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成功以口服類固醇治療無合併關節炎之多源性網狀組織細胞增生症-病例報告與文獻回顧

Multicentric Reticulohistiocytosis without Arthritis Successfully Treated with Systemic Corticosteroids-A Case Report and Literature Review

摘要


我們報告一位四十六歲男性患者,於臉頰及四肢有輕微疼痛的紅色丘疹約兩個月,並無黏膜之侵犯或關節炎。手背僅有少數病灶且無甲溝周圍之分佈。皮膚切片顯示含顆粒狀、“毛玻璃”樣細胞質的單核或多核組織球在真皮浸潤。免疫組織化學染色下,該組織球為CD68陽性、S-100 Protein及CD30陰性。診斷為無關節炎之多源性網狀組織細胞增生症。除輕微膽固醇及三酸甘油脂偏高外,其他實驗室檢查均正常,亦無惡性腫瘤之證據。在每天Prodnisolono 0.5mg/kg治療之下反應良好,逐漸降低劑量,在第十五週時所有皮疹幾乎完全消退。我們認為無關節炎之多源性網狀組織細胞增生症可能為多源性網狀組織細胞增生症中對治療反應較佳之亞型。此假設需更多的病例報告加以證實。

關鍵字

無資料

並列摘要


A 46-year-old male presented with a 2-month history of multiple red papules on the cheeks and extremities without mucosal involvement or arthritis. There were only some lesions on the dorsal hands without periungual involvement. Biopsy of a skin lesion showed a dermal infiltrate consisting of mononuclear and multinucleated histiocytes with granular ”ground glass”-like cytoplasm. These histiocytes were CD68-positive, and S-100 protein and CD30 negative in immunohistochemical studies. The diagnosis of multicentric reticulohistiocytosis without arthritis was made. Laboratory data were within normal limits except mild hypercholesterolemia and hypertriglyceridemia. A survey for internal malignancy showed negative results. The skin lesions responded to prednisolone 0.5mg/kg daily. The dose was tapered gradually; the cutaneous lesions almost completely resolved after 15 weeks of treatment. Whether multicentric reticulohistiocytosis without arthritis is a subtype with better response to treatment remains to be confirmed by more clinical observation, but systemic corticosteroids may be tried first before starting other immunosuppressive or cytotoxic drugs.

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