頁籤選單縮合
題名 | Hand-Schuller-Christian氏病=Hand-Schuller-Christian Disease |
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作者 | 洪勖峰; 陳建州; 沈瑞隆; 楊安航; | 書刊名 | 中華皮膚科醫學雜誌 |
卷期 | 17:2 1999.06[民88.06] |
頁次 | 頁140-147 |
分類號 | 415.725 |
關鍵詞 | S-100蛋白質; Hand-schuller-christian disease; S-100 protein; Birbeck granules; |
語文 | 中文(Chinese) |
中文摘要 | 一位18個月的男孩出生後不久即於頭皮處出現許多脫屑性紅色丘疹及斑塊,合併 有痂皮、抓痕及些許膿皰。曾經被當作脂漏性皮膚炎治療,但對外用類固醇療效欠佳。且最 近兩個月,凸眼及皮膚病灶變得更加惡化。光學顯微鏡檢查顯示在真皮表皮交接處有廣泛性 的組織球浸潤,這些細胞亦往上侵犯表皮。S-100蛋白質染色呈陽性且電子顯微鏡檢查下發 現有典型的Birbeck granules。除此之外,發現多處的溶骨性病灶及缺乏多重器官的功能缺損。 因此診斷為Sch?ller-Christian disease。病患接受prednisolone及vincristine治療,病況獲得 改善。 |
英文摘要 | A 18-month-old boy suffered from multiple erythematous scaling papuloplaques with some serous crusts, excoriation markings and few pustules on his scalp soon after birth. He had been treated as seborrheic dermatitis, but poor response to topical steroid was noted. Exophthalmos and skin lesions got worse in recent two months. Light microscopic examination of the skin specimen revealed extensive histiocyte infiltration on dermo-epidermal junction and exocytosis of these cells. S-100 protein stain revealed positive reaction and typical Birbeck granules were noted under electronic microscopic examination. Additionally, multi-focal osteolytic lesions and lack of dysfunction of multiple organs were noted. Therefore, Hand- Sch?ller-Christian disease was diagnosed. The patient received prednisolone and vincristine treatment and general condition improved. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。