蕈狀肉芽腫/Sezary病候群：罕見但值得注意的疾病

Mycosis Fungoides/Sezary Syndrome: A Rare Disease But Needs More Attention from Physicians

10.7023/TJFM.200209.0137

劉怡琴(I-Chin Liu)；郭集慶(Victor C. Kok)；吳美鳳(Mei-Fong Wu)；林正修(Jen-Shiou Lin)；張惠媚(Julia-Hueimei Chang)；劉幕台(Mu-Tai Liu) 蘇育德(Yu-Deh Su)

mycosis fungoides ； Sezary syndrome ； cutaneous T-cell lymphoma ； total skin electron beam radiation therapy

台灣家庭醫學雜誌

12卷3期（2002 / 09 / 01）

137 - 147

繁體中文

蕈狀肉芽腫(mycosis fungoides)/Sezary 症候群(Sezary syndrome)是一罕見的T-細胞淋巴癌，但卻是最常見的皮膚T細胞淋巴癌(cutaneous T-cell lymphomas)。其早期斑期病灶的臨床表現及病理變化，常很難和一些類似的發炎性皮膚病症作區分，即使有經驗的皮膚科醫師早期仍不易診斷，需長期追蹤病程，必要時必須重覆切片驗証，以避免誤診。我們以一病例配合文獻回顧，討論此疾病的臨床特徵、診斷、疾病分期及治療，提醒基層醫師能注意此疾病，並與常見的發炎性皮膚病症作區分，適時轉介皮膚科作切片診斷，勿延誤治療時機

Mycosis fungoides(MF) and Sezary syndrome(SS) are rare T-cell lymphomas, but they account for the majority of cutaneous T-cell lymphomas. A long history of undiagnosed skin difficult to distinguish from various inflammatory skin lesions in clinical findings and histopathology. Even an experienced dermatologist may miss the diagnosis in the so-called “premycotic” and subsequent “plaque” stage of MF. Therefore it requires clinicopathologic correlation and long-term follow-up. Repeated periodic biopsies may be required to avoid misdiagnosis. We present a case report and review some literature to discuss clinical features, diagnosis, staging and treatment of MF and SS. We hope this article can remind general practitioners to pay adequate attention to this disease and maintain a high index of suspicion. It is important to make a referral to a dermatologist for a tissue diagnosis so that disease can be detected in an earlier stage.

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