嗜伊紅性筋膜炎：一病例報告與文獻回顧

Eosinophilic Fasciitis: One Case Report and Literature Review

嗜伊紅性筋膜炎(Eosinophilic fasciitis)，是一種臨床上表皮的變化，類似於硬皮症(Scleroderma-like)，但卻沒有雷諾氏現象(Raynaud's phenomenon)、甲摺鏡檢(Capillary microscopy)正常，且在病理上其主要發炎反應在筋膜層而不是在表皮層的一種病變。臨床上其表現有嗜伊紅性白血球血症(Eosinophilia)，高伽瑪球蛋白血症(Hypergammaglobulinemia)及高紅血球沈降速率(high erythrocyte sedimentation rate)。本文中報告一位54歲女性於一次車禍之後，左足踝出現一不癒合的傷口，並有嗜伊紅性白血球血症及四肢腫脹與嚴重壓痛的現象，其初期對於類固醇(steroid)有顯著的療效，但在其後雖然繼續給予類固醇及其他藥物的治療，皮下卻仍有進行性硬化的現象，且其皮膚也出現典型的橘皮徵候(Peau d'orange sign)與溝紋徵候(groove sign)。同時我們回顧文獻，並討論此病的臨床表現、病理特徵、鑑別診斷、治療與預後。

Eosinophilic Fasciitis is a disease spectrum similar to that of scleroderma and the inflammation essentially involves the fascia but not epidermal layer. However, the absence of Raynaud phenomenon and capillary microscopic findings in this disease can significantly differ from the typical presentations of scleroderma. Usually it presents with eosinophilia, hypergammaglobulinemia, and high erythrocyte sedimentation rate. Herein, we report a 54-year-old female who developed a non-healing wound over her left ankle and eosinophilia after a traffic accident. Four limbs painful swelling with severe tenderness was followed subsequently. She was treated with prednisolone with a initial good response. Even though the steroid and other immunosuppressive treatment administered, her skin developed progressive sclerotic changes. In this study, we will review the literatures, and discuss the clinical features, pathologic findings, differential diagnosis, treatment, and prognosis of this disease.