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題 名 | Phacomatosis Pigmentokeratotica: A 4-month-old Infant with Rare Melanocytic-Epidermal Twin Nevus Syndrome--Case Report=色素角化性母斑症:一位罹患罕見黑色素母斑與表皮母班合併症候群的四個月大男嬰--病例報告 |
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作 者 | 吳青穎; 張莞渝; 吳介山; 陳國熏; | 書刊名 | 中華皮膚科醫學雜誌 |
卷 期 | 25:4 2007.12[民96.12] |
頁 次 | 頁256-260 |
分類號 | 415.687 |
關鍵詞 | 色素角化性母斑; 黑色素小痣; Phacomatosis pigmentokeratotica; Speckled lentiginous nevus; Schimmelpenning syndrome; |
語 文 | 英文(English) |
中文摘要 | 色素角化性母斑是個很罕見的症候群,臨床表現為黑色素母斑合併皮脂腺分化,及皮膚以外的異常。此疾之皮膚症狀可由twin spotting的體染色體重組模式來解釋。我們報告一個四個月大的男嬰身上有黑色素小痣出現在線狀皮脂腺母斑上,並且伴隨有頑固性癫癇,視覺和聽覺異常。本例為台灣地區首次病例報告。本疾之皮膚病灶曾有惡性轉變案例,因此必須長期追蹤病灶的皮膚變化。 |
英文摘要 | Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. Such a combination of nevus sebaceous with melanocytic lesions may be explained by the concept of twin spotting. We describe a 4-month-old boy with a speckled lentiginous nevus arising from linear sebaceous nevus, intractable seizure, visual impairment, and hearing loss. To the best of our knowledge, this is the first case reported in Taiwan. Since cases of malignant degeneration of both nevus components have been reported, adequate follow-up of those with phacomatosis pigmentokeratotica is necessary. |
本系統中英文摘要資訊取自各篇刊載內容。