结节病合并梅-罗综合征：病例报告。
Melkersson-Rosenthal syndrome in the context of sarcoidosis: a case report.
机构信息
Department of Nephrology and Hypertension, Hanover Medical School, Carl-Neuberg-Strasse 1, 30625, Hanover, Germany.
Department of Nephrology, Städtisches Klinikum Braunschweig, Freisestraße 9-10, 38118, Brunswick, Germany.
出版信息
J Med Case Rep. 2021 Oct 4;15(1):488. doi: 10.1186/s13256-021-03044-5.
BACKGROUND
Melkersson-Rosenthal syndrome is a rare disease characterized by the triad of recurrent orofacial swelling with facial paralysis and fissured dorsal tongue. Histologically, noncaseating granulomatous inflammation occurs that confirms the diagnosis. Overlaps between granulomatous diseases such as sarcoidosis and Crohn's disease are described. Systemic corticosteroid therapy is the treatment of choice for acute attacks.
CASE PRESENTATION
We here present a case of a 59-year-old White woman suffering from Melkersson-Rosenthal syndrome with a past history of sarcoidosis on therapy with leflunomide in combination with low-dose tacrolimus successfully treated with the anti-leprosy drug clofazimine after failure of systemic steroid therapy.
CONCLUSIONS
We propose clofazimine as an alternative treatment in steroid-refractory cases.
背景
梅尔克松-罗森塔尔综合征是一种罕见疾病，其特征为复发性面颌肿胀伴面神经麻痹和舌裂背。组织学上出现非干酪样肉芽肿性炎症，可确诊该病。结节病和克罗恩病等肉芽肿性疾病之间存在重叠。急性发作时，全身皮质类固醇治疗是首选。
病例介绍
我们在此介绍一例 59 岁白人女性，患有梅尔克松-罗森塔尔综合征，既往有结节病病史，曾接受来氟米特联合低剂量他克莫司治疗，在全身类固醇治疗失败后，使用抗麻风病药物氯法齐明成功治疗。
结论
我们建议氯法齐明可作为类固醇难治性病例的替代治疗方法。
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