以急性发热性嗜中性皮病样皮疹为表现的白塞病1例

白塞病是一种多系统自身免疫性疾病,以急性发热性嗜中性皮病(Sweet综合征)样皮疹为表现的白塞病临床比较少见[1],现临床报道1例.     

急性发热性嗜中性皮病继发皮肤松垂和致死性心血管病

急性发热性嗜中性皮病(Sweet综合征)是一种成人良性自限性疾病.Heyl等曾首先报导一例原因不明的急性皮炎之后继发皮肤松垂死于心肌梗塞的病人.本文报导一例儿童急性发热性嗜中性皮病之后,发生皮肤松垂和致死性心血管病,并为尸检     

Sweet综合征与恶性肿瘤

Sweet于1964年首先以急性发热性嗜中性皮病报告的Sweet综合征(SS)有5个主要的特征:1.发热;2.嗜中性白细胞增多;3.多发性、高起的、不对称、红斑性、痛性皮肤斑块;4.由成熟的嗜中性细胞组成的浓密的皮肤浸润;5.对类固醇激素治疗迅速获效.本文复习了全世界有关伴有恶性肿瘤的急性发热性嗜中性皮病的文献,并进行了综述.SS与血液的恶性肿瘤最常发生于SS患者的肿瘤是急性髓细     

类风湿性关节炎的皮肤粘膜表现

类风湿性关节炎是一种以关节损害为主要特征的系统性风湿性关疾病，皮肤亦为其受累的靶器官之一，该文介绍了类风湿性关工一身病主卢的皮肤粘膜损害，包括非异特异性炎症的表现，类风湿结节，类风湿性关血管炎，类风湿性丘疹，类风湿性关嗜中性皮病以及溃疡和坏疽性脓皮病，并对自身免疫性大疱性皮病，急性发热性嗜中性皮病，Ｓｊｏｇｒｅｎ综合征，淀粉样变性等与类风湿性关节炎相关的皮肤疾病及其特征作一概述。     

类风湿性关节炎的皮肤粘膜表现

类风湿性关节炎是一种以关节损害为主要特征的系统性风湿性疾病,皮肤亦为其受累的靶器官之一。该文介绍了类风湿性关节炎本身病变引起的皮肤粘膜损害,包括非特异性炎症的表现、类风湿结节、类风湿性血管炎、类风湿性丘疹、类风湿性嗜中性皮病以及溃疡和坏疽性脓皮病,并对自身免疫性大疱性皮病、急性发热性嗜中性皮病、Sj(?)gren综合征、淀粉样变性等与类风湿性关节炎相关的皮肤疾病及其特征作一概述。     

Sweet综合征临床研究进展

Sweet综合征又称急性发热性嗜中性粒细胞皮病,是一种少见的特发性疾病,近年来其临床研究有些新的进展.在分类上,发现一种局限性亚型,称为"手部嗜中性皮病",在临床表现上则发现更多不同于典型表现的皮疹,而组织学上皮损的炎性浸润方式及深度有所扩展,诊断及系统评估方法亦更加完善.     

血管炎的实验室检查

血管炎的实验室检查，不仅对肯定血管炎诊断有一定的价值，而且在分析病因和发病机制，指导临床分类诊断以及治疗均有较重要的意义。1常规检查包括血尿常规、血沉、肝肾功能等。1．1血常规白细胞总数升高和中性粒细胞比例增加，不仅见于细菌感染引起的血管炎，更多可见于急性发热性嗜中性皮病、结节性多动脉炎、Wegener肉芽肿、白塞病、荨麻疹性血管炎等。嗜酸粒细胞升高见于嗜酸性蜂窝织炎、面部肉芽肿、Wegener肉芽肿、变应性皮肤血管炎、结节性多动脉炎。贫血见于严重的变应性皮肤血管炎、结节性多动脉炎、Wegener肉芽肿、白塞病等。在各种类型的血管炎中血小板的质和量均基本正常或暂时性降低。     

以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例

 报告以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例。患者女,50岁,反复全身红色斑块伴疼痛4个月,加重2 d。皮肤科检查：颈部及双上肢分布大小不等红色斑块,皮损边缘略隆起于皮面,呈环状,未见水疱及破溃,触痛阳性。上唇少许糜烂面。组织病理符合急性发热性嗜中性皮病改变。入院后查血液系统异常,诊断急性髓系白血病,以急性发热性嗜中性皮病为首发症状。予EA方案预化疗。三个月后患者因严重脑出血死亡     

急性发热性嗜中性皮病的皮下嗜中性白细胞浸润

本文报告一例白血病患者,伴发急性发热性嗜中性皮病(ND),其病理表现为限于皮下组织的嗜中性白细胞浸润。女性患者,52岁,因发热、皮下结节,疑有恶性血液肿瘤而住院。体检:T37.8℃,在臂及股内侧有多个2～3cm大小红斑结节,触痛。血细胞容积24％,白细胞计数16900,中性白细胞72％,髓母细胞4％。骨髓活检显示白血病前期改变,符合进行性急性髓样或髓性单核细胞白血病。皮肤结节活检可见致密的片状成熟的嗜中性白细胞浸润,局限在真皮下1～2mm处皮下脂肪组织小叶内,     

Sweet综合征与白血病

一、Sweet 综合征的回顾远在1949年,Sweet 就开始收集一组少见的病例,到1964年他首先报告了8个病例,指出该病具有发热、疼痛性隆起性红斑、血中嗜中性白细胞增多、组织病理表现为真皮浅层有显著的嗜中性白细胞浸润等四项主要特征,命名为急性发热性嗜中性白细胞增多性皮病。1968年 Sweet 本人又补充报告了4例具有相同症状的患者,并且进一步提出在     

Dermatosis neglecta: a series of case reports and review of other dirty-appearing dermatoses

Localized scaling and hyperpigmentation is a common finding and often a diagnostic and therapeutic challenge. Dermatosis neglecta (DN) represents a failure to adequately clean or scrub the skin, often in an area of hyperesthesia or prior trauma. We identified five cases of DN in two clinical practices in just the past few years. The condition is characterized by scrupulous avoidance of scrubbing, leading to a buildup of hyperpigmented, adherent, cornflake-like scales. An alcohol swabbing provided diagnosis and prompt clearing. Washing with soap and a washcloth also often leads to clearing. Dermatosis neglecta should be considered in the differential diagnosis of hyperpigmented scale; alcohol swabbing can serve as a diagnostic and therapeutic tool.     

Neutrophilic eccrine hidradenitis in the absence of an underlying malignancy

Neutrophilic eccrine hidradenitis is an entity previously reported to occur in association with malignancy and chemotherapy. We report a case of clinical and histopathologic findings characteristic of neutrophilic eccrine hidradenitis occurring without apparent underlying disease. In addition, the only medication this patient was taking was acetaminophen. Neutrophilic eccrine hidradenitis appears to represent a reaction pattern without specificity for underlying disease or inciting agent.     

Neutrophilic dermatosis of the dorsal hands related to cocaine abuse

Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.     

Serum interleukin 18 and tumour necrosis factor-alpha levels are increased in Behcet's disease

Inflammation in Behcet's disease is thought to be mediated by cytokines derived from T-helper type 1 (Th1) lymphocytes. In this study, we tried to determine serum interleukin (IL)-18 and tumour necrosis factor (TNF)-alpha levels of patients with Behcet's disease. Twenty-seven patients with active Behcet's disease, and 20 healthy control subjects were included in this study. Differences between mean serum IL-18 and TNF-alpha level of patients with Behcet's disease were significantly increased when compared with the control group. A significant correlation was found between serum IL-18 and TNF-alpha levels of Behcet patients (rs = 0.627, P < 0.0001). IL-18 and TNF-alpha levels may be related to disease pathogenesis. Increased levels of IL-18 also support Th1 predominance in Behcet's disease.     

Dermatosis Papulosa Nigra in a Young Child

Abstract: Dermatosis papulosa nigra was diagnosed in a 3-year-old black boy. This folllcular nevoid condition, which is common in adult blacks, is seldom diagnosed in prepubescent children. The diagnosis was confirmed by the biopsy specimen that showed features of epidermal acanthosls and papillomatosis, similar to seborrheic keratosis.     

Familial cases of Behcet''s disease

Seven families with Behcet's disease are presented. HLA-B5 tissue type was shown in the three families in whom lymphocyte microcytotoxicity tests were carried out. Genetic factors appear to be important in the pathogenesis of Behcet's disease.     

N-acetyltransferase 2 polymorphisms in patients with Behcet's disease

It is possible that dietary, environmental factors and/or genetic polymorphisms in xenobiotic-metabolizing enzymes may contribute to the development of Behcet's disease. As N-acetyltransferase (NAT) 2 is an important xenobiotic-metabolizing enzyme and theoretically the nonacetylated xenobiotics may induce an autoimmune mechanism, the aim of the present study was to investigate whether the genetic polymorphism of NAT2 plays a role in susceptibility to Behcet's disease. Forty Behcet's disease patients and 82 control subjects were enrolled in the study. NAT2*5A, NAT2*6A, NAT27*A/B and NAT2*14A polymorphisms were detected by using real time PCR with LightCycler (Roche Diagnostics GmbH, Mannheim, Germany). The NAT2*5A and NAT2*6A mutant genotypes carried an increased risk of developing Behcet's disease [odds ratio (OR) = 66.29, 95% confidence interval (CI) = 8.21-535.33; and OR = 24; 95% CI = 2.04-304.98, respectively]. The NAT2*7A/B and NAT2*14A gene polymorphisms were not an increased risk for developing Behcet's disease. As a result of this study we conclude the NAT2 slow acetylator status may be a determinant in susceptibility to Behcet's disease. This finding may have implications for the theories of the pathogenesis of the disease as well as for therapeutic aspects.     

Behcet病21例临床分析及眼内超声表现

目的：探讨Behcet病的临床特征以及超声检查对眼部损害的诊断价值。方法：回顾性分析了21例Behcet病的临床资料，包括8例眼部损害患者（13只眼）的超声图像特点。结果：口腔溃疡、外生殖器溃疡、皮肤损害、眼部病变是本组Behcet病的基本临床特征，以口腔溃疡最常见。前房积脓，玻璃体雪球状浑浊，伴粟粒样强回声光点及纤维光带形成等是Behcet病的主要超声表现。结论：某些口腔溃疡反复发作常规治疗效果欠佳时，可能是Behcet病的早期表现。超声检查可对Behcet病眼部损害以及本病的早期诊断提供有用的影像学依据。     

象限性色素性紫癜性皮病1例

患者男,50岁。左大腿外侧瘀点和瘀斑5月余。皮损组织病理示:表皮轻度角化过度,基层灶性液化,真皮浅层胶原纤维增生,毛细血管扩展充血,血管周围可见单核细胞、淋巴细胞浸润及外溢的红细胞及含铁血黄素。诊断:象限性色素性紫癜性皮病。