Department of Skin and Venereal Diseases, Medical Academy, Lithuanian University of Health Sciences, Eivenių 2, 50028 Kaunas, Lithuania.
Medicina (Kaunas). 2010;46(10):649-56.
Localized scleroderma is a chronic inflammatory disease primarily of the dermis and subcutaneous fat that ultimately leads to a scar-like sclerosis of connective tissue. The disorder manifests as various plaques of different shape and size with signs of skin inflammation, sclerosis, and atrophy. This is a relatively rare inflammatory disease characterized by a chronic course, unknown etiology, and insufficiently clear pathogenesis. Many factors may influence its appearance: trauma, genetic factors, disorders of the immune system or hormone metabolism, viral infections, toxic substances or pharmaceutical agents, neurogenic factors, and Borrelia burgdorferi infection. Various therapeutic modalities are being used for the treatment of localized scleroderma. There is no precise treatment scheme for this disease. A majority of patients can be successfully treated with topical pharmaceutical agents and phototherapy, but some of them with progressive, disseminated, and causing disability localized scleroderma are in need of systemic treatment. The aim of this article is not only to dispute about the clinical and morphological characteristics of localized scleroderma, but also to present the newest generalized data about the possible origin, pathogenesis, and treatment modalities of this disease.
局限性硬皮病是一种主要累及真皮和皮下脂肪的慢性炎症性疾病，最终导致结缔组织的瘢痕样硬化。该病表现为各种形状和大小不同的斑块，伴有皮肤炎症、硬化和萎缩的迹象。这是一种相对罕见的炎症性疾病，其特征为慢性病程、病因不明和发病机制尚不清楚。许多因素可能影响其表现：创伤、遗传因素、免疫系统或激素代谢紊乱、病毒感染、有毒物质或药物、神经源性因素和伯氏疏螺旋体感染。目前有多种治疗方法用于局限性硬皮病的治疗。针对这种疾病，尚无确切的治疗方案。大多数患者可以通过局部药物治疗和光疗成功治疗，但有些患者的局限性硬皮病呈进行性、播散性，且导致残疾，需要进行系统性治疗。本文的目的不仅在于讨论局限性硬皮病的临床和形态学特征，还在于介绍关于这种疾病的可能起源、发病机制和治疗方法的最新综合数据。
