种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤1例

患者女,17岁。全身反复起丘疹、水疱、坏死、凹陷状瘢痕伴瘙痒、发热15年,四肢起肿块2年。血清抗EBV-IgM(-),抗EBV-IgG(+)。肿块处皮损组织病理示真皮中下层和皮下组织见弥漫性致密的瘤细胞浸润,细胞核呈间变性;免疫组化示CD3(+),浸润的大细胞CD30(+),CD43(+),80%浸润细胞Ki-67(+)。水疱处皮损组织病理示表皮网状变性及多个水疱,真皮和皮下组织可见血管和附属器周围以淋巴细胞为主的、伴少量嗜酸粒细胞浸润,部分浸润细胞呈明显异形性;免疫组化示CD3(+),CD30(-),CD43(+),Ki-67(+)。诊断:种痘样水疱病样T细胞淋巴瘤伴发原发性皮肤CD30阳性大细胞淋巴瘤。确诊后建议患者转肿瘤科化疗,随访中。     

原发性皮肤弥漫大B细胞淋巴瘤(腿型)1例

患者男,89岁。左小腿结节、肿块2个月。皮肤科情况:左小腿伸侧可见一鸡蛋大小肿块,中央破溃、结痂。周边可见多个红色结节,质硬,伴轻度压痛。皮损活检示:真皮弥漫性淋巴样肿瘤细胞浸润,不累及表皮,与表皮间可见无浸润带。免疫组织化学示:CD3(-),CD20(+),CD10(-),CD21(-),BCL-2(+),BCL-6(-),MUM-1(+),Ki-67(80%~90%+),CD30(-),CD79a(+),EBER(-),ALK(-)。诊断:原发性皮肤弥漫大B细胞淋巴瘤(腿型)。     

Sézary综合征累及骨髓1例并文献复习

患者男,90岁,全身红皮病,伴顽固性瘙痒和脱屑3+年,淋巴结肿大和白细胞升高1+月。外周血淋巴细胞数达41.83×109/L。外周血流式细胞免疫荧光分析结果:T淋巴细胞占有核细胞总数约为56.5%,免疫表型为CD2(+),CD3(+),CD5(+),CD4(+),CD8(-),CD7(表型丢失),CD4∶CD810。TCRγ基因重排检测(片段分析)检测到单克隆重排。骨髓穿刺活检组织病理检查示:间质内见散在脑回状细胞(Sézary细胞)浸润,免疫表型为CD2(弱+),CD3(弱+),CD4(弱+),CD8(-),CD5(弱+),CD7(表型丢失),CD43(弱+),Ki-67增殖指数约35%。病理诊断为Sézary综合征累及骨髓。     

原发性系统型间变性大细胞淋巴瘤1例

患者男,52岁。颈部红斑1月余伴颈部多发淋巴结肿大。皮损组织病理示:真皮及表皮浅层水肿,弹力纤维撕裂,并可见较多急、慢性炎细胞浸润;皮损免疫组织化学染色:CD30(-),ALK(-),EMA(-),Ki-67(+5%)。颈部淋巴结病理诊断:间变性大细胞淋巴瘤;淋巴结免疫组织化学染色瘤细胞:CD2(+),CD3(+),CD30(+),ALK(+),CD4(2+),CD5(2+),CD43(2+),EMA(3+),Ki-67(+80%)。诊断:原发性系统型间变性大细胞淋巴瘤。     

种痘样水疱病样淋巴组织增殖性疾病1例及其文献分析

患者女,17岁。面部红斑、丘疹、水疱伴疼痛十年,加重20d,皮损消退部位遗留痘疮样瘢痕。面部皮损组织病理示:表皮海绵水肿,可见坏死,真皮血管及附属器周围淋巴细胞浸润,可见异型淋巴细胞。免疫组化示:CD3(+),CD4(+/-),CD5(-),CD7散在(+),CD8(+),CD45RO(+),Granzyme B (+),TIA-1 (+),CD20 (-),CD30 (-),CD56(-),EMA(-),ALK(-)。EBER原位杂交(+)。TCR基因重排:未检测到该样本TCR基因克隆性重排。诊断:种痘样水疱病样淋巴瘤(HVLL,该病后更名为种痘样水疱病样淋巴组织增殖性疾病)。进一步对国内外文献报道中180例HVLL病例进行分析,简要综述该病的研究历史、流行病学特点、可能发病机制、临床与病理表现以及诊疗进展。     

累及中枢神经系统的皮肤CD30~+间变性大细胞淋巴瘤1例

患者男,75岁,反复皮肤肿块11年。1个月前第3次复发伴语言、记忆和生活自理能力减退。头颅MRI示:左额叶,右顶叶多发占位,颅内淋巴瘤浸润。左大腿肿块组织病理示:真皮内大量异型细胞,体积较大,形态不规则,核扭曲,核分裂相可见;免疫组化:CD3(+),UCHL-1(+),CD30(+),ALK(-),MIB-1>90%。诊断:皮肤CD30+间变性大细胞淋巴瘤。中枢神经系统(central nerve system,CNS)累及可以是皮肤间变性大细胞淋巴瘤发生皮外累及的唯一部位,患者的意识改变是进行CNS筛查的重要提示。     

以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状的间变性大细胞淋巴瘤1例

患儿女,7岁,以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状,半年后左下肢出现皮下肿物确诊为间变性大细胞淋巴瘤。患儿首发临床表现为全身皮肤水疱、溃疡、结痂伴发热、腹股沟淋巴结肿大。皮损组织病理:表皮可见角化不良细胞,界面改变,表皮少量淋巴细胞浸润,未见异型细胞;淋巴结病理:淋巴结大片坏死,其间血管壁坏死,周围绕以多数淋巴样细胞;肿物病理:大量淋巴样细胞弥漫分布。免疫组织化学:CD30(+),ALK胞浆(+),CD5(-),CD7(+),CD4(+),CD8(-),TIA-1(+),CD2(+),LCA(+),EMA(+),CD3(-),Vimentin(+),Ki-67(>80%+)。诊断:间变性大细胞淋巴瘤。     

CD56~+,ALK~+间变性大细胞淋巴瘤广泛累及皮肤1例

患者男,26岁。入院前4个月无明显诱因于颈项部、左侧鼻根部、肘窝出现数个暗红色、蚕豆大结节,无明显自觉症状,部分结节可自行消退。2个月后结节增多、扩大,累及躯干、四肢,部分表面溃烂。实验室检查示:血清LDH升高,EBV-Ig G(+),EBV-DNA定量﹤5.00+02 copies/m L(-)。外周血涂片及骨髓穿刺未见异常。彩超示:双侧锁骨上窝、腋窝、腹股沟及左侧腘窝淋巴结肿大。PET-CT示:右侧上颌窦、右肺上叶、心包右缘软组织结节影代谢活跃;左侧锁骨上区、纵膈主动脉弓旁、胸骨旁、右侧腋窝、腹腔及右侧腹股沟多发淋巴结肿大代谢活跃;全身皮下、肌间多发软组织结节或肿块影代谢活跃。皮肤结节组织病理示:真皮全层及皮下组织致密单一核细胞浸润,可见异型细胞及不典型核分裂相。免疫组织化学:CD20(-),CD3(+),CD43(+),CD30(+),ALK(+),EMA(+),CD56(+),TIA-1(+),Granzyme B(+),CD15(-),MPO(-),CK(-),Ki-67(80%+)。纵隔淋巴结活检与上述结果一致。诊断:ALK阳性间变性大细胞淋巴瘤,Ⅳ期。确诊后转入肿瘤病区治疗,患者随访中。     

原发性皮肤弥漫大B细胞淋巴瘤1例

患者男,58岁。右颈肩部肿物7个月,加重3周。皮肤科情况:右颈肩部见20cm×20cm大小肿块,质地中等度硬,未触及疼痛。皮损组织病理示:真皮中部至皮下脂肪组织内见密集淋巴样细胞浸润,细胞体积较大,胞浆丰富,核圆形、卵圆形或不规则形,核膜厚,染色质粗颗粒状,核仁明显,核分裂可见。免疫组化显示:大淋巴样细胞CD20弥漫(+),CD79a,BCL6,MUM1(+),CD3,CD4,CD8散在(+),CD56,CD30,CD10,BCL-2(-),Ki67(+)约80%。诊断原发性皮肤弥漫大B细胞淋巴瘤。经8个疗程CHOPE方案化疗及1个月放疗,皮肤肿块完全消退。随访5年未见复发。     

误诊为坏疽性脓皮病的结外NK/T细胞淋巴瘤(鼻型)1例

患者女,38岁。全身红斑、坏死、溃疡伴疼痛发热十月余,加重1个月。皮损表现为小腿部位多发红斑、结节、溃疡及坏死,多次行皮损组织病理检查误诊为"坏疽性脓皮病"。1个月前皮损累及面部,再次行病理活检示:真皮小血管及神经纤维周围伴多量淋巴样细胞浸润,皮下组织并见脂膜炎反应。免疫组化:CD2(+),CD3(+),TIA-1(+),Gr B(+),CD56(+),Ki67[约90%+]及合并EBER(+);TCR无克隆性基因重排。诊断:结外NK/T细胞淋巴瘤(鼻型)。     

The pathology of the eye in armadillos experimentally infected with Mycobacterium leprae

One hundred and twenty-seven eyes from 66 Mycobacterium leprae inoculated armadillos were studied histologically and some ultrastructurally. Inflammatory reactions were found in the following extraocular tissues: the eyelid, including the orbicularis muscle and the third eyelid, extraocular muscles, tear gland and Harder's gland. The early and slight changes of the intraocular tissues, small amounts of lymphocytes, plasma cells and macrophage infiltrations were confined to the area around the anterior angle specifically within the trabeculae and the adjacent ciliary body, the root of the iris and the limbus region of the cornea. But in the cases with severe lesions the whole uvea was densely infiltrated with large, foamy macrophages intermingled with small amounts of lymphocytes, plasma cells and frequently, neutrophils. No specific necrosis of the granulomas was seen. No explanation for the neutrophil infiltrations was given. The lesions in the cornea were significantly less severe than those in the uvea. Retinal lesions comprised of macrophage infiltrations were all obvious extensions of the adjacent uvea lesions. Acid-fast bacilla (AFB) were found within all tissues. The infection of the intraocular tissues in the armadillo eyes seemed to be mainly, if not solely, haematogenous.     

A Unique Association of Unilateral Idiopathic Calcinosis Cutis with Ipsilateral Porokeratotic Eccrine Ostial and Dermal Duct Nevus

An 11‐year‐old boy presented with complaints of multiple skin‐colored hard lumps on the right side of his body and progressive deformity of the right leg of 7‐years duration. His parents had also noticed multiple asymptomatic pits over his right arm, palms, and soles since childhood. Examination revealed skin‐colored nontender nodules on the right half of his body and shortening of his right leg. The multiple hyperpigmented pits over the right arm, palm, and sole raised diagnostic difficulties, but histopathologic, radiologic, and biochemical investigations confirmed the features of idiopathic calcinosis cutis and porokeratotic eccrine ostial and dermal duct nevus. Unilateral idiopathic calcinosis cutis has not been previously reported in the literature, and the association with ipsilateral porokeratotic eccrine ostial and dermal duct nevus makes this case unique. Diagnostic difficulties and limited options for treatment make this case interesting academically.     

泛发性毛囊角化病1例

患者男,22岁。自6岁始面部出现针尖至米粒大灰褐色或近正常肤色毛囊性散在分布的坚实小丘疹,逐渐增多、变大,泛发全身。部分融合成斑块,可见疣状增生。左肩部皮损组织病理示:角化不全,毛囊角栓形成,可见圆体和谷粒、裂隙和馅窝,有绒毛突入馅窝,表皮乳头瘤样增生,慢性炎症细胞浸润。诊断:毛囊角化病。     

Pathogenesis of Mucocutaneous Lesions in Behçet's Disease

Behcet's disease (BD) is characterized by recurrent oral aphthae, skin lesions, eye lesions, and genital ulceration. To determine the pathogenesis of BD, we performed histological and immunohistochemical studies of these mucocutaneous lesions, an assay of neutrophil activity, and HLA typing. Dense dermal or subcutaneous infiltrations of polymorphonuclear cells (PMN) without leukocytoclastic vasculitis were found in 28 of 57 lesions. Immunohistochemically, deposits of C3 on the vessels were found in 12 of 31 lesions. Deposits of immunoglobulin were not found except for one of IgM. C3 deposits and PMN infiltrations were significantly related (p<0.05). PMN activity by polarization was enhanced; however, the results did not show a significant relationship with the PMN infiltrations or the C3 deposits. The incidence of HLA-B51 was significantly high in BD, but no significant relationship was found between HLA-B51 and the results of other examinations. These results suggest that the pathogenesis of BD lesions differs from that of collagen diseases and that C3 deposits on the vessels may play an important role in the development of mucocutaneous lesions where PMN have mainly infiltrated.     

Congenital Self-Healing Reticulohistiocytosis: Report of a Patient with a Strikingly Large Tumor Mass

A newborn male Thai infant had numerous brownish red nodules of various sizes scattered over both palms and soles, left thigh, abdomen, chin, and left upper eyelid. An extremely large tumor mass was present on the right sole. Many lesions showed spontaneous ulceration. No extracutaneous involvement was found. All lesions involuted spontaneously within two to three months, some with scar formation. Histology showed large numbers of mononucleated and multinucleated histiocytic cell infiltrations, 10% of which contained Birbeck's granules. Areas of necrosis and calcification were also seen in the largest tumor. No recurrence was observed after follow-up for three years.     

皮下脂膜炎样T细胞淋巴瘤1例

患者女,19岁。左上臂外侧结节、溃疡4个月,泛发全身伴发热2月,加重20天。皮损组织病理示脂肪小叶及小叶间隔内见淋巴细胞弥漫性浸润性生长,肿瘤细胞体积较小,核深染,核仁可见,围绕单个脂肪细胞浸润形成"花环状"结构,并见细胞吞噬现象,脂肪组织坏死伴炎细胞浸润,肿瘤未侵犯真皮和表皮。免疫组化:CD3(+),CD8(-),CD45RO(+),CD20(-),CD56(+),Ki-67阳性细胞大于90%。诊断:皮下脂膜炎样T细胞淋巴瘤。入院3天后因多器官功能衰竭合并败血症而死亡。     

Cutaneous extramedullary hematopoiesis in myelofibrosis

A 62-year-old woman with myelofibrosis developed bluish infiltrations on the trunk. Histologically, the lesions showed extramedullary hematopoiesis consisting of myeloid, erythroid, and megakaryocytic elements.     

Hyaluronidase offers an efficacious treatment for inaesthetic hyaluronic acid overcorrection

Background Hyaluronic acid is generally accepted today as the “gold standard” filler agent, and its use has subsequently grown enormously. In addition, newer facial volume augmentation indications are constantly evolving. Rare adverse events, such as granulomas, have been described. However, complications are more commonly due to product misplacement or overcorrection leading to unsightly lumps and masses. Hyaluronidase treatment of these latter adverse effects can be both effective and rapid. Objective This paper aims to confirm the efficacy of hyaluronidase injections in dissolving unsightly hyaluronic acid overcorrection. Methods A case of hyaluronic acid overcorrection is described with evaluation of the effects of hyaluronidase. Results The use of hyaluronidase, injected intracutaneously permits the elimination of patient discomfort and inaesthetic lumps within a few hours. Conclusions Hyaluronidase is highly effective in eliminating HA volume overcorrection.     

Combined Mastocytoma-hemangioma in a Patient with Urticaria Pigmentosa

A patient with long-standing urticaria pigmentosa presented with a pea-sized reddish to purplish papule on the posterior part of the right ear. Histopathologic examination revealed numerous dilated vascular structures in the upper dermis and mast cell infiltrations throughout the whole dermis, consistent with combined mastocytoma-hemangioma. The mast cells were strongly positive with Giemsa stain.     

Case of adult T-cell leukemia/lymphoma manifesting marked purpura

We present a case of a 62-year-old woman with marked purpura, first appearing on both legs, then spreading over the whole body, including the face. At presentation, the patient was thought to have Henoch-Schonlein purpura. However, a skin biopsy from a purpuric lesion revealed prominent infiltrations of atypical lymphocytes into the papillary dermis and marked extravasation of erythrocytes through the epidermis and upper dermis. Antibody to human T-lymphotropic virus type 1 (HTLV-1) was present in the serum and samples from skin lesions revealed HTLV-1 proviral DNA integration, as well as a clonal T-cell receptor Cbeta1 gene rearrangement. We therefore diagnosed this case as adult T-cell leukemia/lymphoma (ATL), and the purpuric lesions as ATL-specific. Soon after the initiation of chemotherapy, these purpuric lesions began to resolve with pigmentation.