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Lichen sclerosus is a chronic inflammatory condition characterized by pruritic porcelain-white atrophic plaques. Most cases of lichen sclerosus affect the anogenital skin; however, extragenital manifestations occur either in isolation or with accompanying genital involvement. Patients with genital lichen sclerosus may also have accompanying morphea lesions in extragenital sites. Therefore, patients with either form of lichen sclerosus should have a total body skin exam to determine full extent of cutaneous involvement and look for accompanying morphea lesions. Patients with anogenital involvement may experience anal discomfort and fissuring, sexual dysfunction, or dysuria. In women with genital involvement, increased risk of squamous cell carcinoma is associated, particularly in those with long-standing poorly controlled lesions. This warrants education and lifelong monitoring. Extragenital lesions may produce pruritus and skin sensitivity. The goal of therapy is to relieve signs and symptoms of disease as well as prevent disease progression. In the authors’ experience, topical therapy with high-potency steroids is the mainstay treatment for genital and extragenital lichen sclerosus. Genital lesions are generally more responsive to topical therapy with potent topical steroids than extragenital lesions. Phototherapy is our preferred second-line treatment, particularly for extragenital involvement. Phototherapy may also be initiated in patients with extensive disease or in patients who are actively developing multiple new lesions. Systemic therapy, typically methotrexate plus oral prednisone with folic acid supplementation, is reserved for recalcitrant, widespread extragenital lichen sclerosus. Although studies are lacking, there is growing support for the use of systemic immunosuppressives like methotrexate in patients with anogenital disease when activity persists despite ultrapotent topical steroids. See Table 50-1.
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