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Cheilitis granulomatosa associated with melkersson-rosenthal syndrome

机译:与梅尔克森-罗森塔尔综合征相关的肉芽肿性唇炎

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Melkersson-Rosenthal syndrome (MRS) consists of persistent or recurrent orofacial edema, relapsing facial palsy and fissured tongue. The complete triad of symptoms is uncommon, varying from 8 to 25%. The presentation of only one symptom is more common. The most frequent complaint is facial edema and enlargement of the lips. We describe a case of a 17-year-old Brazilian girl with limited edema of the lower lip and fissured tongue due to MRS. Her complaints had started two years before. She referred previous clinical treatments without success. We proposed intralesional injection of triamcinolone at 20 mg every 15 days associated with oral clofazimine at 50 mg/day for three months. The lip became normal after four triamcinolone injections. Recent studies have considered MRS a granulomatous disease, and possibly the initial presentation of Crohn?’s disease in orofacial area of some patients. MRS patients, therefore, should be screened and monitored for gastrointestinal symptoms. Corticosteroid treatment seems to be effective in reducing lip enlargement. We discus the clinical features of this disease, the treatment, and the importance of corticosteroid therapy in cases of MRS-related facial palsy.
机译:Melkersson-Rosenthal综合征(MRS)包括持续性或复发性口面水肿,复发性面神经麻痹和舌裂。症状的完整三联症并不常见,范围从8%到25%不等。仅出现一种症状较为常见。最常见的主诉是面部水肿和嘴唇增大。我们描述了一个17岁的巴西女孩,由于MRS而导致下唇浮肿和舌裂的情况有限。她的投诉始于两年前。她转诊了先前的临床治疗方法,但没有成功。我们建议每15天病死率注射曲安西龙20毫克,并与口服氯氟嗪明以50毫克/天的剂量联合注射,持续三个月。在四次曲安西龙注射后,嘴唇变得正常。最近的研究认为MRS是肉芽肿性疾病,并且可能是某些患者口面部克罗恩病的最初表现。因此,应该对MRS患者进行筛查并监测其胃肠道症状。皮质类固醇激素治疗似乎可以有效减少嘴唇肿大。我们讨论了这种疾病的临床特征,治疗方法以及在MRS相关性面神经麻痹病例中应用糖皮质激素治疗的重要性。

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