Department of Dermatology, University Hospital Basel, Petersgraben 4, 4031 Basel, Switzerland.
Am J Clin Dermatol. 2013 Feb;14(1):27-47. doi: 10.1007/s40257-012-0006-4.
Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin. LS is a debilitating disease, causing itch, pain, dysuria and restriction of micturition, dyspareunia, and significant sexual dysfunction in women and men. Many findings obtained in recent years point more and more towards an autoimmune-induced disease in genetically predisposed patients and further away from an important impact of hormonal factors. Preceding infections may play a provocative part. The role for Borrelia is still controversial. Trauma and an occlusive moist environment may act as precipitating factors. Potent and ultrapotent topical corticosteroids still head the therapeutic armamentarium. Topical calcineurin inhibitors are discussed as alternatives in the treatment of LS in patients who have failed therapy with ultrapotent corticosteroids, or who have a contraindication for the use of corticosteroids. Topical and systemic retinoids may be useful in selected cases. Phototherapy for extragenital LS and photodynamic therapy for genital LS may be therapeutic options in rare cases refractory to the already mentioned treatment. Surgery is restricted to scarring processes leading to functional impairment. In men, circumcision is effective in the majority of cases, but recurrences are well described. Anogenital LS is associated with an increased risk for squamous cell carcinoma of the vulva or penis. This review updates the epidemiology, clinical presentation, histopathology, pathogenesis, and management of LS of the female and male genitals and extragenital LS in adults and children.
硬化性苔藓（LS）是一种慢性、炎症性、黏膜和皮肤疾病，好发于生殖器和生殖器外皮肤。LS 是一种使人衰弱的疾病，可引起瘙痒、疼痛、排尿困难和排尿受限、性交困难以及男女患者明显的性功能障碍。近年来，越来越多的研究发现，LS 是一种由遗传易感患者自身免疫诱导的疾病，而不是由重要的激素因素引起的疾病。先前的感染可能起诱发作用。伯氏疏螺旋体的作用仍存在争议。创伤和闭塞性湿润环境可能起促成因素的作用。强效和超强效外用皮质类固醇仍然是治疗的主要手段。对于外用超强效皮质类固醇治疗失败或对皮质类固醇有禁忌的 LS 患者，外用钙调磷酸酶抑制剂被认为是替代治疗方法。在某些情况下，局部和全身维 A 酸类药物可能有用。光疗可用于治疗生殖器外 LS，光动力疗法可用于治疗生殖器 LS，这两种方法可能是罕见难治性病例的治疗选择。手术仅限于因瘢痕形成导致功能障碍的情况。对于男性，大多数情况下行包皮环切术有效，但复发的情况也有报道。肛门生殖器 LS 与外阴或阴茎鳞状细胞癌的风险增加有关。本文综述了女性和男性生殖器 LS 以及成人和儿童的生殖器外 LS 的流行病学、临床表现、组织病理学、发病机制和治疗方法。
