Moinzadeh P, Kreuter A, Krieg T, Hunzelmann N
Klinik und Poliklinik für Dermatologie und Venerologie, Universität zu Köln, Kerpener Str. 62, 50937, Köln, Deutschland.
Klinik für Dermatologie, Venerologie und Allergologie, Helios St. Elisabeth Krankenhaus Oberhausen, Universität Witten/Herdecke, Oberhausen, Deutschland.
Hautarzt. 2018 Nov;69(11):892-900. doi: 10.1007/s00105-018-4266-7.
Localized scleroderma (LoS) is a very heterogeneous connective tissue disease characterized by progressive sclerosis of the skin with possible involvement of extracutaneous structures. Both children and adults can be affected but show different frequencies for the individual subtypes of the disease. The clinical heterogeneity has already caused several modifications of existing classification criteria. Patients suffering from LoS can essentially be subdivided into five different subsets, which are defined by the horizontal and vertical extent of the tissue involvement. The quality of life of these patients is significantly impaired depending on the extent of the cutaneous and subcutaneous involvement. A causal treatment does not yet exist; however, patients should be treated with the currently available medications for progressive subtypes during the early phase of inflammation to reduce or avoid severe, cosmetic and functional impairments. Lichen sclerosus (LS) usually affects the genital as well as extragenital skin and both children and adults can be affected. This article focuses on the extragenital LS, which occurs more frequently in adults. The cause of the disease as well as causal treatment strategies are still lacking. Currently, treatment is adapted to the therapeutic strategies for LoS.
局限性硬皮病（LoS）是一种非常异质性的结缔组织疾病，其特征为皮肤进行性硬化，可能累及皮肤外结构。儿童和成人均可受累，但疾病各亚型的发病频率有所不同。临床异质性已导致现有分类标准的多次修订。LoS患者基本上可分为五个不同亚组，这是根据组织受累的水平和垂直范围来定义的。这些患者的生活质量会因皮肤和皮下受累程度而受到显著损害。目前尚无病因治疗方法；然而，对于进行性亚型患者，应在炎症早期使用现有药物进行治疗，以减少或避免严重的外观和功能损害。硬化性苔藓（LS）通常累及生殖器及生殖器外皮肤，儿童和成人均可受累。本文重点关注生殖器外LS，其在成人中更为常见。该病的病因及病因治疗策略仍然缺乏。目前，治疗方法是参照LoS的治疗策略。
