Department of Dermatology, Asahikawa Medical College, Asahikawa, Japan.
J Dermatol. 2011 Apr;38(4):368-72. doi: 10.1111/j.1346-8138.2010.00998.x. Epub 2010 Sep 28.
Primary cutaneous γδ-T-cell lymphoma (CGD-TCL) is a rare entity of cutaneous T-cell lymphomas (CTCL) and is characterized by tumoral growth of mature γδ-T-cell expressing cytotoxic molecules. The prognosis of CGD-TCL is generally worse than other CTCL. However, relatively indolent patch/plaque lesions have been described suggesting the heterogeneous nature of this entity. Here, we present a case of CGD-TCL with various skin manifestations, such as erythematous plaques/tumors and subcutaneous panniculitis-like lesions. During the follow up, testicular involvement was detected, which was surgically removed. Histopathology showed mixed features from epidermotropism, dermal infiltration and subcutaneous panniculitis-like lesions depending on the clinical manifestations. The tumor cells were positive for CD3 and revealed cytotoxic markers, TIA-1 and perforin, but not for CD4, CD8, CD20, CD56, TCRβF1 or EBER. Topical glucocorticoid ointment, narrowband ultraviolet B (NB-UVB) irradiation and low-dose methotrexate (MTX) were effective to control these skin lesions. No visceral involvement was detected thereafter. While CGD-TCL is usually associated with poor prognosis, it seems to be composed of various clinical manifestations, and NB-UVB and low-dose MTX could be a choice for indolent patch/plaque and possibly nodular lesions, especially for the aged.
原发性皮肤 γδ-T 细胞淋巴瘤(CGD-TCL)是皮肤 T 细胞淋巴瘤(CTCL)的一种罕见类型,其特征是表达细胞毒性分子的成熟 γδ-T 细胞的肿瘤性生长。CGD-TCL 的预后一般比其他 CTCL 差。然而,已经描述了相对惰性的斑块/斑块病变,表明该实体具有异质性。在这里,我们报告了一例具有多种皮肤表现的 CGD-TCL,如红斑性斑块/肿瘤和皮下脂膜炎样病变。在随访过程中,检测到睾丸受累,并进行了手术切除。组织病理学表现根据临床表现从表皮亲嗜性、真皮浸润和皮下脂膜炎样病变混合存在。肿瘤细胞 CD3 阳性,并表达细胞毒性标志物 TIA-1 和穿孔素,但不表达 CD4、CD8、CD20、CD56、TCRβF1 或 EBER。局部糖皮质激素软膏、窄带紫外线 B(NB-UVB)照射和低剂量甲氨蝶呤(MTX)对控制这些皮肤病变有效。此后未发现内脏受累。虽然 CGD-TCL 通常与预后不良相关,但它似乎由各种临床表现组成,NB-UVB 和低剂量 MTX 可能是治疗惰性斑块/斑块和可能的结节性病变的选择,特别是对于老年人。