早期表現皮膚症狀之原田氏症──病例報告

VKH Syndrome Presenting with Early Integumentary Signs-- A Case Report

目的：報告一例以皮膚症狀為早期表現之原田氏症病例方法：病例報告結果：一名27歲女性於2008年11月時因雙眼畏光兩週至眼科求診，無其他神經學症狀與特殊過去病史。雙眼矯正視力均為0.8。雙眼前房有發炎細胞與羊脂狀角膜內皮沈澱。眼底彩色攝影顯示無明顯漿液性視網膜剝離。眼球超音波檢查 (B-scan) 顯示為原田氏症早期表現之脈絡膜增厚。患者表示過去未曾有類似症狀發生。患者2008年8月起開始有頭髮變白與落髮情形而至皮膚科就診。給予患者局部類固醇點眼治療，二週後眼部發炎症狀消失，矯正視力1.2。於2009年3月患者再次因為雙眼畏光就診，雙眼矯正視力0.8，檢查時可見前葡萄膜炎表現，視網膜檢查可見原田氏症晚期表現包括眼底顏色變紅與脈絡膜視網膜色素脫落。給予患者全身性口服與局部點眼類固醇治療，兩週後眼部發炎症狀消失，雙眼矯正視力再恢復至1.2，藥物逐步減量至三個月後完全停止類固醇治療。治療六個月後至2009年12月未曾再有發作情形。結論：診斷原田氏症並無明確的檢驗方式，主要依靠患者症狀與臨床徵候表現。根據診斷準則認為皮膚症狀應為疾病後期表現，我們報告這例以皮膚症狀為疾病前期表現之原田氏症及其治療經驗。

A 27-year-old female complained of photophobia (OU) for 2 weeks. She had no specific systemic or ocular past history, and there were no neurologic symptoms. The anterior segments were normal except for numerous cells and mutton-fat KP. The patient had alopecia and poliosis of her scalp hair. She had visited a dermatologist because of alopecia and poliosis about 2 months previously. Ultrasonography demonstrated a thickening of the posterior choroid. After treatment with a topical steroid, the ocular inflammation subsided; however, anterior uveitis with photophobia recurred 2 months after the previous episode. Anterior segment examination revealed the same results as previously, except that color photography of the fundus showed much more red-orange coloration. Topical and systemic steroid therapy was prescribed. Two weeks later, the ocular inflammation subsided, and no recurrence was noted 4 months after treatment. Conclusion: According to the revised diagnostic criteria for VKH syndrome, integumentary signs of VKH disease are a late finding. Here we report a patient with VKH syndrome in whom integumentary disorder was the first presentation.