Ehlers-Danlos syndrome – vascular type (ecchymotic variant): cutaneous and dermatopathologic features

Ehlers-Danlos syndrome – vascular type, the only lethal form, is rarely reported in dermatology literature. It is characterized by translucent, atrophic skin, easy bruising, arterial, intestinal and/or uterine fragility manifesting as varicose veins, aneurysms and vascular/visceral/uterine rupture. As its dermatopathologic features are not well elucidated, diagnosis is often made after a catastrophic complication or at autopsy. This 36 year-old non-consanguineous male had brown-black plaques with atrophy and frequent ulceration over legs and dorsal feet and tortuous varicose veins around ankles for the past 15 years. Perivenous skin was translucent and hypopigmented. He had multiple and ecchymotic keloids and small atrophic, pityriasis versicolor-like lesions over trunk. He did not have hypermobile/hyperextensible skin and joints and showed no systemic or investigative abnormality. Histopathologic features of atrophic lesion included blood extravasation in atrophic epidermis/dermis, focal clustering and dilatation of blood vessels, malformed vessel walls, abundant hemosiderin in the dermis and homogenously stained/whorled patterned collagen especially around blood vessels. Pathology of keloidal lesion showed new collagen and vascular fragility. These histopathologic features appear of diagnostic value especially in patients who have compatible clinical findings but cannot afford confirmation by biochemical testing for abnormal synthesis of type III procollagen or identification of mutations in the COL3A1 gene.     

Nucleolin protein expression in cutaneous melanocytic lesions

Background:  Nucleolin is a major nucleolar argyrophilic protein involved in carcinogenesis. There are only few studies on its tissue expression in human cancer and none in melanoma. We aimed at exploring this protein and its prognostic impact in cutaneous melanocytic lesions.
Methods:  We studied 193 cases including benign, dysplastic and malignant melanocytic lesions. Nuclear positivity was evaluated by immunohistochemistry and quantified by automated image analysis.
Results:  Most dysplastic and malignant lesions showed high percentages of cells with abnormal patterns of nuclear positivity (Abn+N) consisting in multiple, irregular, positive dots (ID+) and a coarse, irregularly positive nucleoplasm (CNpl+) or both (ID+CNpl+). The patterns CNpl+ and/or ID+CNpl+ were never observed in benign lesions, in which ID+ were also virtually absent. Abn+N% was significantly lower in dysplastic nevi than in primary melanomas and metastases and in primary melanomas than in metastases (p < 0.05). Furthermore, Abn+N was the second powerful prognostic discriminator, after melanoma thickness, and a significantly lower survival was observed in vertical growth phase melanoma patients showing Abn+N in more than 50% of melanoma cells.
Conclusion:  An altered nuclear nucleolin expression seems to accompany melanoma progression. Further investigation on nucleolin functionality and subcellular trafficking could add information on its altered role in melanoma.     

1例系统性红斑狼疮患者视网膜中央动脉阻塞及脑出血的护理

对1例系统性红斑狼疮并发视网膜中央动脉阻塞及脑出血患者，给予糖皮质激素、免疫抑制剂、高压氧、改善微循环及对症支持治疗．同时予以密切观察病情、药物护理、高压氧治疗护理及健康教育等措施，结果患者恢复良好出院。提出该病虽然少见且后果严重，但及时采取综合性治疗护理措施，可以提高治疗效果。     

The effect of ethanol intake on tendon healing: a histological and biomechanical study in a rat model

Introduction  

Ethanol has a suppressive effect on inflammation and the immune system, but the effect of ethanol on tendon healing in vivo has not been studied. The purpose of this study was to investigate the histological and biomechanical effects of ethanol intake on tendon healing in a rat tendon injury model.     

膀胱肿瘤2350例临床病理学特点分析

目的 探讨膀胱肿瘤的发病现状、总体趋势和病理特点.方法 整理1980-2007年2350例膀胱肿瘤病理档案,分为1980-1989、1990-1999和2000-2007年3个时间段.应用SPSS 13.0软件分析3时间段患者性别、年龄及肿瘤组织学类型间的关系.结果 2350例膀胱肿瘤中男1854例,女496例.良性92例,恶性2258例,膀胱恶性肿瘤的发病人数逐年上升.发病高峰年龄从50～69岁推迟到60～79岁.1980-1989、1990-1999和2000-2007年3个时间段中男、女恶性肿瘤病例数分别为524例和113例(4.64:11 00)、589例和164例(3.59:1.00)、675例和193例(3.50:1.00),男性约为女性的3.80倍;3时间段男性发生膀胱尿路上皮癌年龄分别为(57.5±11.7)、(62.6±12.3)、(65.9±11.3)岁,女性分别为(58.7±13.6)、(60.7±12.1)、(65.8±12.0)岁,男女各年龄段分别比较差异有统计学意义(P<0.05).男性发生鳞状细胞癌、尿路上皮癌和腺癌年龄分别为(68.05±9.7)、(59.85±14.1)、(63.4±9.9)岁,差异有统计学意义(P相似文献   

An unusual cause of adult onset cerebellar ataxia with hypogonadism

We report an unusual case of sporadic adult onset cerebellar ataxia with hypogonadism. A 40-year-old unmarried man presented with progressive ataxia and dysarthria along with complaints of non-development of secondary sexual characteristics and erectile dysfunction. There were complaints of intermittent diarrhea. Clinical examination revealed a pan-cerebellar syndrome with features of hypoandrogenism. No eye movement abnormalities were evident. There were signs of malabsorption. Investigations confirmed the presence of auto-antibodies found in celiac disease, and a duodenal biopsy confirmed the same. Hypoandrogenism was postulated to be due to hypergonadotropic hypogonadism which has been mentioned in a few patients of celiac disease. However, the pattern seen in our patient was of a hypogonadotropic hypogonadism. This is probably secondary to an autoimmune hypophysitis seen in some patients in the absence of other clinical manifestations. Autoantibody testing should be a diagnostic necessity in any adult with a sporadic cerebellar ataxia.     

多节段髓内星形细胞瘤的临床研究

目的 探讨不同年龄段、恶性程度不同的多节段髓内星形细胞瘤患者的诊治特点.方法 21例多节段(3个椎体节段以上)髓内星形细胞瘤患者均行后正中入路显微镜下肿瘤切除术,以UOA分值差(术后UOA和术前UOA差值)评估手术效果.以Logistic回归和多元线性同归分析统计学数据.结果 肿瘤性质直接影响肿瘤的切除程度(X2=10.41,P=0.03),高度恶性肿瘤只能大部切除或减压.年龄(X2=12.53,P=0.01)、肿瘤性质(X2=16.44,P=0.002)和部位(X2=27.12,P=0.04)影响近期手术效果(UOA分值差);青少年患者、低度恶性肿瘤手术效果较好,单纯累及胸段的患者手术效果差.结论 低度恶性或青少年多节段髓内星形细胞瘤患者应尽早手术,术后疗效满意.