Melkersson-Rosenthal Syndrome: A Case Report

默克森－羅森泰症候群－病例報告

10.6313/FJR.2006.20(1-2).10

洪國棟(Guo-Dung Hung)；謝祖怡(Tsu-Yi Hsieh)；陳得源(Der-Yuan Chen)；藍忠亮(Joung-Liang Lan)

默克森－羅森泰症候群 ； 面部水腫 ； 顏面神經麻痺 ； 溝紋舌 ； Melkersson-Rosenthal syndrome ； orofacial edema ； facial paralysis ； lingua plicata

Formosan Journal of Rheumatology

20卷1&2期（2006 / 12 / 01）

72 - 76

英文

默克森－羅森泰症候群是一種以復發性或持續性面部水腫、顏面神經麻痺、溝紋舌爲表現之少見疾病。臨床上此三項典型徵候不一定會全部出現，因而造成此症候群診斷之困難。我們在此提出一位二十四歲男性以復發性面部水腫與顏面神經麻痺爲臨床表現的默克森－羅森泰症候群病例。其運動神經傳導試驗顯示複合肌肉動作電位波(compound muscle action potential)之振幅減小。核磁共振造影顯示在顯影劑釓(Gadolinium)離子注射後，其顏面神經出現顯影增強現象。而眼瞼病理切片結果顯示在深皮層出現非壞死性肉芽腫、淋巴管與顏面神經周圍有淋巴球浸潤現象。該病患經診斷爲默克森-羅森泰症候群後，接受皮質類固醇與azathioprine之治療後，其面部水腫與顏面神經麻痺逐漸獲得緩解。

Melkersson-Rosenthal syndrome is a rare disorder characterized by the triad of recurrent or persist orofacial edema, relapsing facial paralysis and fissured tongue. The syndrome does not always manifest in the complete triad and the diagnosis is often difficult. Here, we report a 24-year-old man presented with recurrent orofacial swelling and facial paralysis. The motor nerve conduction test showed reduction of the amplitude of compound muscle action potential of his facial nerve. The magnetic resonance imaging revealed a T1 gadolinium-enhancement along mastoid segment of left facial nerve. The biopsied specimens from the eyelid revealed a non-necrotizing granulomatous lymphohistiocytic infiltration surrounding the lymphatic duct and branches of his facial nerve. Melkersson-Rosenthal syndrome was diagnosed and the orofacial edema and facial paralysis resolved gradually after treatment with corticosteroid and azathioprine.