Bagot Martine
Dermatologie, APHP, Hôpital Saint Louis, Université Paris 7, Unité Inserm U976, Centre de Recherche sur la Peau, 1 Avenue Claude Vellefaux, 75010 Paris.
Bull Acad Natl Med. 2010 Oct;194(7):1365-72.
Mycosis fungoides is the most frequent cutaneous T cell lymphoma. Mycosis fungoides-related cutaneous T cell lymphomas include folliculotropic mycosis fungoides, with or without mucinosis, pagetoid reticulosis and granulomatous slack skin. Sezary syndrome is characterized by pruriginous erythroderma, adenopathies, atypical lymphocytes in the blood and a more aggressive course. B cell lymphomas represent 25 % of cutaneous lymphomas. Primary cutaneous follicle center cell lymphomas are the most frequent. They are often located on the head and trunk and have an indolent course. Primary cutaneous marginal zone B-cell lymphomas, which are more frequently multicentric and located on the limbs, also have an indolent course. In contrast, primary cutaneous diffuse large B-cell lymphomas, leg type, and primary cutaneous intravascular large B-cell lymphomas have a more aggressive course. Treatment must be adapted to each case. Unnecessarily aggressive treatments must be avoided in patients with indolent lymphomas.
蕈样肉芽肿是最常见的皮肤T细胞淋巴瘤。与蕈样肉芽肿相关的皮肤T细胞淋巴瘤包括亲毛囊性蕈样肉芽肿（伴或不伴黏蛋白沉积症）、派杰样网状细胞增生症和肉芽肿性皮肤松弛症。 Sézary综合征的特征为瘙痒性红皮病、淋巴结病、血液中的非典型淋巴细胞以及病程更为侵袭性。B细胞淋巴瘤占皮肤淋巴瘤的25%。原发性皮肤滤泡中心细胞淋巴瘤最为常见。它们常位于头和躯干，病程呈惰性。原发性皮肤边缘区B细胞淋巴瘤更常为多中心性，位于四肢，病程也呈惰性。相比之下，原发性皮肤弥漫性大B细胞淋巴瘤（腿部型）和原发性皮肤血管内大B细胞淋巴瘤的病程更具侵袭性。治疗必须根据具体病例进行调整。对于惰性淋巴瘤患者，必须避免不必要的激进治疗。
