单发性肥大细胞瘤

右手背可见一1.4cm×2.5cm结节,边界清楚,中央为肤色,周边呈棕红色图1单发性肥大细胞瘤患儿手背部皮损真皮内有许多胞质内有紫红色异染颗粒的肥大细胞浸润Giemsa染色×400图3单发性肥大细胞瘤患儿皮损组织病理像表皮下水疱形成,疱液中有嗜酸粒细胞和纤维蛋白网,水疱下真皮有细胞浸润HE染色×400图2单发性肥大细胞瘤患儿皮损组织病理像患儿女,5个半月。主诉:右手背皮肤结节、水疱、血疱,伴全身反复出现红斑、风团5个半月。现病史:患儿出生时,右手小指掌指关节伸侧有一绿豆大结节,摩擦后肿大,表面起水疱、大疱或血疱,曾多次到医院就诊,经穿…     

婴儿弥漫性皮肤肥大细胞增生症

报告1例弥漫性皮肤肥大细胞增生症。患儿女，10个月。躯干水疱及大疱5个月。皮肤科检查：全身皮肤呈橘黄色、纹理粗糙、颜色较暗，背部见水疱簇集性分布，尼氏征阴性。背部散在分布色素减退斑，色素减退区域摩擦后可见皮肤发红。额部、右侧颞部可见风团。背部皮损组织病理检查：表皮内陈旧性水疱，真皮内肥大细胞弥漫性浸润，肥大细胞呈圆形或梭形，细胞质丰富，呈嗜碱性。免疫组化：CD117(+),CD2及CD25为（-）。诊断：弥漫性皮肤肥大细胞增生症。     

非典型种痘样水疱病

报告1例非典型种痘样水疱病.患儿女,6岁8个月.反复发热、皮肤出现水疱4年,水疱愈合后遗留凹陷性瘢痕.皮肤科检查:面部、颈项部、臀部和四肢伸侧散在分布黄豆大凹陷性瘢痕,下肢可见数个暗紫红色浸润性斑块,压痛不明显,未见明显水疱.血清病毒学检测:EB(Epstein-Barr)病毒IgG抗体(EBV-cA-IgG Ab)(+).3年前曾行皮损组织病理检查:表皮水肿,水疱形成,真皮及皮下可见大量淋巴样细胞、中性粒细胞及少量嗜酸性粒细胞浸润,可见血管炎改变.皮损组织EBER原位杂交(+).诊断:非典型种痘样水疱病.     

表现为大疱血疱的持久性隆起性红斑一例

患者男,75岁,因四肢伸侧红斑、水疱2年,斑块1个月,于2008年11月18日到我院就诊.2年前无明显诱因左腿胫前起红斑、小水疱,随后四肢关节伸侧逐渐出现类似皮损.搔抓后水疱破溃、结痂后愈合.在外院取大腿水疱行组织病理检查:皮损中央见一坏死痂,可见一表皮下疱,疱内见大量中性粒细胞浸润,真皮内弥漫分布大量中性粒细胞,诊断为"大疱性类天疱疮".     

水疱型钙化上皮瘤1例

患者为14岁女孩。左上臂中段伸侧一2.0cm×2.5cm半透明、厚壁、紫红色水疱持续3个月,疱内及水疱基底可以扪及一结节。发病前局部有钝物碰撞史,家族无类似病史。皮损组织病理示有嗜碱性细胞和影细胞团块,其中可见钙化区,真皮浅层有淋巴水肿,淋巴管高度扩张。皮损手术切除后随访2年无复发。     

痒疹样营养不良型大疱性表皮松解症1例

患者女,10岁。双下肢红色扁平丘疹、水疱、结节伴瘙痒4年,加重1年。2个月前于本院行右下肢皮损组织病理示:表皮角化过度,基底细胞层未见异常,真皮浅层小血管及纤维组织增生,血管周淋巴细胞浸润,可见粟丘疹。皮肤科情况:双小腿胫前可见密集黄豆至蚕豆大小的暗红色扁平丘疹、结节,偶见水疱,可见粟丘疹、抓痕及色素沉着;双手背、足背及肘伸侧散在类似皮损。COL7A1基因c. 1241-6G A杂合变异,为剪切位点变异。诊断:痒疹样营养不良型大疱性表皮松解症。经治疗,病情得到控制后出院。现仍在随访中。     

大疱性肥大细胞增生症

报告1例以头皮大疱为主要特点的大疱性肥大细胞增生症.患儿女,11个月.头皮反复起水疱、大疱3个月,每次发作前,面部潮红,头部出现风团样皮损,而后出现水疱、大疱,皮肤划痕征阳性.皮损组织病理和Giemsa染色证实为大疱性肥大细胞增生症.     

金黄色苔藓1例

患者男,17岁。右下肢暗红色斑8个月,于2005年10月就诊于我科。8个月前患者无意中发现右下肢内侧及屈侧出现带状分布的暗红斑,无自觉症状。10余天后皮损颜色逐渐变为紫红色及黄褐色,未予治疗。体格检查:一般状况好,系统检查未见异常。皮肤科检查:右下肢内侧和屈侧可见带状分布、大小不一的黄褐色斑片,压之不褪色,表面轻度苔藓样变,较大的皮损周围可见许多黄褐色斑丘疹(图1A)。皮损组织病理检查:表皮轻度角化,局灶性基底细胞液化变性;真皮乳头层有中等密度的淋巴细胞和组织细胞浸润,其间可见红细胞外渗(图1B),未见含铁血黄素。诊断:金黄色苔…     

右上肢带状寻常狼疮一例

患者，男，18岁。右手背至前臂伸侧带状分布暗红斑、丘疹9个月。皮损组织病理示真皮上部可见较多上皮样细胞、淋巴细胞和多核巨细胞浸润形成结核样肉芽肿。诊断：寻常狼疮。治疗：给予标准四联抗结核 (异烟肼0.3 g/d，利福平0.45 g/d，吡嗪酰胺1.25 g/d，乙胺丁醇0.75 g/d)，6个月后皮损大部分变平、消退。     

新生儿弥漫性皮肤肥大细胞增生症1例

患儿男,10个月。全身皮疹伴瘙痒10月。检查见全身多发苔藓化丘疹,皮肤明显增厚,并见散在血疱及水疱。皮损组织病理示:真皮血管周围可见肥大细胞浸润,胞核圆形或卵圆形,Giemsa染色阳性。诊断:新生儿弥漫性皮肤肥大细胞增生症。     

Relationship between clinical findings of patients with bullous pemphigoid and antigens recognized by their circulating antibasement membrane zone antibodies

INTRODUCTION: Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum. PATIENTS AND METHODS: One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts. RESULTS: Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence. CONCLUSION: Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.     

复发性皮肤坏死性嗜酸性血管炎1例并文献复习

报道1例复发性皮肤坏死性嗜酸性血管炎并对相关文献进行复习。患者，女，64岁，因双下肢痛性皮疹12天就诊。偶有干咳，无哮喘病史。皮肤科查体：双下肢散在紫红色斑片、斑块，胫前数个结节、血疱样损害；右前臂有少量类似损害。外周血嗜酸性粒细胞计数增多。胸部X线提示双肺渗出性改变。皮损组织病理符合皮肤坏死性嗜酸性血管炎。口服强的松45mg/天治疗1周后临床症状明显好转，激素减量过程中曾有复发。     

Evidence supporting a role for immune complex-mediated inflammation in the pathogenesis of bullous lesions of systemic lupus erythematosus

Evidence supporting an immune complex pathogenesis of bullous lesions in systemic lupus erythematosus includes immune deposits, acute inflammation, and blister formation at the cutaneous basement membrane zone. Since cutaneous immune deposits are a general feature of lupus, an attempt has been made to determine whether deposits in lupus patients with blisters are functionally different from those in patients without blisters. Skin was obtained from 4 consecutive patients with blisters and 14 controls. The groups were matched for clinical and serologic features, duration and activity of disease, and treatment. Skin was examined by direct immunofluorescence for immune deposits and by the leukocyte attachment assay for quantification of complement-activating immune complexes. Clinically normal, viable skin from 1 patient with blisters and 1 patient without blisters was incubated in organ culture with normal human leukocytes and serum complement. All patients in both groups had immune deposits at the basement membrane zone with an equivalent incidence of the major Ig classes. Deposits in patients with blisters were slightly more intense and a linear pattern of fluorescence seen in 75% of these patients was not seen in controls. The leukocyte attachment assay showed significantly greater (p less than .02) cell attachment in patients with blisters (mean = 167) than in patients without blisters (mean = 64) and greater cell attachment in peribullous than normal skin from the same patient. Organ culture showed complement-dependent migration of leukocytes and histologic features similar to those in spontaneous lesions in skin from the patient with blisters but not in skin from the patient without blisters. These results provide evidence supporting immune complex and complement-dependent inflammation in the pathogenesis of bullous lesions in systemic lupus erythematosus.     

Coma blisters with hypoxemic respiratory failure

A 24-year-old woman with quadriplegia was admitted with respiratory failure because of pneumonia. She was on multiple medications including diazepam, oxycodone, and amitriptyline, known to be associated with coma blisters, though she did not overdose on any of them. On hospital day 2, she developed multiple blisters on both sides of her right forearm and hand. Skin biopsy showed eccrine gland degeneration consistent with coma blisters. It was felt that hypoxemia from her pneumonia contributed to the development of these blisters, which occurred on both pressure and non-pressure bearing areas of the arm. Coma blisters are self-limited skin lesions that occur at sites of maximal pressure, mostly in the setting of drug overdose. However, coma blisters may occur with metabolic and neurological conditions resulting in coma.     

IgA-lineare Dermatose im Erwachsenenalter mit klinischen Zeichen eines Stevens-Johnson-Syndroms

Immunohistologically linear IgA disease presents with unambiguous features, whereas clinical manifestations are variable. It sometimes shows similarity to other bullous dermatoses such as bullous pemphigoid and dermatitis herpetiformis. A 73 year old female patient was referred with the diagnosis of bullous pemphigoid. One day after admission clinical examination revealed the classical features of Stevens-Johnson syndrome (SJS): widespread confluent atypical target lesions, partly raised, partly flat with central blisters, and erythematous spots, but few typical targets, as well as blisters and large areas of skin detachment on her back and buttocks, accompanied by erosions of the oral and genital mucosa. Direct immunofluorescence performed on peri-lesional skin showed linear deposition of IgA along the basement membrane zone, leading to the diagnosis of linear IgA disease of adults. Our case report shows that linear IgA disease may present with the clinical pattern of SJS.     

线状IgA大疱性皮病15例分析

目的分析线状IgA大疱性皮病的临床特点、治疗及预后,提高临床医生对本病的认识。方法回顾性分析本科2004年1月-2011年12月诊治的15例线状IgA大疱性皮病的临床资料。结果男6例,女9例;儿童型5例,成人型10例;皮损初始部位:面部4例,口腔黏膜及双下肢各3例;累及躯干和四肢12例,14例临床表现为红斑或正常皮肤上出现水疱,6例水疱呈环形或半环形排列。皮损组织病理均见表皮及复层鳞状上皮下水疱或裂隙,直接免疫荧光均表现为基底膜带线状IgA沉积。13例予糖皮质激素治疗,皮损均控制;2例予氨苯砜治疗,均发生不良反应。结论线状IgA大疱性皮病的皮损以口腔黏膜、面部及下肢首发居多,好发于躯干和四肢,临床表现为红斑或正常皮肤上出现水疱,呈孤立、环形和/或半环形排列;组织病理可见表皮或复层鳞状上皮下水疱或裂隙,直接免疫荧光示基底膜带线状IgA沉积是诊断的主要依据。治疗上可选用氨苯砜、糖皮质激素和磺胺类药物等。     

大疱性系统性红斑狼疮五例分析

目的 探讨大疱性系统性红斑狼疮的临床及病理特征,提高对该病的认识.方法 对5例大疱性系统性红斑狼疮患者皮损进行组织病理检查,并用鼠抗人免疫球蛋白(IgG,IgM,IgA)及补体C3进行直接免疫荧光检查,对临床、组织病理特点及治疗预后情况进行分析.结果 皮损好发于面部、上肢等曝光部位,临床表现为正常皮肤上出现水疱或者大疱,5例患者病理的典型改变为表皮下水疱,水疱内及真皮乳头可见较多中性粒细胞浸润.5例患者免疫病理均显示免疫球蛋白和(或)补体在基底膜带呈带状沉积.患者对氨苯砜治疗反应较好,糖皮质激素联合免疫抑制有效.结论 大疱性系统性红斑狼疮可能与狼疮肾炎活动有关.氨苯砜联合糖皮质激素或者糖皮质激素联合免疫抑制剂治疗有效.     

Influence of blistering lesions on foot functionality in hikers

BackgroundFriction blisters are formed by abrasion from frictional forces on the upper layer of the epidermis and can make physical activity an uncomfortable experience. To our knowledge, no previous studies have considered how these injuries affect the functionality of the foot. For this reason, the main aim of this study was to evaluate foot function in hikers, with or without blisters.Material and methodsThis case-control study examined 298 hikers who walked the Camino de Santiago long-distance trail (in northern Spain); 207 had one or more blistering foot lesions and 91 had no blisters. Sociodemographic and clinical variables were collected, and the number of blisters and their locations on the foot were recorded. All participants self-completed the Foot Function Index (FFI) questionnaire, in their native language.ResultsPain and disability were significantly greater among the hikers with blisters (pain p=<0.001; disability p = 0.015). However, there were no significant differences in the limitation of physical activity between those with blisters (case group) and the control group (p = 0.144). Neither was there any correlation between the number of blisters and pain, disability or limitation of activity. However, the location of the lesion did influence foot functionality. Blisters on the metatarsal heads were more limiting and caused greater pain (right foot p = 0.009; left foot p = 0.017), greater disability (right foot p = 0.005; left foot p = 0.005), greater limitation of activity (on right foot p = 0.012) and more loss of foot functionality (right foot p = 0.002; left foot p = 0.007).ConclusionThe hikers with blisters experienced reduced foot functionality in terms of pain and disability. The number of blisters was not related to foot functionality. Blisters located on the metatarsal heads caused the greatest increase in pain, disability and limitation of activity.     

增殖型类天疱疮一例

【摘要】 患者女，89岁，因腹股沟、外阴、臀间沟皮疹10个月、水疱3周就诊。皮肤科检查：腹股沟、外阴、臀间沟红白色增生性斑块，斑块部位散在绿豆至豌豆大小的糜烂面及水疱，右腋下及右小腿正常皮肤上散在数个类似水疱，部分破溃结痂。臀间沟皮损组织病理检查：棘层增生肥厚，无棘刺松解，部分区域可见表皮下裂隙和水疱，局灶性真皮浅层水肿，伴嗜酸性粒细胞浸润，真皮浅层血管周围可见淋巴细胞为主的浸润。直接免疫荧光：IgG、C3基底膜带线状沉积，IgM真皮簇状小体阳性，IgA阴性。盐裂皮肤间接免疫荧光：IgG、C3沉积在表皮侧。酶联免疫吸附试验检测血清抗体：BP180抗体26.92 U/ml、BP230抗体68.17 U/ml，桥粒芯蛋白1抗体、桥粒芯蛋白3抗体正常。诊断：增殖型类天疱疮。予口服甲泼尼龙，联合外用卤米松软膏及0.03%他克莫司软膏，皮疹逐渐消退。     

Incontinentia pigmenti bei einem fünf Wochen alten Mädchen

Shortly after birth, a five-week-old female infant developed small blisters and erythema that followed the lines of Blaschko on the upper and lower extremities as well as the abdomen. Histological examination confirmed the clinical presumptive diagnosis of incontinentia pigmenti. We discuss the dinical features, diagnosis, and the molecular genetic basis of this rare inherited skin disorder.