. Article and editorial authors considered that the reported study findings support the conclusion that “adequate” VLS treatment (ie, tailored applications of topical corticosteroids and good compliance) prevents scarring and cancer.
We think that that message cannot be so assertive. First, the pertinence of the chosen strategy (topical corticosteroid applications tailored to VLS severity) has not yet been established. In addition, hyperkeratosis was the only severity criterion used by the authors. Although this term is well-defined histologically, it has no consensual, reproducible clinical definition. However, the authors did not provide their working definition in the article and the photos (Figure 1) do not convincingly illustrate the clinical meaning they give to the term hyperkeratosis and consequently to the grading they have adopted. Indeed, it is not clear whether their grading was based on the intensity or the extension of the whiteness, or to the thickness or superficial roughness of the lesions. A consensual, clinical, grading score for VLS activity/severity is still needed.
The intensity of VLS symptoms, the degree of whiteness and the presence of fissure or ecchymosis are some items that could be integrated into a composite clinical score to be established by expert consensus. Because the study was monocentric and treatment decision was based on subjective not consensual outcomes, external validity can be considered low.
We have even more questions about the reliability of the assessment of treatment adherence. Poor compliance with dermatological treatments for chronic skin disease is well-established, ranging from 55% to 66% according to the measurement method used.3 Adherence is poorer for topical treatments and maintenance therapy.4 The rate of non-compliance to maintenance therapy could be even higher for vulvar disease due the fact that the vulva is a “hidden” region. There are at least 11 patient-reported, adherence-assessment tools5 but the authors relied on only 1 question, apparently asked only once, despite the unlikelihood of a constant degree of compliance during follow-up. In addition, self-reported adherence is usually overestimated when compared to objective measures, such as the electronic Medication Event Monitoring System.6 That cutaneous atrophy was not observed more frequently in the presumably compliant patients raises even more doubts about the confidence of the self-reported adherence in this study. Finally, factors influencing compliance were not specified and could have been confounding. For example, persistence of symptoms could have led to poorer adherence.

REFERENCES
1. 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.
2. Cooper SM, Madnani N, Margesson L. Reduced risk of squamous cell carcinoma with adequate treatment of vulvar lichen sclerosus. JAMA Dermatol. 2015;151(10):1059-1060.
3. Serup J, Lindblad AK, Maroti M, et al. To follow or not to follow dermatological treatment – a review of the literature. Acta Derm Venereol. 2006;86(3):193-197.
4. Torrelo A, Ortiz J, Alomar A, Ros S, Pedrosa E, Cuervo J. Health-related quality of life, patient satisfaction, and adherence to treatment in patients with moderate or severe atopic dermatitis on maintenance therapy: the CONDA-SAT study. Actas Dermosifiliogr. 2013;104(5):409-417.
5. Greenlaw SM, Yentzer BA, O'Neill JL, Balkrishnan R, Feldman SR. Assessing adherence to dermatology treatments: a review of self-report and electronic measures. Skin Res Technol. 2010;16(2):253-8.
6. Urquhart J. The electronic medication event monitor. Lessons for pharmacotherapy. Clin Pharmacokinet. 1997;32(5):345-56.





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