๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
TREATMENT
Genital Lichen Sclerosus
Identification, early treatment, and ongoing maintenance therapy of genital LS is recommended, with the goals of relieving symptoms (e.g. pruritus), reversing skin changes (e.g. hyperkeratosis), and preventing the development of scarring, adhesions, and SCC. Table 73.1 outlines a detailed approach to anogenital LS.
Topical corticosteroids are the gold standard for treatment of genital LS in both adults and children and intralesional corticosteroids are also useful for treating areas of hyperkeratosis. Both retrospective and prospective studies have shown that ultrapotent topical corticosteroids (e.g. clobetasol propionate 0.05%) are highly effective in the treatment of genital LS. In boys with genital LS, topical corticosteroids can avoid circumcision in one-third of patients.Topical calcineurin inhibitors, although less potent than the recommended class I corticosteroids, also have a role in topical therapy. Ointments are preferred over creams because they are better tolerated, and frequent use of emollients (e.g. petrolatum) is also recommended. Topical JAK inhibitors (e.g. ruxolitinib 1.5% cream) are currently under investigation for anogenital LS.
Failure to respond to ultrapotent local corticosteroids should prompt evaluation for contributing factors, such as improper use of the corticosteroids, secondary infection (e.g. candidiasis), menopause-related vulvovaginal atrophy, and development of SCC. Second-line therapeutic options for genital LS have more limited efficacy data but include systemic agents (e.g. acitretin, abrocitinib)88,89,89a.
Importantly, because symptoms often recur upon discontinuation of initial therapy and because of the long-term risk of malignancy (i.e. SCC), it is now recommended that patients follow a long-term maintenance regimen upon resolution of signs (e.g. hyperkeratosis, ecchymoses, fissures) and symptoms (e.g. pruritus) after ~6โ12 weeks of induction therapy. Follow-up at regular intervals until a stable maintenance regimen is in place is advised, as is annual genital examination to exclude the development of malignancy.
Phototherapy
Phototherapy, primarily UVA1, has shown efficacy in some patients with genital LS and can be considered if topical/intralesional corticosteroids have failed. The long-term safety of UVA1 therapy in the genital region is unknown. The successful use of photodynamic therapy (PDT) for vulvar LS has been reported in small case series and reports. Additional studies are needed to evaluate the role of PDT in treating genital LS.
Surgery
Circumcision is the treatment of choice for genital LS complicated by phimosis or paraphimosis. Excellent results have been reported. Surgery in vulvar LS is indicated for the treatment of coexistent vulvar intraepithelial neoplasia, SCC, or fusion. LS will frequently recur around the scarring in females. Lastly, fractional laser ablation has been used for treatment of hyperkeratotic LS plaques.
Extragenital Lichen Sclerosus
Fewer studies exist for the treatment of extragenital LS. Phototherapy and weekly methotrexate +/โ systemic corticosteroids are potential treatment options for widespread disease, while potent topical corticosteroids and topical vitamin D analogues (e.g. calcipotriene [calcipotriol]) +/โ under occlusion as well as topical calcineurin inhibitors are options for more limited disease. When available, UVA1
phototherapy is preferred for widespread extragenital LS, followed by NB-UVB and then PUVA. The largest study to date of successful phototherapy for treating extragenital LS involved 10 patients who received 40 UVA1 sessions at a dose of 20โJ/cm four times/week. Case reports exist for successful use of NB-UVB and PUVA.
Systemic immunosuppressive therapy is typically reserved for widespread cases that are refractory to phototherapy or in those patients who cannot undergo phototherapy. Weekly methotrexate alone (10โ20โmg/week) or in combination with systemic corticosteroids (either pulsed [e.g. intravenous methylprednisolone 1000โmg/day for 3 consecutive days/month] or daily dosing [e.g. prednisone 1โmg/kg/day]) has shown efficacy in case reports and small case series. In a case report, dupilumab led to improvement.