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患儿男,11岁,面部、手臂出现红斑、水疱4年,四肢肿块、溃疡4个月。逐渐感觉乏力,有不规则间歇发热。体检:体温37.7℃。全身淋巴结无明显肿大,口腔,颜面和鼻部皮肤片样轻度浸润的粉红色斑片,四肢见大小不等的暗红色浸润性结节和肿块,结节直径2.0～18cm不等,部分表面有坏死和黑色结痂,较大的结节表面形成溃疡,坏死性溃疡深达肌肉并有白色脓性分泌物。皮损边界清楚,质地较硬,有压痛,部分结节周围见到卫星样小结节。取自大腿的皮损病理检查发现,表皮真皮全层至皮下可见大量形态不一、中等大小的肿瘤细胞浸润,肿瘤细胞胞质清晰,核大,扭曲状,染色质丰富,细胞呈巢或散在,可见围管样分布;真皮和皮下见碎核和反应性组织细胞浸润,肿瘤细胞浸入毛囊和外泌汗腺。免疫组化:胞质CD3在核周区可见阳性染色,CD56、CD45RO和TIA-1强阳性(+++),部分肿瘤细胞CD30、CD8和Ki 67阳性(++)。EB病毒编码小核RNA(EBER)阳性,TCR-γ基因重排阳性。最后诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤,这是1例罕见的原发于皮肤长达51个月的进展缓慢的病例。
Children male, 11 years old, face, arm erythema, blisters 4 years, limbs, ulcers 4 months. Gradually feel tired, irregular intermittent fever. Physical examination: body temperature 37.7 ℃. No significant enlargement of the whole body lymph nodes, oral, facial and nasal skin patchy mild infiltration of pink patches, see the limbs ranging from dark red infiltrative nodules and lumps, nodules ranging in diameter from 2.0 to 18 cm, part Surface necrosis and black scab, the formation of larger nodules ulcers on the surface, necrosis of the deep muscular and white purulent secretions. Skin lesions clear, hard texture, tenderness, some of the nodules seen around the satellite-like nodules. Skin lesions from the thigh pathological examination found that a large number of epidermal dermis to the subcutaneous can see a large number of different forms of medium-sized tumor cell infiltration, clear tumor cell cytoplasm, nuclear large, twisted, rich chromatin, cells were nest or scattered , Visible around the tube-like distribution; see dermal and subcutaneous crushed nuclear and reactive cell infiltration, tumor cells immersed in hair follicles and excretory sweat glands. Immunohistochemistry: Cytoplasmic CD3 showed positive staining in the perinuclear region, strongly positive for CD56, CD45RO and TIA-1 (+++), and some tumor cells were positive for CD30, CD8 and Ki 67 (++). Epstein-Barr virus coding small nuclear RNA (EBER) positive, TCR-γ gene rearrangement positive. The final diagnosis of vaccinia-like blister-like skin NK / T-cell lymphoma, which is a rare case of primary skin up to 51 months of slow progression of the case.