Ingen-Housz-Oro S, Franck N, Beneton N, Fauconneau A, Do-Pham G, Carlotti A, Petit T, Liolios I, Bara C, Carpentier H, Storelli D, Prophette B, Garderet L, Haioun C, Petit E, Delfau-Larue M-H, Vergier B, Chosidow O, Beylot-Barry M, Ortonne N
Department of Dermatology, AP-HP, Henri Mondor Hospital, Créteil.
J Eur Acad Dermatol Venereol. 2015 Jan;29(1):77-85. doi: 10.1111/jdv.12454. Epub 2014 Mar 19.
Mycosis fungoides (MF) and pseudo-MF (or MF simulant) can be associated with B-cell malignancies, but distinction between a true neoplasm and a reactive process may be difficult.
To report seven patients with B-cell malignancy and folliculotropic MF or pseudo-MF and emphasize on criteria allowing distinction between the two conditions.
We retrospectively and prospectively included seven patients with B-cell malignancy who presented skin lesions histologically consisting in a folliculotropic T-cell infiltrate and reviewed the literature on the topic.
Four men and three women had a chronic lymphocytic leukaemia (n = 6) or a MALT-type lymphoma (n = 1). Five patients had localized papules, and two had patches and plaques. Histological examination showed in all cases a diffuse dermal T-cell infiltrate with folliculotropic involvement and follicular mucinosis associated with clusters of the B-cell lymphoma, without significant expression of follicular helper T-cell markers. T-cell rearrangement studies showed a polyclonal pattern in the patients with papules and a monoclonal pattern in the cases of patches and plaques. Papular lesions had an indolent evolution, whereas patches and plaques persisted or worsened into transformed MF.
Folliculotropic T-cell infiltrates associated with B-cell malignancies can be either a true folliculotropic MF or a pseudo-MF. The distinction between both conditions cannot rely only on the histopathological aspect, but needs both a clinical pathological correlation and the search for a dominant T-cell clone. Whether the neoplastic T and B cells derive from a common ancestor or the T-cell proliferation is promoted by the underlying B-cell lymphoma remains unsolved, but interaction between B and T cell in the skin does not appear to be dependent on a TFH differentiation of the T-cell infiltrate.
蕈样肉芽肿（MF）和假性MF（或MF模拟物）可能与B细胞恶性肿瘤相关，但区分真正的肿瘤和反应性过程可能很困难。
报告7例伴有向毛囊性MF或假性MF的B细胞恶性肿瘤患者，并强调有助于区分这两种情况的标准。
我们回顾性和前瞻性纳入了7例伴有皮肤病变的B细胞恶性肿瘤患者，这些皮肤病变在组织学上表现为向毛囊性T细胞浸润，并复习了该主题的文献。
4名男性和3名女性患有慢性淋巴细胞白血病（n = 6）或黏膜相关淋巴组织型淋巴瘤（n = 1）。5例患者有局限性丘疹，2例有斑片和斑块。组织学检查显示，所有病例均有弥漫性真皮T细胞浸润，伴有向毛囊性累及和毛囊黏蛋白沉积，与B细胞淋巴瘤簇相关，滤泡辅助性T细胞标志物无明显表达。T细胞重排研究显示，丘疹患者呈多克隆模式，斑片和斑块患者呈单克隆模式。丘疹性病变进展缓慢，而斑片和斑块持续存在或恶化为转化型MF。
与B细胞恶性肿瘤相关的向毛囊性T细胞浸润可能是真正的向毛囊性MF或假性MF。区分这两种情况不能仅依靠组织病理学表现，还需要临床病理相关性以及寻找占主导地位的T细胞克隆。肿瘤性T细胞和B细胞是否源自共同祖先，或者潜在的B细胞淋巴瘤是否促进T细胞增殖仍未解决，但皮肤中B细胞和T细胞之间的相互作用似乎不依赖于T细胞浸润的滤泡辅助性T细胞分化。
