Greene R M, Rogers R S
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
J Am Acad Dermatol. 1989 Dec;21(6):1263-70. doi: 10.1016/s0190-9622(89)70341-8.
The Melkersson-Rosenthal syndrome is an uncommon condition of uncertain pathogenesis and cause. The classic triad of signs includes recurrent orofacial edema, recurrent facial nerve palsy, and lingua plicata. We retrospectively reviewed the medical records of 36 patients (24 women and 12 men) with elements of the Melkersson-Rosenthal syndrome. The complete triad was present in 9 (25%) patients. Orofacial involvement was the dominant feature; it occurred in all 36 patients and was the presenting sign in 15 (42%). Lingua plicata occurred in 18 (50%) patients, and peripheral facial paralysis was present in 17 (47%). Fourteen biopsy specimens were obtained, all from the orofacial region. Eight specimens revealed the classic pathologic picture of granulomatous cheilitis. No etiologic agent was identified in any of the patients. Diagnosis is difficult when all features of the triad are not present. A conservative treatment approach is recommended.
梅尔克森－罗森塔尔综合征是一种发病机制和病因不明的罕见病症。典型的三联征体征包括复发性口面部水肿、复发性面神经麻痹和皱襞舌。我们回顾性分析了36例具有梅尔克森－罗森塔尔综合征特征患者（24例女性和12例男性）的病历。9例（25%）患者出现了完整的三联征。口面部受累是主要特征；36例患者均有此表现，其中15例（42%）以此为首发症状。18例（50%）患者出现皱襞舌，17例（47%）存在周围性面瘫。共获取了14份活检标本，均来自口面部区域。8份标本显示为肉芽肿性唇炎的典型病理表现。所有患者均未发现病原体。当三联征的所有特征未全部出现时，诊断较为困难。建议采用保守治疗方法。
