Nagore E, Llorca J, Sánchez-Motilla J M, Ledesma E, Fortea J M, Aliaga A
Department of Dermatology, Hospital General Universitario, Valencia, Spain.
Int J Dermatol. 2000 Aug;39(8):618-20. doi: 10.1046/j.1365-4362.2000.00022-2.x.
An 80-year-old man, with a past medical history of senile dementia, presented with a 6-month history of a solitary, gradually enlarging tumor, located on his chin. A squamous cell carcinoma had been surgically excised 30 years previously in the same location. Physical examination revealed an erythematous, well-defined plaque of 3 cm in diameter, located on the chin (Fig. 1). The submandibular lymph nodes were enlarged. Squamous cell carcinoma and primary cutaneous lymphoma were considered. Relevant laboratory findings were as follows: white blood cell count, 5.600/microL; eosinophils, 1000/microL; gammaglobulin, 2.4 g/dL; lactate dehydrogenase, 343 IU/L; and immunoglobulin G (IgG) antibodies to Epstein-Barr virus (EBV) positive (at 1 : 128 serum dilution), with negative IgM. Skin and lymph node biopsies were performed. Histopathologic study of the cutaneous specimen revealed a heavy lymphoid infiltrate with numerous lymphoid follicles, with prominent germinal centers involving the subcutaneous fat as well as the deep dermis and muscular fascia. Some germinal centers showed folliculolysis. The lymphoid follicles were surrounded by fibrous tissue. The interfollicular infiltrate was rich in plasma cells and eosinophils that formed scattered eosinophilic microabscesses. Thin-walled vessels were numerous and prominent, but with no epithelioid or vacuolated endothelial cells (Fig. 2). Histopathology of a lymph node biopsy specimen showed reactive lymphoid follicle hyperplasia, with prominent eosinophilic infiltrates in both follicular and interfollicular areas. Eosinophilic deposits and polykaryocytes of Warthin-Finkeldey type were seen in the germinal centers. The paracortical area showed vascular proliferation. Polymerase chain reaction (PCR) for the detection of specific sequences of EBV from routinely processed paraffin-embedded material was carried out under the conditions and with the same set of primers as described previously in detail (Tenorio A, Echevarría JE, Casas E et al. J Virol Methods 1993; 44: 261-269). DNA samples were confirmed to be amplifiable with PCR primers specific for a conserved region of the human beta-globin gene. Every sample was tested at least twice for EBV DNA and beta-globin gene. One sample from one skin lesion of the patient, with confirmed diagnosis of Kimura's disease, and 10 samples from normal skin biopsies retrospectively collected from other patients in archival files of our department were tested. Only the patient's specimen tested positive to EBV. The amplified product of EBV was analyzed using DNA sequencing and confirmed the results obtained. The patient received radiotherapy at doses of 35 Gy. Nevertheless, the tumor enlarged to reach twofold its original size 1 month later. Due to the physical status of the patient, no further treatments were considered, but the disease remained stable over the following 3 years.
一名80岁男性,有老年痴呆症病史,因下巴出现一个孤立的、逐渐增大的肿瘤6个月前来就诊。30年前曾在同一部位手术切除过鳞状细胞癌。体格检查发现下巴处有一个直径3厘米的红斑,边界清晰(图1)。下颌下淋巴结肿大。考虑为鳞状细胞癌和原发性皮肤淋巴瘤。相关实验室检查结果如下:白细胞计数5600/微升;嗜酸性粒细胞1000/微升;γ球蛋白2.4克/分升;乳酸脱氢酶343国际单位/升;抗Epstein-Barr病毒(EBV)的免疫球蛋白G(IgG)抗体阳性(血清稀释度为1:128),IgM阴性。进行了皮肤和淋巴结活检。皮肤标本的组织病理学研究显示有大量淋巴细胞浸润,有许多淋巴滤泡,生发中心突出,累及皮下脂肪以及深部真皮和肌肉筋膜。一些生发中心显示滤泡溶解。淋巴滤泡被纤维组织包围。滤泡间浸润富含浆细胞和嗜酸性粒细胞,形成散在的嗜酸性微脓肿。薄壁血管众多且明显,但无上皮样或空泡状内皮细胞(图2)。淋巴结活检标本的组织病理学显示反应性淋巴滤泡增生,滤泡区和滤泡间区均有明显的嗜酸性粒细胞浸润。生发中心可见嗜酸性沉积物和Warthin-Finkeldey型多核巨细胞。副皮质区可见血管增生。在与之前详细描述相同的条件下,使用相同的引物组对常规处理的石蜡包埋材料进行检测EBV特定序列的聚合酶链反应(PCR)(Tenorio A, Echevarría JE, Casas E等。《病毒学方法杂志》1993年;44:261 - 269)。DNA样本经证实可被针对人β-珠蛋白基因保守区域的PCR引物扩增。每个样本至少检测两次EBV DNA和β-珠蛋白基因。对该患者一个确诊为木村病的皮肤病变样本以及从我们科室档案中回顾性收集的其他患者正常皮肤活检的10个样本进行了检测。只有患者的样本检测出EBV阳性。对EBV的扩增产物进行DNA测序分析并证实了所得结果。患者接受了35 Gy的放疗。然而,1个月后肿瘤增大至原来的两倍。由于患者身体状况,未考虑进一步治疗,但在接下来的3年中病情保持稳定。