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題 名 | 鞏皮性黏液水腫:對Isotretinoin有療效--病例報告=Scleromyxedema: Response to Isotretinoin--Report of a Case |
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作 者 | 楊進隆; 何宜承; 鄭裕文; | 書刊名 | 中華皮膚科醫學雜誌 |
卷 期 | 15:2 1997.06[民86.06] |
頁 次 | 頁115-120 |
分類號 | 415.7 |
關鍵詞 | 鞏皮性黏液水腫; Scleromyxedema; Lichen myxedematosus; Isotretinoin; |
語 文 | 中文(Chinese) |
中文摘要 | 鞏皮性黏液水腫是一罕見、慢性之類鞏皮症疾病,常易伴有良性單源性免疫球蛋白異常;有時亦有皮膚以外器官之病態表現,甚至急性致命病程. 本症並無確實有效之治療方式. 本文報告一位42歲的男性建築工人,因為全身長滿了苔癬樣、無症狀、光亮蠟樣之融合性丘疹,並在骨隆凸處有顯著厚皮及浸潤性之斑塊及結節,而來院求診。皮疹發生已超過3年的時間且最近一年內更形惡化. 此外病人有聲音沙啞、胸部有些許緊繃感、大腿肌稍呈無力、手背部有麻木感及左腕關節痛之情形。皮膚病灶組織病理顯示不論在丘疹或斑塊處,均有大量黏多醣堆積在上半部真皮層之中。全身性檢查顯示多源性免疫球蛋白G之血清值上昇,並有輕微之肌病變及聲帶腫脹. 以isotretinoin口服治療5個月後,有些丘疹及結節有明顯消褪改善。因病人對isotretinoin反應不錯且臨床上相當安全,所以我們建議isotretinoin可當成此病治療藥物之一選擇. |
英文摘要 | Scleromyxedema, a variant of lichen myxedematosus, is a rare and chronic scleroderma-like cutaneous disorder. Benign monoclonal gammopathy was present in most patient. Frequently, it was associated with extracutaneous manifestation. Treatments usually were unsatisfactory. Herein waxy xolescent papules and thicked indurated plaques or nodules oer bony prominences for more than 3 years. The eruptions worsen in recent one year. Histopathology of the skin with special stains showed large accumulation of acid mucopolysaccharide in dermis both in papule and plaque lesions. Other findings included polyclonal IgG gammopathy, mild myopathy and vocal cord swelling. Obvious effacement of papules and some nodules were observed after 5 months' treatment with isotretinoin (30-50 mg/day). Because of the favorable response to isotretionin in our patient and several other patients in the literature, isotretionin can be recommended as a choice of treatment for scleromyxedema. |
本系統中英文摘要資訊取自各篇刊載內容。