组织样麻风瘤1例误诊分析

患者女,48岁。双前臂暗红色丘疹和结节半年,泛发全身伴瘙痒3个月。无麻风病家族史,曾被误诊为结节性痒疹和湿疹,治疗无效。皮损组织病理示:表皮萎缩,表皮突变平或消失;真皮内泡沫样组织细胞呈结节状浸润,少量淋巴细胞浸润。抗酸染色可见大量抗酸杆菌(4+)。诊断:组织样麻风瘤。     

血管内皮生长因子在结节性痒疹皮损表皮的表达

结节性痒疹皮损表皮增生明显，真皮毛细血管增多，管腔扩张。为探讨结节性痒疹是否表达血管内皮生长因子（ＶＥＧＦ），我们用抗ＶＥＧＦ抗体染色结节性痒疹皮损表皮的石蜡切片，同时用ＣＤ３４染色毛细血管，以观察与ＶＥＧＦ的可能相关性。材料和方法病例我院皮肤科门诊结节性痒疹患者１２例，切除皮损表皮，福尔马林固定，石蜡包埋备用；对照为我院整形外科色素痣患者手术切除所余边缘正常皮肤。免疫组织化学染色１．ＶＥＧＦ染色：采用ＨｉｓｔｏｆｉｎｅＳＡＢ—ＡＰ（Ｒ）试剂盒（日本Ｎｉｃｈｉｒｅｉ公司出品），按ＡＢＣ法进行。具…     

泛发性结节性痒疹1例

报告沙利度胺联合糖皮质激素治疗泛发性结节性痒疹1例。患者女,34岁。全身皮肤泛发丘疹、结节伴瘙痒近1年。皮损组织病理示:显著角化过度并角化不全,棘层、颗粒层增生肥厚,可见海绵水肿,皮突延长呈银屑病样或假上皮瘤样增生。真皮乳头层增厚,增粗红染的胶原纤维与表皮垂直走行,真皮浅层血管周围淋巴为主的炎细胞浸润。诊断:结节性痒疹。治疗上予沙利度胺50 mg 2次/天,配合糖皮质激素系统用药1月余后,患者皮损好转,未见明显复发。     

黑素瘤1例

患者女,51岁。右足底近跟部丘疹、溃疡、肿物2年。皮损组织病理示:表皮溃烂,真皮内弥漫分布核大、异型的黑素瘤细胞,瘤细胞内、间质均可见色素颗粒。免疫组化染色示:S-100(+),HMB-45(+),CK(+),Vim(-)。诊断:黑素瘤。     

表皮内透明细胞肿瘤

图1的皮损组织病理特征为：①表皮角化过度,伴有角化不全,棘层肥厚。②表皮的大部分由胞质透明的细胞组成,细胞排列紊乱,细胞大小不一,形态各异,部分细胞未见细胞核,或细胞核偏于一侧,可见双核或多核细胞及核丝分裂像。③表皮下1/5（包括基底细胞层）未见异常。④真皮中上部血管扩张,有以淋巴细胞为主的炎性细胞浸润。免疫组化染色结果示,表皮内透明细胞细胞角蛋白（CK）阳性,HMB45,S-100蛋白及CEA阴性。     

进行性结节性组织细胞瘤

目的描述进行性结节性组织细胞瘤临床、细胞结构及亚细胞结构的特征。方法 活检组织HE染色、免疫组织化学染色显微镜观察及电镜观察。结果镜下可见活检皮损与周围组织界限清晰,并见多核巨细胞、泡沫样组织细胞和组织细胞,部分区域可见席纹状排列梭形细胞,多核巨细胞、梭形细胞、泡沫样组织细胞、组织细胞CD68、溶酶体标志物染色阳性,S-100蛋白染色阴性。冰冻组织切片苏丹黑染色可见泡沫样组织细胞内脂滴。电镜下未发现Birbeck颗粒和Caputo小体。结论本病应与多发性幼年性黄色肉芽肿、网状组织细胞增生症、发疹性组织细胞瘤、播散性黄瘤病鉴别,该病例可以确立进行性结节性组织细胞瘤诊断。     

S-100阳性泛发性幼年黄色肉芽肿一例并文献复习

报道1例S-100阳性泛发性幼年黄色肉芽肿患儿并对相关文献进行复习。患儿,男,6个月,面部、躯干、四肢起疹3个月余。组织病理示：表皮基本正常,真皮内可见团块状结节性浸润,浸润细胞主要为泡沫样组织细胞,梭形组织样细胞,并可见Touton巨细胞。免疫组化：泡沫样组织细胞及梭形组织样细胞CD68(+),S-100(+),CD1a(-),Langerin（-）;电镜检查示：未见Birbeck颗粒。诊断：泛发性黄色肉芽肿。     

颗粒细胞瘤1例

报告1例颗粒细胞瘤。患者男,29岁。颈部结节1年。组织病理学检查示真皮内肿瘤细胞簇集成团,瘤细胞体积大,胞质内充满嗜酸性颗粒。PAS染色示瘤细胞周围绕以耐淀粉酶膜。免疫组化染色S-100蛋白阳性,提示肿瘤来源于施万细胞。     

成纤维细胞性风湿病

报告1例成纤维细胞性风湿病.患者女,67岁.因双手、足多发性皮肤结节伴关节痛20年,双手屈曲挛缩7年就诊.免疫学检查类风湿因子阴性.双手X线检查提示掌指关节、近端指间关节和远端指间关节可见骨质破坏,关节间隙变窄.皮损组织病理检查示真皮梭形细胞增生、胶原纤维增多和纤维化、弹性纤维数量减少.免疫组化染色示增生的梭形细胞平滑肌肌动蛋白(SMA)阳性,而CD68、CD34、S-100蛋白和上皮膜抗原(EMA)均阴性.符合成纤维细胞性风湿病的诊断.临床上该病主要需与类风湿关节炎、结节性硬皮病、多中心网状组织细胞增生症、皮肤进行性结节性纤维化、幼年性透明蛋白纤维瘤病和婴儿指部纤维瘤病等鉴别.     

瘤型麻风伴梅毒非特异性抗体假阳性1例

患者男,46岁,面部、四肢、足侧缘斑块、结节2个月,躯干、四肢暗红色斑疹1个月。面部皮损组织病理示：表皮轻度萎缩,真皮内见由泡沫样组织细胞构成的结节状浸润,与表皮间有无浸润带,抗酸菌染色(4+)。足部皮损组织病理示：表皮轻度萎缩,真皮及皮下脂肪组织内见白色泡沫样组织细胞构成的结节状浸润,与表皮间有无浸润带,抗酸菌染色(4+)。躯干部皮损组织病理示：表皮轻度萎缩,真皮血管周围可见组织细胞、淋巴细胞浸润,部分组织细胞呈泡沫状,抗酸菌染色(2+)。面部皮肤组织涂片,抗酸杆菌阳性(4+)。甲苯胺红不加热血清试验(TRUST)阳性,TRUST半定量1∶1,梅毒螺旋体特异性抗体(TPPA)阴性。诊断：瘤型麻风伴梅毒非特异性抗体假阳性。于当地疾控中心接受麻风联合化疗(MDT-MB)方案治疗好转,目前随访中。     

Two cases of vulval pigmented extramammary Paget's disease: histochemical and immunohistochemical studies

We describe two Japanese female patients with pigmented extramammary Paget's disease (EMPD); one patient had a dark brown plaque and the other had a reddish patch with a pigmented area, both affecting the vulval region. Histochemical and immunohistochemical examinations confirmed EMPD with melanocyte colonization; plump tumour cells with a large nucleus and pale cytoplasm that were positive for CAM 5.2 and CEA proliferated singly or in nests in the epidermis, and scattered among the tumour cells were many dendritic cells with a large amount of melanin that were positive for HMB-45 and S-100 protein. Fontana-Masson (FM) stain showed many positive cells with well-developed dendritic processes within and around tumour nests. Histochemical and immunohistochemical studies of non-pigmented EMPD cases on the same region showed that HMB-45 positive cells were sparse or not detected at all, and that also FM staining-positive cells were decreased or not detected, and their dendritic processes were poorly formed. The present study suggests that there might be heterogeneity in EMPD in terms of relationships between Paget's cells and melanocytes.     

Angiosarcoma with dermal melanocytosis

We report a case of angiosarcoma and dermal melanocytosis occurring simultaneously in the same lesion. We examined the primary and 2 metastatic lesions. Histopathologically, the anaplastic angiosarcoma cells had a tendency to form irregularly shaped small cavities. Immunohistochemically, they were strongly reactive with Ulex europaeus agglutinin (UEAI) stain. The mid and deep dermis of the same lesion had spindle or elongated slender melanocytes containing melanin granules. The melanocytes were positive with S-100 protein stain. Ultrastructurally, pinocytotic vesicles, fine filaments, and Weibel-Palade body-like dense granules were observed in the cytoplasm of angiosarcoma cells. Dermal melanocytes had external lamina and melanosomes in various stages. The melanocytes showed no similarity to the neoplastic tumor cells and there was no apparent intermediate form between the 2 kinds of cells. The etiological implications of dermal melanocytosis with a tumor of vascular origin are discussed.     

Cutaneous indeterminate cell histiocytosis: a new spindle cell variant resembling dendritic cell sarcoma

BACKGROUND: Cutaneous indeterminate cell histiocytosis is a rare neoplastic disorder. Its varied histological presentation and rarity have limited efforts to determine its pathogenic relationship with other histiocytic lesions and possibly, its recognition. METHODS: We report on an unusual histologic pattern of indeterminate cell histiocytosis that resembled follicular dendritic sarcoma. A battery of immunohistochemical stains and electron microscopy were performed to elucidate the phenotype of the "histiocytic" cells. Based on a review of the literature, reported cases of indeterminate cell histiocytosis are presented and the diagnostic differential of spindle-cell lesions is discussed. RESULTS: Spindling histiocytes were positive for S-100 and CD1a. The monocytic/macrophage marker, CD68, and the dendritic cell marker, CD21, were negative. Electron microscopy failed to reveal Birbeck granules. CONCLUSIONS: Relatively few reports of indeterminate cell histiocytosis exist, some of which include discussion of potential overlaps with the non-X histiocytoses. Although the presence of prominent spindling in our case expanded the differential to include non-histiocytic disorders, the identified histiocytes unequivocally fulfilled the criteria of S-100 and CD1a positivity without demonstrable Birbeck granules.     

阴茎恶性黑色素瘤1例附文献复习

患者，男，73 岁,龟头部黑色斑疹4年，血尿半月。皮损病理检查示: 镜下见肿瘤细胞主要位于表皮下，细胞核大且不规则，胞质中含大量黑色素颗粒。免疫组化:肿瘤细胞S-100(+),HMB45(+), Melan-A(+), SOX-10(+),Ki67约50%阳性。     

Multicenter skin tumor of the interdigitating dendritic cell

This report concerns a multicentric skin tumor of dendritic cells. Skin-colored to reddish-brown papules and nodules erupted on the right arm of a 68-year-old man and, during the following years, spread to the upper extremities, shoulders, legs and face. Immunohistochemically the large, histiocyte-like tumor cells are Leu-6 (CD1), Leu-3a (CD4), HLA-DR/DQ and S-100 protein positive, but on thorough ultrastructural investigation were found to lack Birbeck granules. Thus, this disease represents a skin tumor of the interdigitating dendritic cell, an accessory cell which, in this case, was associated with a high content of T-lymphocytes within the cutaneous cellular infiltrate. The clinical course is characterized by periodic progression with intermittent, spontaneous regression. During a 7-year follow-up it was not possible to ascertain involvement of either lymph nodes or internal organs.     

Histiocytic disorders with spontaneous regression in infancy

The histiocytic disorders are uncommon, have a wide spectrum, and are poorly understood. We describe seven cases developing in infancy, seen during a period of 9 years at Asan Medical Center, Seoul, Korea. Clinically the patients had multiple papules over the face, trunk, and extremities that developed at birth or during infancy. Histopathologic examinations revealed an infiltrate of many histiocytic cells in the upper dermis with or without epidermotropism. Four cases were classified as congenital self-healing reticulohistiocytosis in that the histiocytes were identified as Langerhans cells by positive immunohistochemical staining for S-100 protein, ultrastructural studies showing many Birbeck granules, and spontaneous regression of the lesions within 1-4 months. One infant with a solitary lesion on the forehead was diagnosed as solitary, congenital, indeterminate cell histiocytoma because the histiocytic cells were S-100 protein positive, but meticulous ultrastructural studies did not detect Birbeck granules. The lesion was removed by shave excision. Two cases were classified as generalized eruptive histiocytoma. The histiocytic cells were S-100 protein negative and ultrastructurally Birbeck granules were absent. In one patient, eyeball- or popcornlike lysosomal structures were seen. The lesions regressed completely.     

Variations in S-100 protein expression in naevocellular naevi may be related to metabolic activity

Significant amounts of S-100 protein were demonstrated in naevus cells by an immunoperoxidase technique. Distribution of S-100 protein was similar in the various types of naevi tested. In most lesions, the superficial A-type naevus cells stained most heavily, while staining was weaker in the deeper B- and C-type naevus cells. The intensity of staining was not, however, homogeneous in the cells of the various layers of the naevus. S-100 protein was especially abundant in giant naevus cells which occurred in the vicinity of sub-epidermal A-type naevocytes. Cells from neurocutaneous melanosis contained extremely large amounts of S-100 protein. Normal melanocytes in the epidermis over dermal naevi contained variable amounts of S-100 protein, but mainly showed weak staining. However, in the basal epidermis of traumatized naevi there were many unusually large and partly dendritic melanocytes. In this part of the epidermis, S-100 protein-positive cells lay close together, and many stained strongly. In earlier work, large amounts of metabolic enzymes were found at the same sites in traumatized naevi and giant cells. These results suggest that a high degree of expression of S-100 protein is due to metabolic activity shown, for example, by giant naevus cells and basal melanocytes of the regenerating epidermis over traumatized naevi.     

Giant melanotrichoblastoma

Trichoblastomas are benign skin tumors composed mostly of follicular germinative cells recapitulating the differentiation of hair follicles. Several variants of trichoblastomas have been reported, including pigmented trichoblastomas, melanotrichoblastomas, subcutaneous trichoblastomas, or giant trichoblastomas. We report a rare case of a pigmented trichoblastoma with abundant dendritic melanocytes. A 51-year-old Korean man had a round soft tissue mass on his back. The tumor was 6 cm in size and located in the subcutaneous fatty layer. On immunohistochemical stain for Melan A, numerous dendritic melanocytes were demonstrated in both pigmented and nonpigmented epithelial cell components. These cells were also positive for HMB45, and some of the dendritic melanocytes were positive for S-100 protein. With the aid of immunohistochemical staining, we diagnosed the tumor as a giant melanotrichoblastoma.     

Microcystic adnexal carcinoma with Langerhans cells

A slowly growing, subcutaneous mass in the left eyebrow region of a 54-year-old Japanese woman showed numerous small epithelial cell nests surrounded by a dense desmoplastic stroma. The epithelial cell nests showed partially keratinizing cystic structures containing a small amount of glycogen. Immunoperoxidase staining for carcinoembryonic antigen was positive only in the lumina or on the ductal lining surface of tumor cells; staining for S-100 protein was positive within almost all of the tumor nests. Birbeck granules indicating Langerhans cells were found within some of the cells.     

Histamine-containing mast cells and their relationship to NGFr-immunoreactive nerves in prurigo nodularis: a reappraisal

The mast cell, which is a histamine-containing cell, has been found to have far more functions in skin inflammation than hitherto understood. To investigate the appearance of mast cells in prurigo nodularis, histamine immunohistochemistry in combination with nerve growth factor receptor (NGFr) double-staining as well as electron microscopic studies were performed. The results revealed that the histamine-containing cell number was increased in the lesional dermis. The mast cell size was also increased and the shape had become more dendritic. They tended to contact the epidermis and even infiltrated into it. In the histamine and NGFr double-staining, both an increased histamine-containing mast cell number and an increased number of NGFr-immuno-reactive nerve fiber profiles were revealed in the upper dermis of the prurigo nodularis lesional skin. Mast cells were seen in close vicinity to NGFr-positive nerves and sometimes even seemingly to contact single nerve fibers. At the ultrastructural level, it is obvious that the mast cell bodies become larger, having more abundant cytoplasm and organelles (e.g. mitochondria), but comparatively fewer characteristic granules. Mast cells were often observed to sprout long dendrites, with or without granules. The cells were also frequently seen to contact other cell types, and a mast cell infiltration into the epidermis was also found. The statistical results of mast cell numbers showed a significant increase in prurigo nodularis lesional skin compared to the normal controls. The present results further indicate that mast cells, together with cutaneous nerve fibers, are actively involved in the pathogenesis of the disease.