组织细胞坏死性淋巴结炎

患者女,48岁,左颈部淋巴结肿大、疼痛1周。抗炎治疗1周疗效不明显。皮肤科检查：左颈外侧乳突下方至锁骨可触及肿大的淋巴结,呈串珠状。活动度小,直径最大约2 cm,触痛明显,其他部位表浅淋巴结未见明显肿大。淋巴结组织病理：多种形态的组织细胞、免疫母细胞及浆细胞样单核细胞增生,伴有程度不同的核碎片。免疫组化：病变区CD3（+）、CD8（+）、CD68（+）、巨噬细胞炎症蛋白（+）、CD20（+）。诊断：组织细胞坏死性淋巴结炎病。     

成人型黄色肉芽肿1例

 报告成人型黄色肉芽肿1例。 患者男,27岁,左上肢黄色丘疹2年。皮肤科检查：左上肢见直径约3 mm淡黄色半球形丘疹,表面光滑。皮肤镜检查见橘黄色云状无结构区域。共聚焦显微镜检查：表皮轻度萎缩,皮损境界清楚,真皮内见大量周边高折光的盘状结构以及大量点状高亮结构。皮损组织病理检查：表皮轻度萎缩,真皮内见境界清楚的细胞团块,主要由组织细胞及少量炎细胞组成,组织细胞泡沫化,可见Touton巨细胞。免疫组化：CD68（+）,CD163（+）,S100（-）,CD1a（-）。诊断为成人型黄色肉芽肿。外科手术切除后随访6个月,未见复发。     

多中心网状组织细胞增生症 1例

患者,男，65岁。手部皮色丘疹结节伴关节痛3月余，组织病理示：真皮可见大量组织细胞和多核巨细胞，胞体大，胞浆丰富，部分胞浆红染，呈“毛玻璃”样。PAS染色阳性，免疫组化Vimentin（+），CD68（+），CD1a（-），S100（-）。诊断：多中心网状组织细胞增生症。经“强的松、甲氨蝶呤、雷公藤”联合口服治疗后皮疹和关节症状明显改善。     

有红斑表现的多中心网状组织细胞增生症

报告1例有红斑表现的多中心网状组织细胞增生症。患者男，67岁。因头面部，躯干四肢多发性红斑，结节1年就诊。皮损组织病理示真皮内可见结节状分布的组织细胞。组织细胞胞质丰富，细颗粒状（毛玻璃样），可见多核巨细胞。CD1a和S-100蛋白均为（-）；CD68（+）提示非朗格罕细胞来源。诊断为多中心网状组织细胞增生症。     

具有多种表现的蕈样肉芽肿一例

患者,男,80岁。手足反复出现多种皮损伴瘙痒、疼痛4年,加重1年。皮肤活检和组织病理示：表皮内及真皮浅层血管周围灶状淋巴样细胞浸润。免疫组化示：CD3（+）、CD4（+）、CD5（-）、CD7（+）、CD8（+）、CD20（+）,Ki67（10％）。诊断：蕈样肉芽肿。予以抗组胺药物、糖皮质激素药膏、窄谱中波紫外线照射治疗,随访。     

血管免疫母细胞性T细胞淋巴瘤1例

患者,男,64岁,躯干部丘疹1个月,皮疹逐渐增多,瘙痒明显,淋巴结肿大伴高热。淋巴结病理检查可见病理性核分裂象。免疫组化示：Ki-67标记指数约90%、CD21及CD23（示FDC网结构紊乱）、CD3及CD5（+）、CD34（血管+）、CD30（免疫母细胞+）、BCL-2（+）。 诊断：血管免疫母细胞性T细胞淋巴瘤。     

儿童色素减退型蕈样肉芽肿一例并文献复习

报告儿童色素减退型蕈样肉芽肿一例并对相关文献进行复习。患儿,男,7岁。周身皮肤出现色素减退斑、红斑结痂1年,无明显瘙痒。组织病理检查：角层轻度角化,表皮轻度增生,可见淋巴样细胞亲表皮现象,真皮内可见团块状样细胞浸润。免疫组化示：CD3(+);CD20(个别+);CD21（-）;Ki-67（+10%）;CD2（+）;CD5（+）;CD7（+）;CD8（少许+）;CD4（+）。诊断：色素减退型蕈样肉芽肿。给予患儿NB-UVB治疗3个月皮损基本消退,目前维持治疗中。     

早期隐性梅毒对慢性HIV感染者CD4+T及CD8+T淋巴细胞内Caspase-1/3水平的影响

目的：明确早期隐性梅毒感染对HIV感染者CD4+T及CD8+T细胞Caspase-1/3水平的影响。方法：流式细胞仪检测18例早期隐性梅毒合并慢性HIV感染者（合并组）以及18例单纯慢性HIV感染者（HIV组）CD4+T及CD8+T细胞内Caspase-1(细胞焦亡标志物)和Caspase-3(细胞凋亡标志物)含量。驱梅治疗6个月后再检测合并组CD4+T及CD8+T细胞内Caspase-1/3含量,与治疗前进行比较。结果：与单纯HIV感染组比较,合并组CD4+T及CD8+T细胞中Caspase-1升高（均P＜0.05）,Caspase-3含量无统计学差异（均P＞0.05）。与治疗前比较,合并组驱梅治疗6个月后,CD4+T及CD8+T细胞中Caspase-3明显下降（均P＜0.05）,Caspase-1含量治疗前后无统计学差异（均P＞0.05）。结论：合并梅毒螺旋体感染可能影响HIV感染者CD4+T和CD8+T淋巴细胞的焦亡和凋亡。     

程序性死亡分子1及其配体1在尖锐湿疣患者外周血T淋巴细胞的表达及意义

目的：研究程序性死亡分子1（PD?1）及其配体1（PD?L1）在尖锐湿疣患者外周血T淋巴细胞表面的表达,探讨PD?1/PD?L1信号通路在尖锐湿疣患者细胞免疫中的作用。方法采用流式细胞仪检测30例尖锐湿疣患者和20例健康对照者外周血CD4+、CD8+T淋巴细胞表面PD?1、PD?L1的表达及CD4+、CD8+T淋巴细胞计数,采用双抗体夹心酶联免疫吸附测定法检测血清中白细胞介素2（IL?2）,干扰素γ（IFN?γ）的水平,比较两组间的差异,分析尖锐湿疣患者外周血T淋巴细胞表面PD?1、PD?L1表达水平与CD4+、CD8+T淋巴细胞计数、细胞因子IL?2、IFN?γ的相关性。结果尖锐湿疣组CD4+、CD8+T淋巴细胞PD?1表达水平（分别为9.48%±3.31%和12.52%±3.17%）、PD?L1表达水平（4.40%±1.46%、7.07%±2.23%）分别高于健康对照组（PD?1：7.12%±2.16%、9.95%±2.17%,t=2.81、3.16,均P<0.01;PD?L1：3.26%±1.13%、5.39%±1.69%,t=2.96、2.88,均P<0.01）。尖锐湿疣组CD4+T淋巴细胞计数（727.43±138.59个/μl）低于对照组（804.25±92.83个/μl,t=2.17,P<0.05）, CD8+T淋巴细胞计数与对照组差异无统计学意义（t=1.24,P>0.05）,CD4+/CD8+比值（1.23±0.35）低于对照组（1.46±0.34,t=2.24,P<0.05）。尖锐湿疣组血清IL?2、IFN?γ水平均低于对照组（t=2.12、2.16,均P<0.05）。相关分析显示,尖锐湿疣患者外周血CD4+T淋巴细胞表面PD?1、PD?L1表达水平分别与CD4+T淋巴细胞计数、CD4+/CD8+比值、IL?2、IFN?γ水平均呈负相关（P<0.05）;CD8+T淋巴细胞表面PD?1、PD?L1表达水平与CD4+/CD8+比值均呈负相关（P<0.05）,与CD8+T淋巴细胞计数无相关性（P>0.05）。结论尖锐湿疣患者外周血T淋巴细胞高表达的PD?1可能通过与其配体PD?L1作用形成PD?1/PD?L1信号通路,抑制T淋巴细胞免疫应答,导致尖锐湿疣患者CD4+T淋巴细胞数量减少,CD4+/CD8+比值下降,IL?2、IFN?γ减少。     

以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤一例

患者男,51岁,上腹部胀痛4个月,颈项、躯干、双大腿结节、斑块1个月。当地多家医院行胃肠道相关检查,并予对症处理,治疗效果不佳。腹部皮肤结节组织病理检查：真皮下层和皮下脂肪小叶内大量增生的小血管腔内填塞中等偏大异形淋巴样细胞;免疫组化标记：血管腔内异形细胞CD2（+++）、CD4（-）、CD8（-）、CD3ε胞质（++）、CD99（++）、CD43（+++）、CD56（+++）、细胞毒颗粒相关蛋白（TIA-1）（++）、穿孔素（++）、EBER（+++）、CD20（-）、CD79a（-）、CD30（-）、细胞角蛋白（-）、S100（-）、CD68（-）、血管内皮细胞CD31（+）、CD34（+）。诊断：以胃肠道症状首发伴有皮肤表现的血管内NK/T细胞淋巴瘤。给予环磷酰胺、长春新碱、柔红霉素、地塞米松 + 依托泊苷方案化疗,病情得到迅速控制,目前仍在随访中。     

Tissue histiocyte reactivity with CD31 is comparable to CD68 and CD163 in common skin lesions

CD31 is a standard immunostain for evaluating vascular lesions of the skin, but CD31 reactivity for histiocytes is reported in only a small variety of pathological conditions. CD68 and CD163 are well recognized stains for cutaneous histiocytic lesions. We compared immunostaining of CD31 within that of CD68 and CD163 in five cases each of cutaneous lesions containing histiocytes: healing biopsy site, granuloma annulare, xanthogranuloma, ruptured follicular cyst and sarcoidosis. Reactivity was graded on a scale of 0–3 for brightness of immunostaining. Immunoreactivity was seen in histiocytes in all specimens for CD31, CD68 and CD163. The average intensity of staining was 1.7–2.5 for CD31, 2.6–3 for CD68 and 2.9–3 for CD163. The staining was somewhat less for CD31 because the reactivity is localized on the cell surfaces, whereas CD68 and CD163 react with cell surfaces and cytoplasm. We conclude that histiocytes in cutaneous lesions stain for CD31 and the staining is comparable to, but less intense, than that seen with CD68 and CD163. Caution is suggested in interpretation of CD31 staining in skin specimens, as CD31 shows reactivity with histiocytes as well as endothelial cells.     

沙利度胺成功治疗皮肤Rosai-Dorfman病一例

患者,女,53岁。面部暗红色和淡黄色丘疹、结节1年。组织病理示：表皮正常,真皮全层可见致密的组织细胞浸润,伴散在淋巴细胞、浆细胞及中性粒细胞浸润,可见伸入运动。免疫组化染色：S-100(++)、CD68(-)、CD1a(-)。综上诊断为皮肤Rosai-Dorfman病。予沙利度胺治疗,6个月后皮疹基本消退。     

皮肤Rosai-Dorfman病1例

患者男,28岁。左小腿内侧暗红色结节4月。皮损组织病理示:示真皮全层及皮下组织中有大量组织细胞浸润,其间有淋巴细胞及浆细胞,并可见组织细胞内吞噬淋巴细胞及浆细胞。免疫组化结果示淡染的组织细胞S-100蛋白阳性、CD68阳性。诊断:皮肤Rosai-Dorfman病。     

Subcutaneous panniculitis-like T-cell lymphoma in a 26-month-old child with a review of the literature

Subcutaneous panniculitis-like T-cell lymphoma is a rare cytotoxic T-cell lymphoma of the skin that preferentially infiltrates the subcutaneous tissue. We report here this lymphoma occurring in a 26-month-old Korean girl. She presented with multiple erythematous subcutaneous nodules on both extremities and her back along with systemic symptoms. She had a protracted course of multiple erythematous subcutaneous nodules for 1 month and a spiking fever was often noted. The histopathologic findings for the subcutaneous nodules were lobular panniculitis-like material that was composed of atypical lymphocytes and histiocytes. The atypical lymphocytes characteristically rimmed individual fat cells in a lace-like pattern and some of the histiocytes showed phagocytosed white blood cells occasionally. Immunophenotypic studies showed CD3(+), CD45RO(+), CD20(-), CD4(-), CD8(+), and CD56(-). She is currently being treated with combination chemotherapy of cyclophosphamide, doxorubicin, vincristine, and prednisolone.     

Rosai-Dorfman Disease with Massive Cutaneous Nodule on the Shoulder and Back

Rosai-Dorfman disease is a rare, idiopathic, benign, and self-limiting histiocytic proliferative disorder. A 26-year-old man presented with a single massive cutaneous nodule (reaching 30 cm in diameter) on the left shoulder and back for 15 months. The routine hematological and biochemical tests were normal. Magnetic resonance scanning showed the lesion involved the skin, subcutaneous tissue, and subjacent muscle group, accompanied by obvious lymph node enlargement in the left part of the neck, supraclavicular fossa, and axillary fossa. The histopathology of the left cervical lymph node revealed diffuse effacement of the normal nodal architecture, with patchy chronic inflammatory cell infiltrates comprising lymphocytes and sheets of histiocytes. Some histiocytes contained lymphocytes within their pale cytoplasm. Many multinucleated giant cells were found; however, caseating granulomas were not seen. The skin and muscle biopsy specimen obtained from the back revealed infiltrating lymphocytes and histiocytes diffusely distributed in the dermis, subcutaneous tissue, and crevices of the muscle fibers. The phenomenon of emperipolesis and the presence of multinucleated giant cells were also seen. Immunohistochemical staining revealed that the histiocytes were positive for S-100 protein and CD68 but negative for CD1a. Immunophenotyping of the infiltrating lymphocytes indicated positive reactions to CD3, CD45RO, CD5, CD7, CD4, CD8 (partly), CD79a, CD20 (partly), and Ki-67 (<1%). The final diagnosis was Rosai-Dorfman disease. Owing to the extensive and deep involvement of the subcutaneous tissue and muscles, the patient did not undergo surgery to excise the massive skin nodule. The lesion showed no obvious change at the 12-month follow-up.     

窦组织细胞增生伴巨大淋巴结病

报告1例窦组织细胞增生伴巨大淋巴结病.患者男,27岁.头皮、颈部、躯干部丘疹及结节半年,伴双侧颌下淋巴结肿大3个月.皮损组织病理检查示真皮内大量组织细胞增生,可见伸入运动.淋巴结组织病理检查示淋巴窦明显扩张,窦内可见大量组织细胞及伸人运动.免疫组化染色示S-100蛋白及CD68阳性,CD1a阴性.依据临床表现、组织病理改变和免疫组化检查,确诊为窦组织细胞增生伴巨大淋巴结病.     

Sinus histiocytosis (Rosai-Dorfman disease) clinically limited to the skin.

Sinus histiocytosis with massive lymphadenopathy (SHML) is an idiopathic proliferation of unique histiocytes that have vesicular nuclei and voluminous pale cytoplasm, often with emperipolesis. Pure cutaneous involvement is very rare. We describe a patient with SHML limited to the skin whose lesion has spontaneously regressed. A 35-year-old Korean male visited the Department of Dermatology due to facial rash for 2 months. A 3 x 3.5 cm-sized well-demarcated dark erythematous nontender plaque was noted on the right cheek. Skin biopsy showed dense, nodular infiltrates of histiocytes with abundant cytoplasm and vesicular nuclei rimmed by lymphoplasma cell aggregates throughout the upper and mid-dermis. The histiocytes were immunohistochemically positive for S-100 protein and CD68, but negative for CD1a. Laboratory tests and a thorough physical examination revealed no abnormalities. These findings suggested that this was a case of SHML clinically limited to the skin. The skin lesion was initially resistant to steroid therapy, but began to regress 10 months after the onset without further treatment.     

皮肤巨淋巴结病性窦组织细胞增生症

报告1例皮肤巨淋巴结病性窦组织细胞增生症。患者女,47岁。面颈部无痛性棕黄色斑块3年,无淋巴结增大及其他系统受累。皮损组织病理检查显示真皮全层及皮下组织中有大量组织细胞浸润,其间有淋巴细胞及浆细胞,并可见组织细胞内吞噬淋巴细胞及浆细胞。免疫组化染色结果示组织细胞S-100蛋白、DC68均阳性。皮损组织分枝杆菌PCR扩增、分枝杆菌培养及真菌培养均为阴性。诊断：皮肤巨淋巴结病性窦组织细胞增生症。     

Epithelioid cell histiocytoma--histogenetic and kinetics analysis of dermal microvascular unit dendritic cell subpopulations

BACKGROUND: Epithelioid cell histiocytoma (ECH), also known as epithelioid fibrous histiocytoma, is a peculiar dermal tumor, which can mimic melanocytic, vascular, epithelial, or other histiocytic lesions. Thought to arise from dermal dendrocytes, most ECH contain approximately 50% FXIIIa+ histiocytic dendrocytes, but not all lesional cells express FXIIIa. A putative fibroblastic component has not been characterized. METHODS: We analyzed the differentiation and cell kinetics of dermal microvascular unit cells in 12 previously reported ECH using antibodies to FXIIIa, CD68 (KP1), CD34, CD117, CD31, smooth muscle actin, collagen type 1 aminopropeptide, and MIB-1, using single and double immunostains. RESULTS: In ECH, many variably sized CD34/CD31+ tumor vessels with actin+ myopericytes were surrounded by epithelioid-to-dendritic cells of three types. About 5-80% were dendritic histiocytes that expressed FXIIIa but not CD31 or KP1. Fibroblasts, in some cases showing mild nuclear pleomorphism, were usually collagen type 1+, but CD34 and actin- in 11/12 cases. One 'early' ECH had 40% CD34+ epithelioid cells, admixed with 50% FXIIIa+ histiocytes. Most ECH had about 2-20% KP1+, CD117+ mast cells. Mast cell numbers increased with FXIIIa+ histiocyte numbers and the intensity of FXIIIa expression. MIB-1/FXIIIa double-labeling showed only rare cycling histiocytes, with numerous cycling fibroblasts and endothelial cells. CONCLUSIONS: Our findings support the impression that ECH is a vascular fibrous histiocytoma. The constituent cells appear to arise from the activation of resident microvascular CD34+ dermal fibroblasts and the accumulation of FXIIIa+ dendritic stromal assembly histiocytes. The CD34+ cells appear to differentiate toward collagenous fibrocytes in association with histiocytes and mast cells in forming collagenous stroma and vessels. ECH is a tumor composed of all requisite cell types consistent with the origin from the dermal microvascular unit.     

CD1a、CD68在继发性瘢痕疙瘩中的表达及意义

目的 探讨继发性瘢痕疙瘩皮损中表皮朗格汉斯细胞（LC）和真皮CD68阳性组织细胞的分布和密度。方法 取30例继发性瘢痕疙瘩患者的皮损、14例正常人皮肤组织切片进行CD1a和CD68免疫组化染色。以测微尺标定目镜方格计数方格内阳性细胞数,计算出单位面积内细胞的密度。组间比较采用SPSS软件进行 Student t检验。结果 在继发性瘢痕疙瘩表皮内CD1a阳性LC密度为（61 ± 49）个/mm2,正常表皮为（258 ± 61）个/mm2,两组比较,t = 9.88,P ＜ 0.01;继发性瘢痕疙瘩真皮CD1a阳性细胞密度为（40 ± 65）个/mm2。继发性瘢痕疙瘩表皮中无CD68阳性细胞,真皮内CD68阳性组织细胞密度为（287 ± 73）/mm2,正常皮肤为（290 ± 22）个/mm2,两组比较,t = 0.02,P ＞ 0.05。继发性瘢痕疙瘩真皮浅层CD68阳性组织细胞占真皮中所有细胞的62% ± 12%,而正常皮肤为70% ± 14%,两组比较,t = 2.66,P ＜ 0.05。 结论 继发性瘢痕疙瘩表皮中LC减少,无CD68阳性的细胞。真皮中LC增多;真皮浅层CD68阳性组织细胞占真皮中所有细胞的比例下降。