Medeiros Manoel, Araujo Maria Ilma, Guimarães Newton Sales, Freitas Luiz Antonio Rodrigues, Silva Tania Maria Correia, Carvalho Edgar Marcelino
Immunology Service, Hospital Universitario Federal University of Bahia, Salvador, Brazil.
Ann Allergy Asthma Immunol. 2002 Apr;88(4):421-4. doi: 10.1016/s1081-1206(10)62375-7.
Melkersson-Rosenthal syndrome (MRS) is a rare disorder of unknown etiology characterized by a triad of symptoms: recurrent orofacial swelling, relapsing facial palsy. and a fissured tongue. A differential diagnosis must be made with other granulomatous diseases, such as sarcoidosis and oral Crohn disease; however, the histologic findings of noncaseating, sarcoidal granulomas support the diagnosis of MRS.
Many therapeutic modalities have been described for this disease. In this case report, we present a patient with MRS that was treated with thalidomide because of the identification of tumor necrosis factor a in the lesion by immunohistochemical analysis. This is the first reported detection of tumor necrosis factor a in an MRS lesion, as well the first reported use of thalidomide to treat this clinical condition.
梅尔克森 - 罗森塔尔综合征(MRS)是一种病因不明的罕见疾病,其特征为三联征症状:复发性口面部肿胀、复发性面神经麻痹和沟纹舌。必须与其他肉芽肿性疾病进行鉴别诊断,如结节病和口腔克罗恩病;然而,非干酪样、结节样肉芽肿的组织学表现支持MRS的诊断。
针对这种疾病已描述了多种治疗方法。在本病例报告中,我们介绍了一名患有MRS的患者,因其病变通过免疫组织化学分析鉴定出肿瘤坏死因子α而接受了沙利度胺治疗。这是首次报道在MRS病变中检测到肿瘤坏死因子α,也是首次报道使用沙利度胺治疗这种临床病症。