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題名 | Mal de Meleda with Incidental Acantholysis--A Case Report with Histopathological and Ultrastructural Studies=Mal de Meleda伴隨偶發的棘層鬆解--病例報告附帶組織病理及超顯微研究 |
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作者 | 楊淑敏; 紀秀華; 張俊祥; | 書刊名 | 中華皮膚科醫學雜誌 |
卷期 | 15:2 1997.06[民86.06] |
頁次 | 頁87-92 |
分類號 | 415.763 |
關鍵詞 | 棘層鬆解; 組織病理; 超顯微; Meleda type palmoplantar keratoderma; Glove-and-sock erythrokeratoderma; Incidental acatholysis; Composite keratohyalin granules; |
語文 | 英文(English) |
中文摘要 | 二十五歲女性病人被診斷為Meleda型手腳皮膚角化症。病人在二十天大時即已發病,且病程持續發展。來到門診時病人有典型的呈手套及襪子狀分佈的皮膚紅斑及角化。右手第三、四、五隻手指從十五歲開始即有收縮性癵縮. 她的父母親為近親通婚,並有一弟弟有類似皮膚病變。病理切片從右手掌皮膚角化處取得。除了明顯的角質增厚和乾癬樣的棘層增厚外,在部分增長的網狀棘可見類似Hailey-Hailey病病理變化的棘層鬆解。超顯微結構研究顯示在汗管間的表皮有少量的合成角質層透明質顆粒。 |
英文摘要 | A-25-year old female patient was diagnosed to have Meleda-type palmoplantar keratoderma (PPK). The disease had its onset when the patient was 20 days old, and had followed a progressive course. At presentation, the patient had so-called "glove-and-sock erythrokeratoderma". Flexion contracture of the right third, fourth, and fifth fingers had become as complication at age 15. The patient's parents were consanguineous and one of her brothers was similarly affected. A biopsy specimen was taken from her right palm. In addition to marked orthohyperkeratosis and psoriasiform acanthosis, histopathological examination also revealed incidental focal acantholysis of the Hailey-Hailey disease pattern involving four of the elongated rete ridges. Ultrastructural study showed a small number of composite keratohyalin granules in the interductal epidermis and widening of the epidermal cell-cell junctions with sloughing of hemidesmosomes. |
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