Rybojad M
Service de Dermatologie, Hôpital Saint-Louis, 1, avenue Cl.-Vellefaux, 75475 Paris Cedex 10.
Ann Med Interne (Paris). 2001 Mar;152(2):89-95.
Sarcoidosis is a systemic granulomatous condition of unknown origin. It is defined by histological features of epithelioid and giant-cell granuloma without caseous necrosis. Skin manifestations are observed in 25% of the cases of sarcoidosis, sometimes appearing as the inaugural symptom. Although these skin disorders are not life threatening, they do have a major functional and/or esthetic impact and may require specific therapy using aggressive treatments. Recent progress in immunology and molecular biology is helpful in understanding interactions between monocyte-macrophages and T lymphocytes and the cytokine pattern implicated in the granulomatous reaction. Research concerning the agent(s) causing the focus of the immune response on the TH1 pathway may be helpful in developing promising immunomodulation therapies. The very unpredictable course of the disease explains the difficulties encountered in assessing therapeutic efficacy and the small number of controlled trials. General corticosteroid therapy remains the mainstay treatment for severe skin disorders in sarcoidosis. APS and local corticoid therapy can be useful when systemic corticosteroids are not needed or to achieve a steroid-sparing effect. Indications for methotrexate and new compounds such as thalidomide or certain antibiotics with an immunomodulator effect remain to be evaluated.
结节病是一种病因不明的全身性肉芽肿性疾病。它由上皮样和巨细胞肉芽肿的组织学特征定义，无干酪样坏死。25%的结节病患者会出现皮肤表现，有时表现为首发症状。虽然这些皮肤疾病不会危及生命，但它们确实会对功能和/或美观产生重大影响，可能需要使用积极的治疗方法进行特殊治疗。免疫学和分子生物学的最新进展有助于理解单核细胞-巨噬细胞与T淋巴细胞之间的相互作用以及肉芽肿反应中涉及的细胞因子模式。关于导致免疫反应聚焦于TH1途径的因素的研究可能有助于开发有前景的免疫调节疗法。该疾病非常不可预测的病程解释了评估治疗效果时遇到的困难以及对照试验数量较少的原因。全身糖皮质激素治疗仍然是结节病严重皮肤疾病的主要治疗方法。当不需要全身糖皮质激素或为了达到激素节省效果时，抗疟药和局部皮质类固醇治疗可能有用。甲氨蝶呤以及沙利度胺或某些具有免疫调节作用的抗生素等新化合物的适应证仍有待评估。
