Rosebush Molly S, Allen Carl M, Accurso Brent T, Baiocchi Robert A, Cordell Kitrina G
Louisiana State University, 1100 Florida Avenue, New Orleans, LA, 70119, USA.
Central Ohio Skin & Cancer, Inc., Westerville, OH, USA.
Head Neck Pathol. 2019 Sep;13(3):492-499. doi: 10.1007/s12105-018-0923-5. Epub 2018 Apr 26.
Mycosis fungoides (MF) and Sézary syndrome are clonal T-cell proliferations that exhibit skin homing and represent the majority of cutaneous T-cell lymphomas. Early MF is a diagnostic challenge as both the clinical and microscopic features often mimic benign inflammatory conditions. Oral MF is very rare and has been associated in the past with advanced disease and a poor prognosis. Skin lesions are present for an average of > 6 years before oral involvement occurs. The clinical appearance is highly variable with tongue, palate and gingiva most often affected. We report 3 additional cases of oral MF, including one in which oral lesions are the initial disease presentation. Survival in patients presenting with oral MF is improving and can be attributed to advances in therapy.
蕈样肉芽肿(MF)和塞扎里综合征是具有皮肤归巢特性的克隆性T细胞增殖性疾病,占皮肤T细胞淋巴瘤的大多数。早期MF在诊断上具有挑战性,因为其临床和显微镜下特征常常类似于良性炎症性疾病。口腔MF非常罕见,过去一直与疾病晚期和预后不良相关。在出现口腔受累之前,皮肤病变平均存在超过6年。临床表现高度多变,最常累及舌、腭和牙龈。我们报告另外3例口腔MF病例,其中1例口腔病变是最初的疾病表现。以口腔MF就诊的患者生存率正在提高,这可归因于治疗方面的进展。