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題名 | 蕈狀肉芽腫:臨床和病理研究及斑期病灶之早期診斷=Mycosis Fungoides--A Clinicopathologic Study and Early Diagnosis of Patch Lesions |
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作 者 | 蕭嘉蓉; 李玉雲; | 書刊名 | 中華皮膚科醫學雜誌 |
卷期 | 19:2 2001.06[民90.06] |
頁次 | 頁98-111 |
分類號 | 415.74 |
關鍵詞 | 蕈狀肉芽腫; 白斑; 皮膚變色; 苔蘚化糠疹; 玫瑰糠疹; Mycosis fungoides; Pityriasis lichenoides; Pityriasis rosea; Poikiloderma; Dyschromatosis; Vitiligo; |
語文 | 中文(Chinese) |
中文摘要 | 蕈狀肉芽腫〈MF〉在台灣較少被報告,雖然它可能並非如此罕見。MF之是最常見的皮膚T細胞淋巴瘤〈CTCL〉。MF之早期斑期病灶其病理變化常很難和一些類似的發炎性皮膚病症區分。吾人根據臨床並配合病理表現而診斷出之18例MF病例,應是至今台灣的報告中病例數最多的。18例病例之臨床全部是斑或斑塊期病灶。從發病至確定診斷的期間由3個月至28年不等〈平均約6.2年〉〈Table.I〉。6例為較少見的臨床表現,包括局限性白斑樣病灶,頸部瀰漫性色素沉著,廣泛分佈之皮膚變色、色素異常〈dyschromatosis〉斑,慢性苔蘚化糠疹樣皮疹,及玫瑰糠疹樣皮疹。9例病例接受PUVA治療,於平均累積劑量133.5J/㎝²時,可達到完全消褪的療效。仔細分析25個斑期病灶的病理變化,結果如下:〈1〉表淺血管周圍浸潤型和局部苔蘚樣帶狀浸潤型共佔84%,〈2〉76%有輕微至中等程度之淋巴球親上皮現象;其中56%有呈線形沿著基底層排列現象〈basal epitheliotropism〉,36%有Pautrier's microabscesses,〈3〉只有32%有輕微至中等程度異形之淋巴球浸潤,〈4〉68&可見乳頭狀真皮層中有纖維化,呈現輕微至懂程度的鐵絲網狀粗的膠原纖維。早期MF的特徵性病理變化常很輕微,如果有淋巴球之親上皮現象,伴隨著無或輕微的海綿樣水腫,或同時於真皮內看到一些中大型的具腦迴樣或高度迴旋狀細胞核之異形淋巴球,以及於乳頭狀真皮層中有鐵絲網狀之纖維化,則可以幫著我們高度懷疑或診斷早期MF。但顯著的真皮水腫或明顯表皮海綿樣水腫之microvesiculation,基本上與MF的診斷不符。吾人觀察認為早期病理診斷斑期MF雖然困難,但卻是可能的;不過仍須配合臨床表現,以及長期追蹤病程,必要時須重覆切片驗證,以避免誤診。 |
英文摘要 | Mycosis fungoides (MF) is uncommonly reported in Taiwan although it might not be rare. Early, patch MF lesions are often difficult to distinguish from various inflammatory lesions histopathologically. We reported the clinicopathologic findings of 18 cases, the largest series to date in Taiwan. All patients presented with patch or plaque lesions with duration of 3 months to 28 years (mean 6.2 years). Six had unusual manifestations, including localized vitiligo-like lesions, diffuse brownish pigmentation of the neck and face, widespread patch of dyschromatosis, pityriasis lichenoides chronica-like and pityriasis rosea-like eruptions. Nine were treated with PUVA therapy with complete remission after a mean of 133.5J/㎝². The important pathologic features of patch lesions (n=25) included (1) superficial perivascular or patchy lichenoid histologic pattern (84%), (2) epitheliotropism of lymphocytes (76%) with characteristic basal epitheliotropism (56%) and Pautrier’s microabscesses (36%), (3) lymphocytic atypia (32%), mostly slight to moderate degree, (4)fibrosis of papillary dermis with characteristic wiry collagen (68%). Findings of basal epitheliotropism with minimal or no spongiosis, medium to large atypical lymphocytes with hyperconvoluted nuclei and fibrosis with wiry collagen in the papillary dermis, though may be subtle, were important clues to the diagnosis of early patch MF. Conversely, the presence of obvious dermal edema or spongiosis with microvesiculation militates against the possibility of MF. Our observations support the opinions that the early histologic diagnosis of patch stage of MF is possible, though often difficult, and requires clinicopathologic correlation, long-term follow up and repeated biopsy to avoid misdiagnosis. |
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