获得性指(趾)部纤维角化瘤12例

目的 探讨获得性指(趾)纤维角化瘤临床及病理特点.方法 对确诊为获得性指(趾)部纤维角化瘤患者的临床表现、组织病理特点和治疗方法及转归进行分析.结果 12例患者发病年龄17～64.5岁(39.75±17.69)岁,病程0.5 ～ 20年(4.83±5.77)年,男:女为1.4∶1.皮损表现为多形态,主要累及指、趾、甲沟,也可累及非肢端部位.皮损组织病理检查:表皮角化过度和棘层肥厚,表皮突增宽呈分枝状,皮损中心由成熟的胶原束、成纤维细胞和大量扩张毛细血管组成.其中大部分胶原束与表皮相垂直走行,为其特征性表现.结论 获得性指(趾)部纤维角化瘤是一种少见、原因不明的皮肤纤维组织良性肿瘤,多发于成年人指或趾,一般为单发,但也可发生于非肢端.采取手术或激光治疗,少有复发.     

表现为色素性对称性肢端角化性皮损的一组患者临床分析

目的 报道一组表现为色素性对称性肢端角化性皮损患者的临床表现、组织病理学及治疗结果。方法 系统地收集了自1998年5月至2007年9月来我科就诊的以色素性对称性肢端角化性皮损为表现的16例患者的临床资料，包括临床表现、皮损组织病理学改变、治疗、性别、年龄、职业、家族成员情况等。结果 男14例，女2例；年龄9 ~ 54岁；病程2个月至8年，平均（2.0 ± 1.5）年。主要临床表现为出现棕褐或棕黑色斑，均呈对称性斑丘疹，表面粗糙，有少许脱屑，伴轻度瘙痒。皮损部位：16例（100%）患者在双手掌指背、手腕，2例（12.5%）双肘及双膝，3例（18.8%）双踝部。组织病理学表现：表皮角化过度，棘层增厚，乳头瘤样增生；真皮少许淋巴细胞浸润。家庭成员无类似病史。给予口服维A酸类、烟酰胺、维生素B6等，外用维A酸软膏、糖皮质激素类软膏等，疗效欠佳，或病情反复。结论 这是一组主要以肢端出现对称性色素性角化斑或斑丘疹，组织病理学表现以表皮角化过度、棘层增厚、乳头瘤样增生为特征的皮肤病，病因不明。     

47例青斑血管炎临床分析

【摘要】 目的 探讨青斑血管炎患者的临床与病理特点。 方法 分析47例青斑血管炎患者临床资料,主要治疗措施为单纯抗凝,或抗凝联合糖皮质激素抗炎,或抗凝联合柳氮磺吡啶抗炎。结果 临床表现为：皮损首先表现为瘀点或瘀斑、水肿伴胀痛,在此基础上出现点状坏死,逐渐出现虫蚀状溃疡,最后形成白色萎缩。组织病理：真皮浅层或伴真皮深层小血管壁纤维素样变性与管腔内血栓形成、受损血管周围可见稀疏淋巴细胞浸润;单纯抗凝组、抗凝联合糖皮质激素组及抗凝联合柳氮磺吡啶组的平均起效时间分别为（9.14 ± 3.48）、（5.62 ± 1.04）、（8.23 ± 2.68） d,而疾病平均缓解时间分别为（2.57 ± 1.41）、（4.06 ± 1.51）、（5.64 ± 1.32）个月。结论 抗凝联合抗炎治疗青斑血管炎可能比单纯抗凝治疗起效更快,有利于控制复发。 【关键词】 青斑血管炎; 抗凝药; 抗炎药; 病理学     

疣状肢端角化症的临床及病理特点研究

目的：探讨疣状肢端角化症的临床及病理特点。方法：分析本文及近40年来13篇文献报道的17例肢端角化症先证者的临床及病理资料。结果：发病年龄，10例（59％）〈20岁，7例（41％）〉25岁；有家族史者10例（59％）。皮损主要分布于双手背、足背及前臂、小腿伸侧。皮损表现16例（94％）为扁平疣样损害，1例表现为寻常疣样损害，6例（35％）伴有掌跖角化，5例（29％）伴有指趾甲受累，3例（18％）伴有毛囊角化病。皮损组织病理均表现为表皮明显角化过度、颗粒层及棘层增厚、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，无角化不全及空泡化细胞。结论：疣状肢端角化症大部分患者有家族史，常于加岁前发病。皮损主要分布四肢伸侧，基本皮疹为扁平疣样的褐色扁平丘疹。皮损病理显示表皮明显角化过度、乳头瘤样增生及塔尖样表皮局限性隆起等特征性变化，是诊断疣状肢端角化症的主要依据。     

获得性指状纤维角化瘤7例临床分析

目的:加深对获得性指状纤维角化瘤临床及组织病理特点的认识.方法:回顾性分析2008-2011年间我科确诊为获得性指状纤维角化瘤患者的临床表现、组织病理特点和治疗反应.结果:7例患者平均发病年龄(35.6±10.1)岁(26～52岁),平均病程(3.3±4.0)年(0.5～10年),男女比例1∶1.3.皮损表现为单发、大小不等、肤色的增生性肿物,呈圆顶状或柱状.主要累及指、趾部,也可见于手掌及甲沟等部位.组织病理学检查:表皮角化过度,颗粒层增厚,棘层不规则肥厚,表皮突增宽、延长;真皮乳头大量扩张毛细血管及粗大纤维增生,与真皮乳头平行.结论:获得性指状纤维角化瘤为单发增生性肿物,常发生在指、趾部,也可累及肢端其他部位.皮损组织病理检查有特征性改变,切除可治愈.     

银屑病患者血清C3a和C4a的检测

据报道［１～４］在银屑病中外周血可溶性白介素２受体（ｓＩＬ－２Ｒ）水平可不同程度升高。我们应用ＥＬＩＳＡ技术对寻常型银屑病（ＰＶ）患者及正常人进行了血清ｓＩＬ－２Ｒ的检测，以期对银屑病患者的细胞免疫功能作进一步的观察。一、资料和方法１．检测对象：ＰＶ患者４４例，其中男２５例，女１９例。年龄２１～６０岁，平均３８岁。病程１５天至３０年，平均１０．１５±９．１９年。进行期２６例，静止期１８例；皮损斑块状者３０例，点滴状者１４例；泛发全身者３０例，局限于头部、躯干或四肢者１４例。银屑病的皮损严重程度采…     

环状扁平苔藓一例

本例患者的临床与组织病理表现符合扁平苔藓的诊断,但有其特殊之处：（1）无口腔黏膜损害,皮疹分布于上背部及右侧髋部。（2）无瘙痒症状。（3）两处皮损在临床上形态完全不同。上背部皮损表现为环形堤状隆起,临床上类似于汗孔角化病。右侧髋部皮损为椭圆形,边缘为淡红色隆起性边缘,中央褐色色素沉着,表面附着鳞屑。（4）两处皮损虽然临床表现不同,但组织病理表现相同,均符合扁平苔藓的特征,并且均可见到表皮下疱形成。     

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...     

16例季节性对称性肢端角化症临床及病理超微结构分析

目的:对16例季节性对称性肢端角化症患者进行临床及病理超微结构分析,归纳总结其特点。方法:追踪随访并收集我所皮肤科门诊2010年3月至2012年11月16例季节性对称性肢端角化症患者的临床资料,对比治疗前后患者皮损的病理及超微结构的改变。结果:16例患者中男11例,女5例,平均年龄(28.3±2.1)岁,平均发病年龄(19.5±1.2)岁。通常春末夏初发病或复发,夏季加重,秋末冬初减轻或消失;皮损主要对称性分布于手腕、手背、踝部、膝部、足背、肘部、指蹼、手侧缘、肘窝,表现为色素性苔藓样斑片或斑块,无明显不适;真菌学检查阴性;组织病理学表现为表皮角化过度和乳头瘤样增生;超微结构显示角质层层数增多,角质化包膜连续性差,角蛋白丝结构紊乱成撕裂状,颗粒层及棘层上部细胞核周围可见异常聚集的角蛋白微丝。结论:本病发病有明显的季节特点,病理及超微结构类似于大疱性鱼鳞病样红皮病,其发病机制可能与角蛋白代谢相关。     

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。     

皮肤纤维瘤与隆突性皮肤纤维肉瘤的临床病理比较

对皮肤纤维瘤（DF）与隆突性皮肤纤维肉瘤（DFSP）在临床、组织形态及免疫组化等方面进行了比较。DF20例,平均年龄37.75岁,好发于四肢,平均直径0.8cm。瘤细胞呈束状或旋涡状排列,局部可见车辐结构。平均每50个高倍视野核分裂为0.75个。常伴组织细胞和炎细胞浸润、胶原增生、表皮增生及黑色素增多,CD34（－）。瘤细胞密度与胶原增生及炎细胞浸润呈负相关,提示DF可能为反应性病变。DFSP10例,平均年龄49.1岁,好发于躯干,平均年龄49.1岁,好发于躯干,平均直径3.23cm。瘤细胞呈典型的车辐状排列,平均每50个高倍视野核分裂为5.5个。肿瘤背景清晰,少数可见炎细胞浸润及表皮增生,无胶原增生及黑色素增多,CD34（＋）。这些临床及病理特征有助于两者的鉴别诊断。     

Silica-induced scleroderma

In a survey done in East Germany between 1981 and 1988, we found that 93 of 120 male scleroderma patients had long-term exposure to silica dust. We describe our findings in 12 patients with scleroderma and silicosis. The exposure time to silica dust was between 3 and 34 years; the interval between the beginning of exposure and the onset of scleroderma averaged 27.3 years (range 9 to 40 years). Antinuclear antibodies in titers between 80 and 10,240 with nucleolar and/or speckled patterns were found in 10 patients, antibodies against double-stranded DNA in three, Scl-70 (topoisomerase I) in three, and anticentromere antibodies in five. The following markers of collagen metabolism were increased in serum: beta-galactosidase in 12 patients, laminin peptide-P1 in 10 patients, N-terminal procollagen type III peptide in 10, and urinary sialic acid excretion in 7. We propose that crystalline particles of silica less than 5 microns may be phagocytosed by macrophages and release lymphokines and monokines, which activate fibroblasts and enhance their collagen and glycosaminoglycan synthesis. In addition, silica may act as an adjuvant to increase immune reactivity.     

Increased expression of collagen types I and III in human skin as a consequence of radiotherapy

To study the mechanisms of irradiation-induced fibrosis, the expression of types I and III collagen was analysed in radiotherapy-treated human skin. The subjects were ten randomly chosen women who had been treated for breast cancer with surgery and radiotherapy. The subjects ranged in age from 42 to 68 years (mean 53 years) and the time from treatment ranged from 7 to 94 months. The irradiated skin was compared with a corresponding healthy skin area in the same subject. Suction blisters were induced on both skin areas. The aminoterminal propeptides of types I and III collagen (PINP and PIIINP), which reflect actual in vivo skin collagen synthesis, were determined in the suction blister fluid using radioimmunoassays. mRNA of types I and III collagen were determined in skin specimens using a nonradioactive in situ hybridization (ISH) technique. Immunohistochemical staining for PINP was also performed. The level of PINP in suction blister fluid was increased more than threefold and the level of PIIINP more than twofold in irradiated skin compared to control skin. The number of cells containing type I and type III collagen mRNA was increased in the upper dermis of irradiated skin. Immunohistochemical staining showed the amount of PINP-positive fibroblasts to be increased in irradiated skin. We conclude that skin collagen gene expression is increased as a result of irradiation and this leads to fibrosis and thickening of the dermis.     

Recovery of human skin collagen synthesis after short-term topical corticosteroid treatment and comparison between young and old subjects

Summary In the present study, the recovery of the collagen synthesis rate after topical potent glucocorticoid treatment in the human skin in vivo was investigated. In the first experiment, two age groups were compared: young subjects with an age range of 21–26 years (mean 23), and old subjects, aged 55–70 years (mean 64). Twenty healthy male volunteers applied betamethasone-17-valerate to their abdominal skin for 3 days twice a day. Suction blisters were induced on the treated areas, and on the opposite side (healthy non-treated skin), of the abdominal skin on the day following the discontinuation of the treatment, and on the second and seventh day. In another experiment, suction blisters were induced after the treatment and 2 weeks later on the treated area and on healthy skin, in eight male volunteers. In both experiments, the aminoterminal propeptides of type I and III collagens (PINP and PIIINP, respectively) were measured radioimmunologically from the suction blister fluid. Corticosteroid treatment decreased the collagen synthesis in both age groups after a 3-day treatment period, and essentially no recovery in the collagen synthesis could be seen during a 1-week corticoid-free period. The inhibition and downregulation of collagen synthesis in the corticoid-treated skin was similar in both young and old subjects, up to 7 days after the treatment. During the 2-week corticoid-free period, collagen synthesis was recovered to about 50% of the level seen in the non-treated skin. Indicating that collagen synthesis is not completely normalized in the human skin even during a 2-week corticoid-free period.     

Acquired nevoid telangiectasia

BACKGROUND: Acquired nevoid telangiectasia (ANT) is a segmental dilatation of papillary plexus vessels. OBJECTIVE: In the present study, the frequency of ANT and its associations with collagen vascular and spinal disease have been investigated. METHODS: 188 unselected patients, seen at an interdisciplinary dermatorheumatologic outpatient clinic, were clinically examined for the presence of ANT and possible associations to preexisting disease. RESULTS: ANT was seen in 17% of patients, all females. The mean age of ANT patients was 57.5 +/- 8.2 years. Most common diseases were lupus erythematosus and scleroderma. Twenty-four of them suffered from generalized fibromyalgia, 8 had spondylitis deformans, 1 suffered from thoracal syringomyelia and 1 had a spine trauma. Two types of ANT have been seen: the cervicothoracal transition type and the lumbosacral transition type. CONCLUSIONS: ANT is not uncommon among patients attending a dermatorheumatologic outpatient clinic. ANT indicates spinal or neuromuscular complaints, but is not related to collagen vascular disease itself.     

Successful ultraviolet A1 phototherapy in the treatment of localized scleroderma: a retrospective and prospective study

Background Ultraviolet (UV) A1 phototherapy is an effective anti‐inflammatory treatment modality that influences fibroblast functions. Objectives To document the effects of UVA1 treatment in patients with localized scleroderma (LS) in a retrospective study (at least 6 months after UVA1 treatment) and in a prospective study before and immediately after medium‐dose UVA1 irradiation. Methods In total, 30 patients (retrospective study n = 17, prospective study n = 13) with LS receiving UVA1 phototherapy five times weekly (for 3–6 weeks) were investigated. Improvement was documented using standardized questionnaires and clinical evaluation (using modified Rodnan skin score, Cutometer and 7·5‐MHz ultrasound measurements). Levels of collagen I and collagen III metabolites were measured in serum and urine. Results In the retrospective study, medium‐dose UVA1 phototherapy had been performed 6 months–3 years earlier (cumulative dose 750–1400 J cm^?2; mean ± SD number of irradiations 19·3 ± 3·8). Fourteen of 17 patients (82%) reported an improvement in symptoms following UVA1 therapy. In the prospective study, skin elasticity increased in 77% of the patients following medium‐dose UVA1 phototherapy (cumulative dose 750–1250 J cm^?2; mean ± SD number of irradiations 20·8 ± 4·0). 7·5‐MHz ultrasound measurements showed a mean reduction of lesional skin thickness of 13% compared with skin thickness before UVA1 phototherapy. The ratio of deoxypyridinoline to creatinine was significantly elevated in about two‐thirds of the patients. Conclusions This open study showed a positive short‐ and long‐term efficacy of UVA1 phototherapy in patients with LS, with a reduction in sclerotic plaques, an increase in skin elasticity and a reduction of lesional skin thickness. UVA1 phototherapy had a significant effect on collagen metabolism. UVA1 phototherapy can be regarded as a safe treatment modality for patients with LS.     

Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern

Background Interstitial granulomatous dermatitis (IGD) is a rare disease for which a clinical–pathological correlation is essential to establish diagnosis. Objectives To describe the histological and clinical features of patients with IGD seen in our department from 2004 to 2010, and to undertake a literature review and critical analysis of additional cases. Methods Twelve adult patients (nine women and three men; mean age 58·5 years; range 32–73 years) with IGD were enrolled. Lesions consisted of asymptomatic erythematous papules and plaques, symmetrically distributed on the trunk and the proximal limbs. Two patients had skin‐coloured papules. Six patients had articular involvement (arthralgias, spondyloarthritis, rheumatoid arthritis) and three patients had cancer. Results All cases showed a predominant CD68‐positive macrophage infiltrate distributed between collagen bundles of the mid‐ and deep dermis. Macrophages were also surrounding degenerated collagen fibres. A few neutrophils and/or eosinophils were also present. No vasculitis or significant mucin deposition was observed. Of the 62 cases of IGD reported since 1993, 53 fulfilled stringent diagnostic criteria. Erythematous papules and plaques on the trunk and proximal limbs were the dominant manifestation. Approximately 10% of patients had cord‐like lesions. More than 50% of patients with IGD had arthralgia or arthritis, and less commonly other rheumatic disorders. Disease duration is months to years, but long‐term prognosis seems favourable. Conclusions IGD is a distinct entity with a typical histological and clinical pattern. The importance and the nature of the association with extracutaneous diseases remains to be clarified. Patients should be screened for rheumatic and autoimmune diseases.     

Sclerotic (fibroma-like) lipoma: a distinctive lipoma variant with a predilection for the distal extremities

This report details the clinicopathologic and immunohistochemical findings identified in 21 cases of a fibrosclerotic variant of lipoma from acral sites that is frequently misdiagnosed as a fibromatous (nonlipogenic) process. The study includes 12 males and 9 females; aged 7 to 72 (mean and median, 39). The patients presented with solitary, mostly asymptomatic, masses that ranged from 0.6 to 2.2 (median, 1.2; mean, 1.3) cm and involved fingers (n=17), hands or wrists (n=3), and toes (n=1). Microscopically, the lesions were well-circumscribed nodules that showed very low to moderately low cellularity and consisted of cytologically bland spindled and stellate-shaped cells and a minor component of randomly dispersed adipocytes embedded in a collagenous to myxocollagenous stroma. Eight tumors were "fibroma-like" owing to their paucity of spindled cells, dense fibrosclerotic stroma, and inconspicuous vasculature. One tumor showed features of spindle cell/pleomorphic lipoma, whereas another demonstrated a vague onion skin-like arrangement of collagen reminiscent of sclerotic (storiform) fibroma. In all cases, the mitotic activity was negligible. Nonlipogenic tumor cells were immunoreactive for CD99 (6 of 6 cases), CD34 (6 of 8), S-100 protein (4 of 7), and smooth muscle actin (2 of 6). Follow-up data on 8 patients (range, 1 to 20 years; median, 9.5 years) revealed no recurrence in 6, but indicated the possibility of persistent tumor in the remaining 2 individuals after simple excision. Despite histological overlap in 1 case with the sclerotic fibroma, no patient displayed definitive clinical features of Cowden syndrome. Our study indicates that fibrosclerotic lipomas demonstrate a broader histological scope than what was initially described.     

The use of synchrotron infrared microspectroscopy to demonstrate the effect of intense pulsed light on dermal fibroblasts

Abstract

Intense pulsed light (IPL) has been used extensively to treat striae distensae (SD). Twenty-four patients (21 females and 3 males) with age ranging between 15–42 years with a mean of 24.2 ± 5.89 years were included in this study. All patients were treated with IPL for five sessions and were given 4-week interval between the sessions. Formalin-fixed, paraffin- embedded skin biopsies were cut into 5-μm-thick sections from patients before and after treatments. They were stained with hematoxylin and eosin (H&E) and collagen with Masson trichrome stain. Histological examination of SD revealed epidermal thinning, flattened rete ridges, and fragmentation with degeneration of the collagen and elastic fibers. After IPL treatments, collagen expression was found also to be increased in a highly significant manner and P values were < 0.001 after IPL treatments. The use of synchrotron infrared (IR) microspectroscopy also revealed that amide1and beta sheets were expressed in a significant manner after IPL treatment. In conclusion, it was demonstrated that IPL can treat striae through fibroblast stimulation which led to protein expression especially collagen stimulation, and it seems that it is a promising therapeutic modality for striae with minimal side effects.