幼年性黄色肉芽肿二十例组织病理及皮肤镜特征分析

目的 探讨皮肤镜在幼年性黄色肉芽肿诊断中的应用价值。方法 回顾性分析2018年7月—2021年3月西安医学院第一附属医院皮肤科确诊的20例幼年性黄色肉芽肿患者临床、组织病理资料及皮肤镜特征。结果 幼年性黄色肉芽肿20例患者（男12例,女8例）,平均发病年龄（2.12±4.29）岁。典型皮损表现为直径数毫米至数厘米的黄色、红色或棕色丘疹和结节,质硬,表面光滑。20例患者皮损均为单发。组织病理学表现为真皮内见边界相对清楚、无包膜的细胞团块,主要由组织细胞、Touton巨细胞及少量炎性细胞组成。20例幼年性黄色肉芽肿患者皮肤镜模式均表现为橘黄色、粉红色无结构区+血管结构,表现为落日征17例（85%）、苍白黄色球形云13例（65%）、白色条纹5例（25%）。血管结构呈不规则分布,其中线状血管60%、点状血管55%、分枝状血管35%。结论 幼年性黄色肉芽肿患者皮肤镜下具有独特的表现,可作为其辅助诊断技术。     

32例幼年黄色肉芽肿临床与病理分析

 目的:归纳总结幼年黄色肉芽肿的临床及组织病理特点。方法:回顾性分析2011年12月-2019年12月我院确诊的32例幼年黄色肉芽肿患儿的临床资料和组织病理学检查结果。结果:32例患儿皮损多表现为头面部、躯干、四肢黄色或黄褐色丘疹、结节,也可表现为红色或暗红色,有1例伴皮肤外症状。皮损组织病理特点可分为3种类型：早期（占12.50%）,可见大量组织细胞浸润,散在多核巨细胞、淋巴细胞、少量嗜酸性细胞浸润;成熟期（占78.12%）,可见典型泡沫细胞、多核巨细胞及Touton巨细胞;消退期（占9.38%）,可见梭形细胞,少许组织细胞及多核巨细胞。免疫组化结果为CD68（+）、CD1a（-）、Langerin（-）及S-100（-）。结论:临床上根据典型皮损特点及病理表现一般可诊断幼年黄色肉芽肿,但临床上有必要行病理检查以帮助减少误诊率。     

铒激光治疗汗管瘤34例临床疗效初步观察

应用铒激光治疗仪治疗34例汗管瘤患者，6个月后观察疗效，结果表明34例患者中29例皮损完全消退，治愈率85％，未见色素沉着或色素减退；2例患者共2个皮损有轻微的皮肤表面纹理改变，我们认为铒激光治疗汗管瘤安全而有效。     

成人肥厚型鲜红斑痣82例肥厚相关因素分析

目的 分析肥厚型鲜红斑痣患者的临床特点及导致皮损肥厚的相关因素。 方法 2010年1月至2014年8月,安徽省立医院和安徽医科大学第一附属医院皮肤科门诊成人鲜红斑痣患者纳入研究。分析肥厚型鲜红斑痣患者临床特点,并采用单因素和多因素非条件Logistic回归分析导致皮损肥厚的相关因素。结果 共收集资料完整的成年鲜红斑痣患者262例,其中肥厚型鲜红斑痣82例,男30例,女52例,年龄18 ~ 54岁,中位年龄32.5岁;66例（80.48%）表现为斑块样肥厚型,9例（10.98%）为丘疹/结节型,7例（8.54%）为混合型。肥厚型鲜红斑痣患者年龄 ≥ 30岁者占56.10%（46/82）,皮损面积以11 ~ 30 cm2为主（41.46%,34/82）,皮损颜色以紫红色为主（85.36%,70/82）,与扁平型鲜红斑痣相比,两组差异有统计学意义（χ2 = 25.559、10.580、90.630,P < 0.05）,而性别、Fitzpatrick皮肤分型、皮损部位及皮损分布与肥厚无关。多因素非条件Logistic回归分析显示,年龄≥ 30岁（OR = 2.889,95% CI：1.459 ~ 5.721）、皮损呈紫红色（OR = 19.984,95% CI：5.704 ~ 70.023）是鲜红斑痣肥厚的相关因素。 结论 年龄≥ 30岁和皮损颜色呈紫红色是肥厚型鲜红斑痣患者肥厚的相关因素。     

隆突性皮肤纤维肉瘤50例临床病理分析

目的 探讨隆突性皮肤纤维肉瘤（DFSP）的诊断及鉴别诊断要点。 方法 回顾性分析中国医学科学院皮肤病研究所1998—2014年诊治的50例DFSP患者的临床表现、组织病理学、免疫组化特点及治疗和预后。 结果 50例患者发病年龄（29.5 ± 15.9）岁,平均病程9.6年。皮损位于躯干部33例,占66.0%;其次为四肢和头颈部。典型皮损表现为,萎缩性斑片/斑块13例,占26.0%;在此基础上多发性大小不一结节30例,占60.0%;正常皮肤上单发或多发结节7例,占14.0%。组织病理学特征由单一编席样或车辐状排列的梭形细胞组成,呈浸润性生长,肿瘤细胞表达CD34和波形蛋白。20例患者皮损切除后原位复发,复发率43.5％,未见远处转移和死亡患者。 结论 DFSP皮损形态变化多样,容易误诊,组织病理及免疫组化检查可确诊。DFSP切除后局部复发率高,可多次复发,但罕见淋巴结和远处转移。     

鲍恩样丘疹病皮损中HPV16、18的检测

目的：探讨人乳头瘤病毒（HPV）16、18在鲍恩样丘疹病皮损中的感染情况。方法：采用杂交捕获试验筛选高危型HPV和低危型HPV阳性标本,对高危型HPV阳性标本经PCR技术检测其HPV16、18DNA阳性者。结果：45例鲍恩样丘疹病患者皮损中34（75.6%）例为高危型HPV阳性,在高危型HPV阳性标本中82.2%（28/34）为HPV16阳性,5.9%（2/34）为HPV18阳性,8.8%（3/34）HPV16、18均阳性,2.9%（1/34）未检测到HPV16及HPV18DNA。结论：多数鲍恩样丘疹病皮损中存在高危型HPV感染,尤其HPV16,故高危型HPV16感染与鲍恩样丘疹病的发生、发展密切相关。     

无色素痣的临床、组织学和超微结构观察

目的 探讨无色素痣的临床和组织学特征。方法 分析85例无色素痣患者的发病年龄、类型和皮损特点,并对部分患者行皮肤色素测定和反射式共聚焦显微镜（RCM）观察。对其中17例患者的皮损区和正常区皮肤组织进行组织病理检查,透射电镜观察皮损区超微结构。免疫组化法检测皮损区和正常皮肤处酪氨酸酶（TYR）、HMB45、酪氨酸酶相关蛋白1（TRP-1）、TRP-2和CD117表达。结果 85例无色素痣患者中,23例（27.1%）出生时发现皮损,21例（24.7%）出现于3岁以后,最大发病年龄为29岁。皮损分布于躯干部25例（29.4%）,颈部13例（15.3%）;72例（84.7%）皮损边缘不规则,54例（63.5 %）仅有1处皮损。19例无色素痣患者患处的黑素指数（186.56 ± 52.86）和相对黑素指数（80 ± 11）低于正常人皮肤（分别为223.88 ± 63.19和100）,高于12例白癜风患者皮损处（分别为128.57 ± 64.31和60 ± 20）,差异均具有统计学意义（P < 0.01）。反射式共聚焦显微镜示,无色素痣皮损中含黑素细胞数量减少,亮度减低,黑素分布均匀,皮损区与正常皮肤分界区常不清晰。皮损区Fontana-Masson染色示皮损区黑素强度为1810.12 ± 327.96,较正常区（2064.24 ± 260.41）明显减弱。电镜下发现黑素细胞数量减少,黑素小体减少,黑素细胞胞质和树突以及角质形成细胞中可见Ⅱ、Ⅲ期未成熟的黑素小体,角质形成细胞中可见聚集成团的黑素小体。17例患者正常区TYR表达水平为1827.35 ± 307.09,TRP-1为6102.54 ± 1642.64,而皮损区TYR（1477.35 ± 224.05）和TRP-1（5322.33 ± 1565.26）表达下降,正常区与皮损区比较,P均 < 0.01;HMB45、TRP-2、CD117表达两处比较差异均无统计学意义。 结论 无色素痣是一种早期发病、非家族聚集性、稳定的不规则色素减退性疾病,其皮损中黑素细胞和黑素小体数量均减少,可见未成熟黑素小体。相对黑素指数和反射式共聚焦显微镜检查可作为诊断无色素痣的无创性检测方法。     

关节病性银屑病29例临床分析

目的：探讨关节病性银屑病的临床特点及治疗。方法：对29例关节病性银屑病患者的临床资料进行回顾性分析。结果：本组病例平均发病年龄31．4岁,男18例、女11例,男：女为1．64：1。以皮损为首发者20例（69．0％）,关节炎首发者6例（20．7％）,皮损和关节炎同时发生者3例（10．3％）;皮损类型为寻常型27例（93．1％）,脓疱型1例（3．4％）,红皮病型1例（3．4％）;关节炎分类为非对称性少关节炎型10例（34．5％）,远端指（趾）间关节炎型7例（24．1％）,脊柱关节病型6例（20．7％）,对称性多关节炎型4例（13．8％）,残毁性关节炎型2例（6．9％）;25例患者接受关节X线检查,其中20例有阳性改变,表现为关节间隙变窄、骨质疏松、骨质吸收和骨刺形成。结论：本病男性多发,各型银屑病皮损都可并发关节炎病变。非对称性少关节炎和远端指（趾）关节易受累是本病的特点。甲氨蝶呤、来氟米特、阿维A、雷公藤多苷、沙利度胺、环孢素、吗替麦考酚酯、非甾体类抗炎药、小剂量糖皮质激素等合理联合用药可取得较好疗效。     

先天性平滑肌错构瘤16例临床及组织病理分析

目的：探讨先天性平滑肌错构瘤（CSMH）的临床及组织病理特点。方法：回顾性分析2014年1月—2020年10月于该皮肤医院确诊的16例CSMH患者的临床及组织病理资料。结果：16例患者中女性9例（56.3%）,男性7例（43.7%）,就诊年龄2～28岁,平均（11±12）岁。其中15例患者（93.7%）出生即有皮损,1例（6.3%）于2岁发病。15例患者（93.7%）表现为单发皮损,1例（6.3%）为多发皮损;皮损发生于面部者11例（68.7%）,四肢者3例（18.8%）,躯干者2例（12.5%）;皮损表现为斑片或斑块者14例（87.5%）,表现为群集性丘疹者2例（12.5%）;皮损颜色为褐色者11例（68.7%）,红色者3例（18.8%）,肤色者2例（12.5%）;8例患者（50.0%）表现为多毛。皮肤镜下可见病变区域毛发数量增加。皮损组织病理均表现为真皮内无一定走向的成熟平滑肌束,肿瘤细胞胞质呈明显的嗜酸性,细胞核呈雪茄样,两端钝圆。免疫组化示肿瘤细胞表达平滑肌肌动蛋白（SMA）及结蛋白（desmin）。结论：CSMH多出生即有,单发多见,可发生于面部、四肢和躯干,主要表现为褐色斑...     

湿疹皮炎患者过敏原特异性IgE检测阳性结果的多中心研究

目的 探讨湿疹皮炎患者血清过敏原检测常见的阳性过敏原及其临床意义。 方法 回顾性分析中国10家医院皮肤科就诊并进行了血清变应原特异性IgE检测且至少一项阳性的湿疹皮炎患者。 结果 458例患者纳入研究,其中男209例（45.63%）,女249例（54.37%）,年龄（28.13 ± 23.11）岁。前3位常见阳性过敏原为蟑螂（180/458,占39.30%）、尘螨组合（152/458,占33.19%）、花生（116/458,占25.33%）。89.66%（26/29）特应性皮炎患者及86.84%（33/38）面部湿疹患者存在多种过敏原。皮损相对泛发者过敏率（88/110,占80%）较皮损相对局限者（245/348,占70.40%）高,差异有统计学意义（χ2 = 3.880,P = 0.049）。吸入变应原阳性率在11 ~ 20岁年龄组最高（39/43,占90.70%）;牛奶（26/98,占26.53%）、牛肉（19/98,占19.39%）,特异性IgE 阳性多见于3岁以下婴幼儿。北方与南方常见变应原不同,艾蒿、蟑螂、牛奶、黄豆、牛肉、淡水鱼特异性IgE阳性率（10.35%、42.42%、14.39%、13.89%、11.36%、13.38%）均分别高于南方（1.61%、19.35%、3.23%、3.23%、1.61%、3.23%）,差异有统计学意义（?字2 = 4.917、11.959、5.958、5.594、5.641、5.235,均P < 0.05）。 结论 蟑螂是湿疹皮炎患者致敏率较高的变应原,临床意义值得研究。面部湿疹及特应性皮炎更容易多发过敏。年龄是影响变应原致敏的较重要因素。不同地域常见变应原不同。     

Structural correlations between dermoscopic and histopathological features of juvenile xanthogranuloma

Juvenile xanthogranuloma(JXG) is the variant of non‐Langerhans’ cell histiocytosis. The orange‐yellow background coloration with clouds of paler yellow deposits is the most characteristic dermoscopic finding of JXG. Other dermoscopic features include erythematous border, subtle pigment network and white linear streak. The objective of this study was to present the structural correlation between dermoscopic features and histopathological findings of JXG and to find the different dermoscopic features in various stages of JXG. Eleven patients with histologically proven JXG were examined with polarized light dermoscopy. Histopathological findings were assessed and dermoscopic features including setting sun appearance, clouds of paler yellow globules, whitish streak, and branched and linear vessels were evaluated. Among 11 patients, five patients were in early evolutionary stage, four patients in fully developed stage and two in late regressive stage. The setting sun appearance was found in all patients in different stages except one in late regressive stage (90.9%). The clouds of paler yellow globules were present in nine patients (81.8%) and were constant features in fully developed stage and late regressive stage. The whitish streak was present in four patients (36.4%) and telangiectasia in 10 patients (81.8%). The setting sun appearance may hold diagnostic value in early evolutionary stage to fully developed stage, but not in late regressive stage. The clouds of paler yellow globules are more predominant in fully developed stage and late regressive stage. In addition to the use of dermoscopy as an accurate diagnostic tool for differential diagnosis, it could be applied in evaluation of histopathological maturation of JXG.     

Disseminated Juvenile Xanthogranuloma

Juvenile xanthogranuloma (JXG) is the most common type of non‐Langerhans cell histiocytosis occurring predominantly in infants and children. Typical lesions are asymptomatic red‐yellow papules and nodules on the scalp or in the axillae or groins. Multiple lesions are more common in children than in adults. A 2‐year old girl presented with approximately 20 red‐brown lesions, leading to the diagnosis of disseminated JXG. Internal involvement was excluded. Spontaneous regression occurred over 6 months of follow‐up. A wait‐and‐see strategy is recommended for cutaneous JXG.     

Juvenile Xanthogranuloma with Central Nervous System Involvement

Abstract: An 18-year-old men has been followed in our department since age 10 years when he began to develop numerous yellow, papular lesions on the scalp, face, neck, trunk, and upper extremities. The lesions enlarged slowly to form yellow-brown nodules measuring up to 6 cm. During this period five of the nodules and two small papules were excised. Histopathology was consistent with juvenlle xanthograuloma (JXG). Recently, the patient complained of significant loss of memory, and a computed tomography scan was performed showing several cerebral and cerebellar lesions. The cutaneous lesions In our patient were almost identical to those described elsewhere as being characteristic of progressive nodular histiocytoma. The many clinical and histopathologic similarities between lesions of progressive nodular histiocytoma and JXG suggest that they may represent a continuum rather than two distinct disease processes. Given the fact that there are no histopathologic differences with JXG, as well as the wide range of clinical lesions that JXG may adopt, there Is no reason to separate the entities. Although JXG lesions are usually believed to be benign and self-healing, targe nodular forms can be associated with visceral lesions. Only four previous cases of cutaneous JXG with central nervous system involvement were found in a review of the literature.     

Ulcerated Spitz Nevus Masquerading as a Juvenile Xanthogranuloma

We report a case of ulcerated atypical Spitz nevi that demonstrated a yellow to light orange background under dermoscopy, which can be seen in juvenile xanthogranuloma (JXG) and is referred to as the “setting sun” appearance. This yellow to orange appearance was due to serous crusting and not histiocytic infiltration, which is seen in JXG. This case highlights overlapping dermatoscopic features between the two skin lesions and polymorphous vascular structures, which are unique to atypical Spitz nevi.     

皮肤镜和反射式共聚焦显微镜检查在幼年黄色肉芽肿中的诊断价值

目的：总结幼年黄色肉芽肿（JXG）在皮肤镜及反射式共聚焦显微镜（RCM）下特征,探讨皮肤镜、RCM及二者联合应用对JXG的诊断价值。方法：分析43例拟诊为JXG患儿皮损的皮肤镜及RCM镜下表现,由4名皮肤科医师采用五级法诊断,绘制受试者工作特征（ROC）曲线,行DeLong检验。结果：(1)皮损组织病理检查确诊40例为JXG,1例皮脂腺异位,1例spitz痣,1例肥大细胞瘤。(2)确诊患儿皮肤镜图像特征：15例皮损外观呈“落日征”,中央线状、分枝状血管;23例外观“落日征”,中央黄色小球云、棕褐色色素沉着、白色纤维条;2例呈“云状”棕黄色。(3)确诊患儿RCM图像特征：34例真皮层见大量形状不规则中等折光细胞,不等量环状靶形细胞;4例未见环状靶形细胞;2例角化过度,灶状角化不全,棘层以下显影不清。(4)RCM与皮肤镜单独诊断JXG的ROC曲线下面积比较,差异无统计学意义（P>0.05）;单用皮肤镜、单用RCM分别与二者联合应用诊断JXG的ROC曲线下面积比较,差异均有统计学意义（P<0.05）。结论：皮肤镜及RCM可作为JXG早期筛查方法,二者联合比单独诊断更有价值。     

An Unusual Juvenile Xanthogranulomaon a Finger MCP Joint

Juvenile xanthogranuloma (JXG) is a benign self-limited histiocytic proliferative disorder that usually occurs in early childhood. JXG appears as reddish to yellow, papules, or nodules, and although the head, neck, and trunk are the most frequent locations, it can occur at any body site. However, JXG involving the finger is rare. Histologically, JXG is characterized by an ill-defined, unencapsulated, dense histiocytic infiltrate within the dermis, some of which is contained in Touton giant cells, foreign body giant cells and foamy cells. Because the cutaneous lesions spontaneously regress, treatment is not usually indicated. The authors report a case of JXG in a 4-year-old girl who had tender, yellowish papule on the ventral aspect of the MCP joint of the right fourth finger consistent with JXG.     

尖锐湿疣HPV导流杂交快速基因分型及临床意义

目的：探讨外生殖器及子宫颈尖锐湿疣患者皮损脱落细胞或组织中人乳头瘤病毒(Human PapillomaVirus，HPV)9种常见的基因亚型感染分布状况及临床意义。方法：采集患者生殖器或子宫颈尖锐湿疣组织或脱落细胞样本，分离DNA，PCR扩增后与9种常见HPV亚型探针进行导流快速反斑点印迹杂交检测HPVDNA。结果：经PCR扩增并通过通用探针杂交分析后，108例患者标本中，101例标本呈HPVDNA阳性，7例HPV阴性。HPVPCR阳性标本中，98例成功分型。5种高危型HPV16，18，3l，33，58的检出率分别为31．5％(34／108)、16．7％(18／108)、6．5％(7／108)，4．6％(5／108)，13．0％(14／108)。三种低危型HPV6，11，53型的检出率分别为41．7％(45／108)，56．5％(61／108)和6．5％(7／108)。危险度待定HPV亚型CP8304检出率为4．6％(5／108)。结论：结论HPV11、6、16型感染是尖锐湿疣的主要型别。     

Juvenile Xanthogranuloma with Hematologic Changes in Dizygotic Twins: Report of Two Newborn Infants

Abstract: We describe the first reported instances of juvenile xanthogranuloma (JXG) in dizygotic twins. They had characteristic skin lesions and subcutaneous nodules as well as hepatomegaly, anemia, and thrombocytopenia. These extracutaneous symptoms improved in 5 months, coincident with the resolution of the skin lesions. Although most patients with JXG have only cutaneous symptoms, some show such dramatic extracutaneous manifestations that the possibility of malignant disease is occasionally the principle concern. It is therefore necessary to make a precise diagnosis based on specific immunohistochemical and ultra-structural findings, and to evaluate for other organ involvement, including hematologic abnormalities.     

Juvenile xanthogranuloma: A clinical, histopathologic and immunohistochemical study

Juvenile xanthogranuloma (JXC) is a benign histiocylic proliferation of uncertain histogenesis which usually resolves spontaneously. Histopathologically, classic lesions are characterized by diffuse proliferations of foamy histiocytes, many of which may be multinucleated (Toulon cells), admixed with lymphocytes and eosinophils. Histologic variants of JXG, perhaps representing evolving lesions, may lack these typical histopathological features, showing diffuse infiltrates of non-foamy mononuclear histiocytes without Toulon cells, posing problems in differentiation from other histiocylic or melanocylic proliferations. Immunohistochemically, JXG is characterized by variable expressions of several histiocytic markers as well as the absence of staining for SI00 protein. To assess better the spectrum of histopathological and immunohistochemical features of JXG, we studied nine cases of classic or histologic variant of JXG. The cases were evaluated by light microscopy and with an extensive battery of antibodies. All 9 cases, regardless of their light microscopic appearance, showed markedly positive staining with histiocytic markers including CD68, HAM56, cathepsin B and vimentin, but did not stain for S100 protein. Antibodies to factor XIIIa stained positively in 8 cases while staining for other markers was variable. Our results suggest that the histiocytes in JXG lesions have macrophagic differentiation, probably representing a reactive process to an unknown stimulus.