成人朗格汉斯细胞组织细胞增生症1例

患者男,36岁。会阴部反复红斑、斑块及溃疡1年余。患者既往有垂体瘤及尿崩症病史17年。皮肤科情况:左腹股沟见小片状浸润性斑块,中央浅溃疡。皮损组织病理示:真皮浅层见弥漫性淋巴细胞、嗜酸性粒细胞及组织样细胞浸润。增生的组织样细胞轻度异型,部分胞核呈肾型。免疫组织化学示:CD1a(+),S-100(+),CD68(+),Ki-67(5%+)。诊断:成人朗格汉斯细胞组织细胞增生症。     

原发性皮肤CD30~+间变性大T细胞淋巴瘤1例

患者男,68岁。左臀部巨大溃疡伴离心性扩大10余年。皮肤组织病理检查:真皮全层可见弥漫性肿瘤细胞浸润。肿瘤细胞体积大,细胞异型性明显。细胞核染色质深,可见明显核分裂相。多核瘤巨细胞及R-S样细胞未见。免疫组化示肿瘤细胞约90%以上细胞CD30+染色阳性、CD3、CD4和LCA染色(+),而CD8、CD20、CD79α、TIA-1和ALK-1均为阴性。诊断为原发性皮肤CD30+间变性大细胞淋巴瘤。     

皮肤淋巴细胞浸润症1例

患者男,60岁,左股内侧斑块1年余增大伴破溃1个月。体检:左股内侧有一4 cm×8 cm红色浸润性斑块,质中,界清,中心破溃后形成直径1 cm,深约0.8 cm大小的溃疡。组织病理:真皮全层以淋巴样细胞为主的混合性炎细胞浸润。淋巴细胞核异型性明显,免疫组化:CD3(+),CD20(+),CD30(+),CD56(-)。确诊。     

泛发性原发皮肤CD30+间变性大细胞淋巴瘤

报告1例泛发性原发皮肤CD30+问变性大细胞淋巴瘤.患者男,44岁.因全身皮肤多发红斑、结节、溃疡50余天就诊,皮损组织病理及免疫组化检查结果提示间变性大细胞淋巴瘤,免疫组化检查:瘤细胞CD3(++),CD30(++),Ki-67约50%(+),CD20、Ckpan、S-100蛋白、HMB45、间变性淋巴瘤激酶(ALK)-1均阴性,诊断为间变性大细胞淋巴瘤.     

皮肤γ/δ T细胞淋巴瘤

报告1例皮肤γ/δT细胞淋巴瘤。患者女,65岁。全身出现结节、斑块、溃疡伴发热1个月。皮疹初起为红色结节,迅速增多、增大形成斑块,并破溃形成溃疡,自觉疼痛,伴间歇性发热和淋巴结肿大。皮损组织病理示表皮未见异常,真皮层内幼稚淋巴细胞呈多形性,广泛片状浸润,尤以皮肤附属器官、血管周围和胶原束之间浸润显著,肿瘤细胞明显异形。皮损免疫组化检查:CD3、CD2、CD45RO阳性,CD4、CD8、CD20、CD56、CD1O、CD30、T细胞抗原受体(TCR)βF1均为阴性。T细胞抗原受体TCRγ基因重排分析显示T细胞呈单克隆性增生,诊断为皮肤γ/δT细胞淋巴瘤。患者以环磷酰胺、吡柔比星、长春地辛、泼尼松、依托泊苷等药物化疗,病情部分缓解,停止治疗后仍复发、进展,最终于发病8个月后死亡。     

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。     

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)。     

原发性皮肤侵袭性亲表皮CD8+细胞毒性T细胞淋巴瘤1例

患者女，74岁，右背部红斑伴糜烂、溃疡8个月余，加重1个月。皮疹初起为红斑，迅速增大伴糜烂、溃疡。皮损组织病理示：真皮浅中层中小异型淋巴细胞弥漫浸润，免疫组织化学示：CD3(80%+)、CD8(80%+)、CD4(50%+)、CD30(背景细胞+)、CD20(背景B细胞+)、CD56(10%+)、CD138(-)、TIA-1(80%+)、Granzyme B(60%+)、ALK(-),Ki67(60%+)。T细胞基因重排：TCR-β(+)。诊断：原发性皮肤侵袭性亲表皮CD8⁺细胞毒性T细胞淋巴瘤。经8次CHOP化疗后患者皮损愈合，随访3个月后患者病情复发。     

原发性皮肤CD30~+间变性大细胞淋巴瘤

报告1例原发性皮肤CD30~+间变性大细胞淋巴瘤(PC-ALCL)。患者女,58岁。左侧大腿暗红斑及斑块1年余,背部红斑及斑块伴溃疡3个月余。背部皮损组织病理检查:表皮部分坏死,真皮至皮下大量胞质丰富的大淋巴样细胞成片浸润,细胞核呈间变性,其间可见中性粒细胞和嗜酸性粒细胞浸润。免疫组化示大淋巴样细胞强阳性表达CD30,阳性表达CD2、CD3、CD4和多发性骨髓瘤原癌基因(MUM)-1,部分表达CD5和T细胞内抗原(TIA)-1,Ki-67阳性率95%;而不表达CD8、CD20、CD79α、CD56、EB病毒编码RNA(EBER)和间变型淋巴瘤激酶(ALK)。结合临床及组织病理改变,诊断为PC-ALCL。     

CD8~+的淋巴瘤样丘疹病1例

患者女,38岁。全身反复红斑、斑片8年,丘疹、结节1年。全身多处泛发色素沉着斑及红色小丘疹,左腰部有一直径1cm大小的斑块。斑块处组织病理示:真皮及皮下弥漫大细胞,有异形性;免疫组化:异形细胞LCA(+)、CD3(+)、CD4(+)、CD8(+)、CD30(+)、GranzymeB(+),诊断为淋巴瘤样丘疹病,A型。该患者以红斑起病之后出现丘疹,但从未出现溃疡坏死,丘疹的大小和可自行消退的特点符合淋巴瘤样丘疹病,CD8+是LyP较为少见的免疫表型。该病应与皮肤CD30(+)间变性大细胞淋巴瘤和蕈样肉芽肿鉴别,需监测其预后及转归。     

原发性皮肤间变性大细胞淋巴瘤1例

报告1例原发性皮肤间变性大细胞淋巴瘤。患者女,49岁。右小腿结节1年,溃烂5个月。皮损组织病理检查:真皮内有密集的淋巴样细胞浸润,瘤细胞大、核呈肾形或不规则形、核分裂像多见,免疫组化示瘤细胞约70?30阳性、约20?45Ro阳性,而CD3、CD20、MPO、TIA-1、ALK-1均为阴性。诊断为原发性皮肤间变性大细胞淋巴瘤。     

CD34-reactive myxoid dermal dendrocytoma.

Normal skin is composed in part of cells that express CD34. These include periadnexal spindle cells, vascular endothelial cells, and interstitial dendritic cells. We report on a tumor composed mainly of CD34-reactive spindle cells. A 66-year-old Japanese woman presented with a skin-colored, dome-shaped, cutaneous papule on her left palm that was 7 mm in diameter and had developed within the preceding 3 months. Light microscopic examination showed a well-circumscribed polypoid tumor consisting of spindle-shaped cells and thin collagen fibers arranged loosely in a fascicular pattern within a myxoid matrix. Immunohistochemically, most of the tumor cells stained strongly for CD34, but did not stain with antibodies to S-100 protein, smooth muscle actin, desmin, neuron-specific enolase, epithelial membrane antigen, or factor XIIIa. Staining for vimentin and CD68 was positive. We believe this lesion to be a CD34-reactive myxoid dermal dendrocytoma of a type that has not been described previously.     

Cutaneous Presentation of Plasmablastic Post‐transplant Lymphoproliferative Disorder in a 14‐Month‐Old

Abstract: We present a 14‐month‐old female child who developed multiple erythematous nodules on her abdomen 5 months after liver and small bowel transplantation. Skin biopsy revealed a dense infiltrate of large cells in the dermal and subcutaneous layers with frequent mitotic figures. The cells were noted to have abundant cytoplasm, prominent nucleoli, and open chromatin. Immunohistochemical stains were positive for CD138, CD56, Ki67 (>90%), and lambda chain restriction. Rare mature B cells (CD20) and rare T cells (CD3) were noted. She was diagnosed with high‐grade post‐transplant lymphoproliferative disorder most consistent with plasmablastic lymphoma.     

皮肤Merkel细胞癌1例

患者女,63岁。发现右肘皮肤暗红色肿块3月。皮损组织病理示:肿块位于真皮呈巢状分布,肿瘤细胞大小形态较一致,胞质较少,略嗜碱性,胞核圆形,伴典型的细颗粒状(尘样)染色质及多个核仁,核分裂相及核碎片易见。免疫组化示:肿瘤细胞CK20,CHG,CD56,Syn均阳性,LCA阴性。诊断:右肘皮肤Merkel细胞癌。     

下肢肌周细胞瘤一例

患者,女,33岁。因右小腿皮肤丘疹30余年,伴红肿疼痛3个月。予以手术切除,术后病理示:瘤细胞以多层同心圆型围绕薄壁血管周围生长,部分可见血管管腔闭塞。免疫组化示:ERG(血管+),CD34(血管+),CD31(血管+),SMA(+),Desmin(-),H-caldesmon(+)。结合形态学及免疫组化考虑肌周细胞瘤。予以手术扩大切除后植皮。     

Cutaneous pleomorphic large cell lymphoma.

The case of a primary cutaneous pleomorphic large cell lymphoma occurring in a twenty-one-year-old woman who presented with a blue-reddish nodule on her left cheek of three months' duration is presented. The tumor consisted of pleomorphic blast cells showing high mitotic activity. On immunohistochemical examination, the majority of the tumor cells expressed CD 3 (Leu-4), CD 4 (Leu-3), HLA-DR CD 30 (Ki-1/Ber-H 2), and CD 25 (IL2 receptor). Twenty-two months after excision of the tumor there is no detectable systemic spread of the lymphoma. This case provides further evidence for recent observations that primary cutaneous Ki-1-positive large cell lymphomas without lymph node involvement may have a favorable prognosis after local treatment despite showing histologic pattern of malignancy.     

多中心网状组织细胞增生症1例

患者女,35岁。全身红斑、丘疹、结节伴瘙瘁7个月,双手指间关节、腕关节、肘关节肿痛2个月。肌电图示左三角肌、右股二头肌肌源性损害。右前臂皮疹组织病理示：真皮可见大量组织细胞和多核巨细胞,细胞体积大,胞浆丰富、嗜酸性,均质或细颗粒状,呈“毛玻璃”样,瘤细胞Vimentin（＋）,CD68（＋）,CD163（＋）,S-100（-）,CD1a（-）。诊断：多中心网状组织细胞增生症。经“甲泼尼龙、MrD（、羟氧喹”联合治疗后皮疹和关节症状明显改善．     

Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma

An 8-year-old Taiwanese girl had a 6-month history of a relapsing papulovesicular eruption on her face that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of large atypical lymphocytic cells expressing CD8. TCR-gamma gene rearrangement study revealed a monoclonal band present in the DNA extracted from the specimen. A diagnosis of CD8+ cutaneous T-cell lymphoma (CTCL) was made. The patient was treated with Chinese herbal drugs and her skin lesions waxed and waned. At this writing, 11 months after establishment of the diagnosis, the skin lesions have been limited to the facial area and no definite evidence of systemic involvement is noted. To our knowledge, this is the first case of CD8+ primary CTCL with clinical features resembling HV.     

Lymphomatoid papulosis with pseudocarcinomatous hyperplasia in a 7‐year‐old girl: a case report

Lymphomatoid papulosis (LyP) belongs to the group of cutaneous CD30+ lymphoproliferative disorders. Pseudocarcinomatous hyperplasia has rarely been reported in patients with LyP. In this report, we describe a case of LyP presenting as pseudocarcinomatous hyperplasia. The patient was a 7‐year‐old girl who presented with a recurrent papulonodular eruption on her face and trunk for 2 months. Histopathologic examination revealed an irregular growth of hyperkeratotic epidermis into the whole dermal layer with marked nests of squamous cells in the background of diffuse atypical lymphoid cells, eosinophils and neutrophils. The large atypical cells were positive for CD30 and CD3, but negative for CD4, CD5, CD8, CD20 and CD56. A TCR‐γ clone was identified by polymerase chain reaction (PCR). The correct diagnosis in cases of LyP with overlying pseudocarcinomatous epithelial hyperplasia can be very difficult both clinically and histopathologically. Clinical and histopathologic characteristics should be integrated to avoid an erroneous diagnosis of squamous cell carcinoma or keratoacanthoma.     

Extra nodal NK/T-cell lymphoma nasal type that responded to DeVIC combination chemotherapy

We report a 76-year-old woman with extra nodal NK/T-cell lymphoma nasal type (ENKL). She had large tumors on her left leg with inguinal lymphadenopathy and gastric tumors. The tumor cells showed angiocentric growth with necrosis. Immunohistologically, the tumor cells from the skin lesion expressed CD2, cytoplasmic CD3, CD56, and T-cell intracellular antigen-1 (TIA-1), but not surface CD3, CD19, and TdT. The gastric tumor cell, also expressed cytoplasmic CD3, CD45RO and CD56. She was diagnosed as having ENKL (stage IV of Ann Arbor). The tumors responded remarkably well to radiation therapy followed by multi-drug resistance independent DeVIC (carboplatin, etoposide, ifosfamide, and dexamethasone) combination therapy. After two series of this therapy, no tumors were detected in clinical, histopathological, endoscope and computerized tomogram (CT) examinations. However, she suddenly died of brain stem metastasis three months later. Although there may be a limitation of effects on metastasis of tumors in the central nervous system, radiation and DeVIC combination therapy is a potent therapeutic method for ENKL.