Baughman Robert P, Lower Elyse E
Department of Medicine, Interstitial Lung Disease and Sarcoidosis Clinic, University of Cincinnati, Cincinnati, Ohio, USA.
Am J Clin Dermatol. 2004;5(6):385-94. doi: 10.2165/00128071-200405060-00003.
Skin involvement occurs in a third of patients with sarcoidosis. The type of lesions can range from the transient erythema nodosum to the chronic facial lesion lupus pernio. For some patients with sarcoidosis, lesions on the face or elsewhere on the body may be the major or only indication for therapy. These lesions are often chronic and the use of corticosteroids may lead to more long-term complications. Conventional alternatives to corticosteroids include antimalarial agents, methotrexate, and azathioprine. Recently, several drugs have been studied for chronic cutaneous sarcoidosis; thalidomide has been the most widely used. Thalidomide has been demonstrated to suppress tumor necrosis factor (TNF) release, which may be important at both the initial and chronic phases of the inflammation of sarcoidosis and appears to be crucial as part of the initial granulomatous response. Thalidomide has a different toxicity profile than corticosteroids or immunosuppressives. The usual dosage has recently been investigated in a dose-escalation trial, with the majority of patients responding to 100 mg/day. Drug toxicity has been reported in the sarcoidosis trials. The most serious adverse effect has been peripheral neuropathy, which often resolves by reducing the dose or discontinuing the medication. Other drugs that have been studied for sarcoidosis include infliximab and tetracyclines. Infliximab is a chimeric monoclonal antibody against TNF, and several published reports have shown it to be effective for the treatment of cutaneous sarcoidosis. The efficacy of tetracyclines for cutaneous sarcoidosis could be on the basis of their immunologic properties. In addition, these drugs have potent antimicrobial activity against Propionibacterium acnes; there is increasing evidence to suggest this may be one of the causes of sarcoidosis. However, most of the newer agents for cutaneous sarcoidosis have only been studied in small series. Over the next few years, it is hoped that there will be clinical trials to determine the role of each new therapy in the treatment of cutaneous sarcoidosis.
三分之一的结节病患者会出现皮肤受累。皮损类型多样,从短暂的结节性红斑到慢性面部损害冻疮样狼疮。对于一些结节病患者,面部或身体其他部位的皮损可能是主要或唯一的治疗指征。这些皮损往往是慢性的,使用皮质类固醇可能会导致更多的长期并发症。皮质类固醇的传统替代药物包括抗疟药、甲氨蝶呤和硫唑嘌呤。最近,有几种药物被用于研究慢性皮肤结节病;沙利度胺是使用最广泛的一种。沙利度胺已被证明可抑制肿瘤坏死因子(TNF)释放,这在结节病炎症的初始和慢性阶段可能都很重要,并且似乎是初始肉芽肿反应的关键部分。沙利度胺的毒性特征与皮质类固醇或免疫抑制剂不同。最近在一项剂量递增试验中研究了其常用剂量,大多数患者对每日100毫克的剂量有反应。在结节病试验中已报告了药物毒性。最严重的不良反应是周围神经病变,通常通过减少剂量或停药可缓解。其他用于研究结节病的药物包括英夫利昔单抗和四环素。英夫利昔单抗是一种抗TNF的嵌合单克隆抗体,一些已发表的报告表明它对治疗皮肤结节病有效。四环素对皮肤结节病的疗效可能基于其免疫特性。此外,这些药物对痤疮丙酸杆菌具有强大的抗菌活性;越来越多的证据表明这可能是结节病的病因之一。然而,大多数用于皮肤结节病的新型药物仅在小系列研究中进行了研究。希望在未来几年能进行临床试验,以确定每种新疗法在治疗皮肤结节病中的作用。