Vulvar lichen sclerosus (VLS) is an underdiagnosed and undertreated chronic dermatosis.1,2 It has an estimated prevalence of 0.1%–1.7%2 and a bimodal age distribution, predominantly affecting prepubertal girls and postmenopausal women. It is characterized by itching and scarring lesions that hinder normal sexual and urinary function.1 Overregulation of the Th1 pathway gives rise to a chronic proinflammatory state in keratinocytes. Inactivation of tumor suppression genes such as the p53 gene and CDKN2A leads to dysplastic changes that may facilitate subsequent development of vulvar squamous cell carcinoma (vSCC).1–4 The purpose of the various therapies used (including topical corticosteroids, calcineurin inhibitors, and photodynamic therapy) is to relieve symptoms, prevent scarring, and avoid malignant transformation.1 High-potency topical corticosteroids constitute the treatment of choice (Table 1).1–5 While their use may hinder transformation to vSCC,5 it is not known whether they can reduce vSCC recurrence.
Recommended Treatment of Vulvar Lichen Sclerosus.
Recommended treatment | High-potency topical corticosteroids such as clobetasol propionate 0.05%* |
Recommended regimen | Daily topical application for 1–3 months, depending on clinical response, subsequently on alternate days, and ultimately twice per week as a maintenance regimen |
Potential benefits | Reduction in symptomsReduction in associated structural changes, such as synechiae and scarringDecreased development of vSCCReduced recurrence of vSCC |
Treatment duration | Not defined |
Potential adverse effects | Cutaneous atrophy, telangiectasias, irritation, and pigmentation alterations, and other local secondary effects. However, studies with lengthy follow-up periods have reported no serious adverse effects |
Other alternatives include other topical corticosteroids, such as 0.1% mometasone fuorate, intralesional corticosteroids, topical calcineurin inhibitors, 2% topical testosterone, 2% or 8% topical progesterone, topical retinoids and cyclosporine, phototherapy, and photodynamic therapy. Systemic treatments used include various immunosuppressants such as glucocorticoids, cyclosporine and methotrexate. Surgery and CO2 laser are reserved for the treatment of associated complications.
Additional recommendations include use of emollients and soft underwear (silk or similar), and avoidance of rubbing, scratching, soap, and frequent washing with water.
Abbreviations: vSCC, vulvar squamous cell carcinoma. Source: Lee et al.5
Chin et al.3 recently published the results of a cohort study in patients with VLS with a history of excised vSCC or differentiated vulvar intraepithelial neoplasia (dVIN). The authors studied the association between indefinite therapy with topical corticosteroids (daily use with a subsequent adjustment upon achieving disease control, defined as skin with normal texture and no white discoloration) and the risk of recurrence of vSCC or dVIN. Patients excluded (n=11) were those with vSCC or dVIN associated with papillomavirus, those with poor adherence to corticosteroid treatment, and those with less than 5 years of follow-up. Eight patients (73%) remained free of recurrence, with a mean follow-up duration of 10.5 years (range, 5.1–16.5 y).
Two patients (18%) experienced recurrence: one (9%) had multiple recurrences of vSCC, and the other (9%) experienced recurrence of dVIN. In total, 27% experienced some form of recurrence. This rate differs from the 5-year recurrence rates reported in the literature for patients who did not undergo maintenance treatment (44%–47%). Only one patient (9%) experienced multiple recurrences, a much lower percentage than that reported in other studies.3,4 No significant adverse effects associated with topical corticosteroid therapy were described. One of the limitations of the study is its small sample size, although the long follow-up period adds to the robustness of the findings. Lee et al.5 reported better clinical outcomes in patients receiving indefinite treatment with high potency corticosteroids, which reduced anatomical alterations and symptoms and prevented the development of vSCC, compared with previous studies in which topical corticosteroids were used only when patients presented clinical signs. Prolonged use of topical corticosteroids could reduce chronic inflammation associated with the development of vSCC, thereby decreasing health costs and morbidity.
A growing number of physicians are opting for indefinite treatment with topical corticosteroids, although many remain wary of possible side effects and opt to discontinue treatment once symptoms are controlled.1,2 We wish to emphasize the importance of chronic topical corticosteroid treatment in patients with VLS, especially those with prior vSCC/dVIN, to avoid recurrences and post-surgical sequelae.