Long-term Management of Adult Vulvar Lichen Sclerosus A Prospective Cohort Study of 507 Women
Long-term Management of Adult Vulvar Lichen Sclerosus A Prospective Cohort Study of 507 Women
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DOI：
10.1001/jamadermatol.2015.0643
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发表时间：
2015-10-01
期刊：
影响因子：
10.9
通讯作者：
Fischer, Gayle
中科院分区：
文献类型：
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作者：
Lee, Andrew;Bradford, Jennifer;Fischer, Gayle
成人外阴苔藓硬化症(VLS)可合并外阴结构丧失和外阴癌。对于维持长期缓解和预防并发症的理想方法，缺乏证据。目的确定长期预防性局部皮质类固醇(TCS)治疗VLS，目标是诱导和维持正常的皮肤纹理和颜色，是否降低外阴癌的风险，缓解症状，改善功能，保护外阴结构，并评估治疗的不良反应。设计、设置和参与者从2008年1月2日到2014年9月26日，在悉尼皮肤科医生和妇科医生的私人诊所中，对507名VLS患者进行了前瞻性纵向队列研究干预使用不同效力的TCS进行预防性治疗，调整以达到正常肤色和质地的目标结果，并由皮肤科医生或妇科医生进行定期的长期随访。主要结果和测量VLS、疤痕形成、恶性肿瘤发展和不良反应的症状或体征。结果平均发病年龄55.4岁(范围18-86岁)；症状持续时间为5.0年(0.1-40.0年)，随访时间为4.7年(2.0-6.8年)。用有效的TCS诱导缓解，然后定期预防性TCS治疗，效价滴定以实现目标结果。患者至少每年进行一次随访。共有150名患者(29.6%)没有执行建议的治疗，被认为是部分依从性。共有357名患者(70.4%)遵守了治疗说明，并被认为是依从性的。活检证实的鳞状细胞癌或外阴上皮内瘤变在顺从性患者中为0例，部分顺从性患者中为7例(4.7%)(P<；.001)。333名(93.3%)依从性患者症状得到抑制，而部分依从性患者为87(58.0%)(P<；.001)。12例(3.4%)依从性患者和60例(40.0%)部分依从性患者在随访期间发生粘连和瘢痕形成(P<；.001)。4例(1.1%)依从性患者和3例(2.0%)部分依从性患者发生可逆性皮肤萎缩。结论与相关性这项针对成年VLS患者的前瞻性、单中心、纵向队列研究表明，个性化预防性TCS方案可实现皮肤颜色和质地的客观正常，并供定期长期随访的顺从性患者使用，可能会改变病程。依从性和部分依从性患者在症状控制、疤痕形成和外阴癌发生方面有显著差异。TCSs的不良反应微乎其微。
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.