皮下脂膜炎样T细胞淋巴瘤1例

报道1例皮下脂膜炎样T细胞淋巴瘤。患者女，34岁。全身反复出现肿块、结节性损害1年。4个月前部分肿块破溃，伴发热、乏力、贫血等症状。皮损组织病理显示皮下脂肪层的淋巴细胞、组织细胞浸润，脂膜炎样T细胞瘤。免疫组化证明下细胞来源，伴有TCRγ基因克隆性重排，诊断为脂膜炎样T细胞瘤。     

脂膜炎样T细胞淋巴瘤1例

报告1例皮下脂膜炎样T细胞淋巴瘤,患者女,44岁,左侧臀部出现红肿、结节,伴疼痛和发热半个月,皮损组织病理检查：皮下脂肪层有淋巴细胞、组织细胞浸润,免疫组化证明浸润淋巴细胞为T细胞来源,组织病理改变符合脂膜炎样T细胞淋巴瘤。     

硬化性脂膜炎1例

患者女,74岁,左小腿反复水肿性红斑伴疼痛10个月,边界清,伴色素沉着,有触痛,皮温稍高。组织病理检查示:真皮毛细血管增生,含铁血黄素沉积,皮下脂肪小叶内可见淋巴细胞、组织细胞及泡沫细胞等混合性炎症浸润,局部可见膜性脂肪坏死,胶原纤维增生。诊断为硬化性脂膜炎。     

儿童色素减退型线状苔藓反射式共聚焦显微镜图像特征分析

目的 分析儿童色素减退型线状苔藓皮肤反射式共聚焦显微镜(RCM)影像学特征.方法 RCM检查11例临床诊断为色素减退型线状苔藓的患儿皮损及皮损附近正常皮肤,再与该处皮损组织病理学检查进行对比.结果 色素减退型线状苔藓皮损组织病理学检查显示,表皮细胞间或细胞内轻度水肿,伴不同程度棘层增厚,基底细胞灶性液化变性,真皮浅层血管周围较多淋巴细胞及少数噬黑素细胞浸润.RCM水平扫描皮损显示,多灶性基底细胞液化变性,导致表真皮界面模糊,色素环不完整或不清晰,真皮乳头及真皮浅层较多高折光的噬黑素细胞及中、低折光的炎症细胞浸润.结论 RCM影像学特征可为儿童色素减退型线状苔藓的诊断及鉴别诊断提供有力依据.     

以假性水疱为首发症状的不完全型白塞病1例

患者男,23岁。因"面部红色斑块,伴关节痛2年加重2个月"入院。皮肤组织病理:表皮见浆痂,棘层海绵水肿,基底细胞液化变性,色素失禁,少许炎细胞侵入表皮,真皮浅层水肿,较多淋巴组织细胞,噬色素细胞,少许嗜中性白细胞浸润,中层血管、毛囊周围少许淋巴组织细胞、个别浆细胞。免疫荧光:阴性。结合临床诊断为:不完全型白塞病。     

皮下脂膜炎样T细胞淋巴瘤九例临床病理及免疫表型研究

目的 分析皮下脂膜炎样T细胞淋巴瘤(SPTL)的临床及组织病理表现、免疫表型、治疗和预后.方法 回顾性分析9例SPTL患者的临床与实验室资料,完善相关病理及免疫组化标记,并随访.结果 9例患者中8例表现为多发皮下结节和肿块,主要累及下肢(8例)和躯干(6例),7例伴发热.3例行全身PET-CT,7例行骨髓穿刺,未见皮肤外内脏系统肿瘤,均不伴噬血细胞综合征.皮肤组织病理示异形单个核细胞核大、深染,主要浸润皮下脂肪组织,围绕单个脂肪细胞呈环状排列,真皮层附属器和血管周围可见肿瘤细胞浸润(5/9例).免疫表型示9例肿瘤细胞表达βF1、CD3、CD8,8例表达粒酶B和T细胞胞内抗原1(TIA-1),均不表达CD4、CD20、CD30、CD56.5例接受化疗(1例儿童和1例产后妇女),1例儿童接受甲泼尼龙冲击治疗,随访8例治疗后均达到临床完全缓解.结论 SPTL来源于α/βT细胞,组织病理和免疫组化检查有助于诊断及鉴别诊断.     

肉芽肿性唇炎1例

报告肉芽肿性唇炎1例。患者女,36岁。反复上唇弥漫性肿胀7个月。组织病理学检查示:表皮轻微角化过度伴角化不全,真皮内淋巴细胞、组织细胞及浆细胞呈片状浸润,可见上皮样细胞,可见扩张的淋巴管腔。诊断为肉芽肿性唇炎。     

多中心网状组织细胞增生症1例

报告1例多中心网状组织细胞增生症.患者女,46岁,双手指、右脚(足母)趾丘疹、结节伴双手指末节指间关节肿胀、疼痛3年.皮损组织病理检查:真皮内见多灶性或团块状组织细胞浸润.免疫组化染色:Mac387(+),CD68(+),S-100(-),EMA(-).诊断:多中心网状组织细胞增多症.给予静脉点滴环磷酰胺、口服泼尼松4周后病情好转.     

儿童原发性皮肤γδ-T细胞淋巴瘤合并噬血细胞综合征一例

【摘要】 患儿女,13岁。臀部、四肢反复起斑块6个月,加重伴间断发热5个月。体检：各系统检查未见明显异常。皮肤科情况：臀部及双下肢散在分布直径3 ～ 11 cm质硬、浸润性、紫红色皮下斑块,界不清,表面少许脱屑,无明显压痛及溃疡形成。实验室检查：白细胞（0.03 ～ 3.7） × 109/L,红细胞（2.8 ～ 4.4） × 1012/L,血小板计数正常;纤维蛋白原1.79 g/L,NK细胞比例4.6%;骨髓涂片示骨髓增生活跃,粒系比例降低,网织细胞易见,并见少量分类不明细胞及噬血现象。皮损组织病理学检查：表皮、真皮未见明显异常,皮下脂肪组织内可见大量淋巴样细胞浸润,以脂肪小叶为主,部分小叶间隔受累;浸润细胞体积中等偏大,胞质少,核多形,部分核扭曲;可见坏死及吞噬核碎片、淋巴细胞现象;免疫组化：CD3、颗粒酶B、T细胞细胞内抗原-1（TIA-1）、TCRγδ阳性,CD5、CD7部分阳性,CD4、CD8、TCRαβ、CD56阴性,Epstein-Barr病毒编码RNA原位杂交检测阴性。诊断：原发性皮肤γδ-T细胞淋巴瘤合并噬血细胞综合征。 【关键词】 淋巴瘤,T细胞,皮肤; 淋巴组织细胞增多症,嗜血细胞性     

单侧发病的持久性色素异常性红斑一例

患者,女，40岁。右下肢红色斑片伴褐色色素沉着6月余。组织病理示：表皮轻度萎缩，基底细胞空泡变性，真皮浅层大量噬色素细胞伴稀疏炎症细胞浸润。诊断：持久性色素异常性红斑。0.1％他克莫司软膏外用1个月，皮疹略变淡。     

Diagnostically challenging spindle cell lipomas: a report of 34 "low-fat" and "fat-free" variants

Spindle cell lipomas (SCL) classically occur as subcutaneous masses in the upper trunk/neck of older men and are composed of mature fat, CD34-positive spindled cells, ropey collagen, myxoid matrix, and blood vessels. A number of variants have been reported, including SCL with pseudoangiomatous change, composite SCL hibernoma, and composite SCL/pleomorphic lipoma. A review of over 300 consultation cases diagnosed as SCL revealed 34 cases in which fat was noted to be present in <5% of the tumor (n = 30) or absent (n = 4). These cases posed diagnostic difficulties because of the dearth of fat; we propose the terms "low-fat" and "fat-free" SCL for these variants. The tumors presented in older men (mean, 56 years; ratio of males to females, 11:1) and presented as small (mean, 2.0 cm) circumscribed dermal or subcutaneous masses of the head/neck (n = 18), back (n = 7), shoulder (n = 5), leg (n = 2), arm (n = 1), or unknown location (n = 1). In the majority, referring pathologists considered benign diagnoses, usually benign nerve sheath tumors, but in four cases low-grade sarcoma was considered. In only three cases was SCL considered. The tumors were composed of aggregates of CD34-positive, bland spindled cells arranged in characteristic parallel arrays, admixed with ropey collagen and myxoid matrix. Isolated clusters or single adipocytes were present in 30 cases; four were devoid of fat. CD34 was diffusely positive (10/11). A high index of suspicion based on clinical context and identification of other typical features of SCL are key features to the diagnosis of low-fat and fat-free SCL.     

Angiomyxolipoma (vascular myxolipoma) of subcutaneous tissue

Three cases of angiomyxolipoma (vascular myxolipoma) have been published to date. We report an angiomyxolipoma located in the subcutaneous tissue of the scalp. Histologically, it consisted of an admixture of paucicellular myxoid areas and mature fat tissue, both containing numerous dilated blood vessels with hyalinized thin walls. The cells in the myxoid areas expressed CD34. This lesion requires differential diagnosis with other benign fat tissue tumors, especially myxoid spindle cell lipoma, superficial angiomyxoma, myxoid liposarcoma, and low-grade myxofibrosarcoma.     

假性黑棘皮病患者血清瘦素及可溶性受体与胰岛素的测定

目的 探讨假性黑棘皮病患者血清瘦素及其受体与胰岛素之间的关系.方法 分别采用放射免疫分析法、酶联免疫吸附实验和葡萄糖氧化酶法检测25例假性黑棘皮病、20例单纯肥胖者瘦素、胰岛素、C肽可溶性受体与血糖水平,并与正常人对照组比较.结果 假性黑棘皮病患者和单纯肥胖者体质量、体质指数、脂肪百分比、腰臀围比明显高于正常人对照组,两组差异有统计学意义(P<0.01).假性黑棘皮病患者和单纯肥胖者胰岛素、C肽水平明显高于正常人对照组(P<0.01),胰岛素敏感指数低于正常人对照组(P<0.01);单纯肥胖者血糖水平和正常人对照组相比,差异无统计学意义(P>0.05);假性黑棘皮病患者与单纯肥胖者血清中瘦素水平和正常人对照组相比显著升高(P<0.01),可溶性瘦素受体水平显著降低(P<0.01).结论 假性黑棘皮病患者存在瘦素抵抗、高胰岛素血症和胰岛素抵抗,瘦素抵抗在假性黑棘皮病患者高胰岛素血症、胰岛素抵抗与肥胖中起一定的作用.     

Granuloma with lymphocytic hyperplasia following vaccination: 10 cases. Presence of aluminium in the biopsies

BACKGROUND: Few cases of cutaneous lymphocytic hyperplasia secondary to vaccination have been published, although such lesions are not rare.PATIENTS AND METHODS: We report a series of 10 cases registered between 1993 and 2003.RESULTS: Mean age was 25. The clinical aspect was solitary or multiple subcutaneous nodules, located on the arm, developing after a delay of 1 to 18 months after vaccination. Histologic examination showed a lymphocytic infiltration of the subcutaneous fat, with diffuse and/or follicular pattern, without nuclear atypia, the morphological and immunohistochemical analysis of which revealed the benign nature. In all cases, there was fibrosis and granuloma composed of lymphocytes, plasma cells, eosinophils and macrophages with basophilic cytoplasm. Morin stain showed intralesional aluminium in the 6 investigated cases. Evolution was always benign, with no relapse following exeresis.DISCUSSION: Cutaneous lymphocytic hyperplasia secondary to vaccination has to be suspected in a young patient with subcutaneous nodules appearing at a vaccination site. Evidence of aluminium in the lesions supports the diagnosis and the hypothesis that aluminium in the vaccine excipient might have a role in the onset of such lesions.     

Cytokeratin 15 expression in apocrine mixed tumors of the skin and other benign neoplasms with apocrine differentiation

To clarify the features of apocrine mixed tumors (AMT) of the skin among benign neoplasms with apocrine differentiation in their relationship to follicular stem cells, we investigated the immunohistochemical expression of CK15 (LHK15 and C8/144B), which is a relatively specific marker of hair follicle stem cells in the bulge, in 35 cases of eight different benign neoplasms with presumed apocrine differentiation. All eight cases of AMT of the skin showed CK15 immunostaining of the neoplastic cells, and all four cases of syringocystadenoma papilliferum, all five cases of spiradenoma, and both cases of cylindroma also showed a focally positive reaction to CK15. None of the other benign neoplasms with presumed apocrine differentiation showed CK15 expression. In AMT of the skin, the proportion of CK15-positive cells in the follicular or sebaceous differentiation group (78.8%, average of four cases) was significantly higher than the group without this differentiation (8.8%, average of four cases). AMT of the skin are unique among benign neoplasms with apocrine differentiation in their substantial and constant CK15 expression, suggesting that they derive from multipotent epithelial stem cells in the bulge. AMT of the skin with follicular or sebaceous differentiation are considered to show an immature stage of apocrine differentiation still rich in stem cells or to originate from stem cells with an incompletely established apocrine fate. The partially positive reaction for CK15 in syringocystadenomas papilliferum and spiradenoma/cylindroma may depend on the ability to express CK15 in stem cells with an apocrine fate or result from the follicular and apocrine nature of this neoplasm.     

假性黑棘皮病肥胖儿童：2型糖尿病的高危人群

目的探讨肥胖儿童伴假性黑棘皮病与胰岛素抵抗及2型糖尿病的关系。方法对42例肥胖伴假性黑棘皮病儿童,60例单纯性肥胖儿童及20例正常儿童的空腹血糖及胰岛素水平、空腹血糖/ 胰岛素比值及人体测量学参数[腰围/臀围比值(wHR)、全身体脂含量、体脂百分数、体质指数]进行分析比较,并对肥胖伴假性黑棘皮病儿童组与单纯性肥胖儿童组行葡萄糖耐量试验,以比较两组患儿的糖耐量异常情况。结果假性黑棘皮病组有2例诊断为2型糖尿病,21例糖耐量异常,明显高于单纯肥胖儿童组;葡萄糖耐量试验提示60 min及120 min的血糖水平显著高于单纯肥胖组(P<0.01)。人体测量学参数包括腰围/臀围比值、全身体脂含量、体脂百分数、体质指数及空腹血胰岛素水平明显高于单纯性肥胖组(P<0.01)和对照组(P<0.01),空腹血糖/胰岛素比值明显低于单纯肥胖组(P<0.0I)和对照组(P<0.01)。结论儿童假性黑棘皮病与肥胖、高胰岛素血症、胰岛素抵抗密切相关,是发生2型糖尿病的高危人群。     

Argyrophilic nucleolar organizer region counts in malignant melanoma associated with benign intradermal nevus.

A silver colloidal technique to demonstrate argyrophilic proteins of the nucleolar organizer regions (AgNORs) was performed on sections of 20 cases of malignant melanoma (MM) associated with underlying benign nevus (BN). In these cases, significant different AgNOR counts were found for MM and BN. In addition, this technique permitted the identification of melanocytic cells located between malignant and benign cells showing AgNOR scores intermediate (5.51) between BN (2.6) and MM (7.71) with a more complex and bizarre morphology than that observed in BN. The AgNOR technique can be suitable in the identification of residual nevus cells in MM, especially when their number is minimal and the common histologic criteria are unsatisfactory; it can also increase the understanding of the natural history of MM.     

Cellular blue nevus with nevus cells in a sentinel lymph node

Regional lymph node involvement by a cellular blue nevus has been reported. However, it has recently been suggested that specific cases with "benign metastasizing" cellular blue nevi are actually rare. This study describes a typical case of a cellular blue nevus with nevus cells in a sentinel lymph node. We demonstrated that a cellular blue nevus clearly involved the regional lymph node and investigated the immunohistochemical profiles of such nodal cellular blue nevus cells. The location of the nevus cells fundamentally indicated a benign type, with limitation to the capsule and the fibrous trabeculae. However, only a few, isolated nevus cells were also seen in the parenchyma of the lymph node. The nevus cells in the capsule and the fibrous trabeculae were positive for c-kit, like the migrating melanocytes from the neural crest. In cellular blue nevi or lesions with similar histopathological features, it may be appropriate to consider the predominant involvement of the capsule as well as the benign cytological features and the immunohistochemical profiles (Ki-67-, PCNA-, and c-kit+) of the nodal cells to be a benign sign.     

Malignant Schwannoma Arising in Patients with Von Recklinghausen's Disease: Report of Two Cases and the Comparison of Mast Cells between Benign and Malignant Portions

We described two cases of malignant schwannoma arising in patients with von Recklinghausen's disease and examined the mast cells infiltrated into histologic sections. One of the two cases histologically revealed apparent mast cell infiltration in some areas of malignant schwannoma as well as in the benign neurofibroma. The malignant lesion demonstrated significantly increased percentages of degranulated mast cells over the benign lesion using FITC-avidin staining. In an electron microscopic study, mast cells in the malignant lesion displayed empty granules, piecemeal degranulation, and canaliculi structures suggesting activation. These findings were not observed in the benign lesion. The other patient histologically showed no mast cells in the malignant lesion, although the benign neurofibroma in the patient disclosed numerous mast cells. The first patient had neither recurrence nor distant metastasis. On the other hand, the second patient without mast cells in the histology had multiple distant metastases. These findings raise questions about the role of mast cells in malignant schwannoma.     

Poroma with sebaceous differentiation: Report of three cases

We herein report three cases of poroma, which showed sebaceous differentiation. The patients were two men and one woman, and the involved sites were the thigh, head and back. In all three cases, histopathological examination revealed anastomosing nests consisting of poroid cells proliferating into the dermis. Tubular structures lined by eosinophilic cuticular cells were scattered in the tumour nests. Furthermore, aggregations of mature sebocytes with vacuolated cytoplasm were also observed forming clusters, or intermingled with the poroma cells. Focal calcification was found within the nests in two cases. Sebaceous component were recognized in the presented three cases among the 62 cases (4.8%) examined in our department within the past 10 years. We also include a review of the 31 reported cases of poroma with sebaceous differentiation. None of them occurred in acral regions, where typical eccrine poromas predominate. The majority of cases were benign. This review indicates that some poromas may show sebaceous differentiation and be of apocrine lineage.