Mori Yasuji, Kahari Veli-Matti, Varga John
Section of Rheumatology, University of Illinois at Chicago College of Medicine, 1158 MBRB, 900 S. Ashland Avenue, Chicago, IL 60607-7171, USA.
Curr Rheumatol Rep. 2002 Apr;4(2):113-22. doi: 10.1007/s11926-002-0006-0.
Several distinct entities associated with dermal fibrosis can mimic scleroderma/systemic sclerosis. The list of scleroderma-like conditions or scleroderma variants includes eosinophilic fasciitis, localized forms of scleroderma, scleredema and scleromyxedema, keloids, and environmental exposure-associated conditions including eosinophilia-myalgia syndrome and pseudosclerodermas induced by various drugs. Although these conditions are relatively uncommon, their accurate recognition is essential to avoid misdiagnosis and inappropriate therapy. The pathogenesis of these scleroderma variants appears to share similarities with each other and with that of scleroderma. Better understanding of scleroderma-like disorders is emerging through epidemiologic investigations, and in vivo and in vitro experimental research. Activation of eosinophils and disordered regulation of fibroblast collagen synthesis, apoptosis, and proliferation are recurrent findings in these disorders. The etiologic role of infection with Borrelia species or other microorganisms remains controversial. Cytokines such as transforming growth factor-beta, interleukin-4, interleukin-13, and connective tissue growth factor contribute to fibrosis in these disorders by inducing an accentuated and persistent fibrogenic response to tissue injury. The role of genetic factors in susceptibility and clinical expression of scleroderma-like conditions remains to be systematically addressed. Because of the relative rarity of these conditions, few well-controlled clinical treatment trials have been performed. In addition, there is no consensus on optimal management. Much anecdotal information and small clinical series indicate that phototherapy may have a role in the treatment of scleroderma-like conditions.
几种与皮肤纤维化相关的不同疾病实体可酷似硬皮病/系统性硬化症。硬皮病样疾病或硬皮病变体包括嗜酸性筋膜炎、局限性硬皮病、硬化性水肿和硬化性黏液水肿、瘢痕疙瘩，以及与环境暴露相关的疾病，包括嗜酸性粒细胞增多性肌痛综合征和各种药物诱发的假性硬皮病。尽管这些疾病相对不常见，但准确识别它们对于避免误诊和不恰当治疗至关重要。这些硬皮病变体的发病机制似乎彼此之间以及与硬皮病的发病机制有相似之处。通过流行病学调查以及体内和体外实验研究，人们对硬皮病样疾病有了更好的了解。在这些疾病中反复发现嗜酸性粒细胞活化以及成纤维细胞胶原合成、凋亡和增殖的调节紊乱。伯氏疏螺旋体或其他微生物感染的病因学作用仍存在争议。细胞因子如转化生长因子-β、白细胞介素-4、白细胞介素-13和结缔组织生长因子通过诱导对组织损伤的增强和持续的纤维化反应，在这些疾病中促成纤维化。遗传因素在硬皮病样疾病易感性和临床表型中的作用仍有待系统研究。由于这些疾病相对罕见，很少进行严格对照的临床治疗试验。此外，对于最佳治疗方案尚无共识。许多轶事性信息和小型临床系列表明，光疗可能在硬皮病样疾病的治疗中发挥作用。
