Induction of pemphigoid lesions in neonatal mice

In order to study the pathogenetic role of anti-basement membrane zone antibodies (BMZ-Ab) in bullous pemphigoid (BP), parenteral transfer of serum, heat inactivated serum, blister fluid, or IgG from three patients with BP into neonatal mice was carried out. BP-like lesions developed in 6 of 8 mice receiving the serum from one of the patients. However, no lesions were observed in 100 other mice that were given serum, blister fluid or IgG from the other two patients or three healthy controls. Analysis of the characteristics of these injected materials revealed that, for induction of the BP-like lesions in neonatal mice, it is necessary that both complement-fixing BMZ-Ab and components of complement are present. The role of inflammatory cells in the pathogenesis of BP could not be determined by the present experiments.     

A rare presentation of congenital syphilis: Pemphigus syphiliticus in a newborn infant with extensive desquamation of the extremities

Congenital syphilis is an infection transmitted from mother to fetus and can present with early but variable cutaneous manifestations. In rare situations, a bullous eruption known as pemphigus syphiliticus may develop. We present an unusual case of broad desquamation of the extremities in a newborn infant who was found to have congenital syphilis. Pemphigus syphiliticus should be considered in the differential diagnosis of neonatal bullous eruptions and erosions.     

Bullous scabies mimicking bullous pemphigoid

Scabies is an infestation caused by Sarcoptes scabiei and characterised by polymorphous lesions that may include burrows, papules, nodules, excoriation and crusts. Vesicular and bullous lesions are rather rare. Several diseases may be confused with scabies. We report a case of bullous scabies which, on the basis of the clinical and histopathological picture, mimicked bullous pemphigoid. Direct and indirect immunofluorescence were negative. Bullae recurred and persisted despite systemic corticosteroids. The patient was successfully treated with 5% permethrin and remained disease free for up to 12 months of follow-up.     

大疱性类天疱疮230000和160000抗原的区域性分布

利用免疫印迹技术筛选出2份大疱性类天疱疮(BP)血清,一份只和230 000分子结合,一份只和160 000分子结合,分别和人体22处正常皮肤作间接免疫荧光(ⅡF),发现230 000抗原和160 000抗原的表达存在明显的区域性差异.230 000抗原在胸腹和大腿屈侧含量最高,头皮和足跖最低.160 000抗原在胸腹含量最高,头皮和颈部含量最低.230 000抗原和160 000抗原在同一部位的表达基本一致,但在腋窝、大腿伸侧和大腿屈侧三个部位明显高于后者.上述差异与临床上BP的好发部位有一定关联,靶抗原表达的区域性差异是造成自身免疫性皮肤病皮疹特异分布形式的一个重要原因.     

Three cases of bullous morphea: histopathologic findings with implications regarding pathogenesis

Bullous morphea is a rare variant and is not frequently reported. We present three cases of bullous morphea. Although lymphangiectases have been suggested as the most likely mechanism for the development of the bullae in cases of morphea, none of the cases presented with lymphangiectases. To the contrary, all of our cases showed hemorrhagic content in the bullae, which suggests local trauma as a mechanism involved in bulla formation.     

13例线状IgA大疱性皮病临床病理特征分析

收集2012年7月至2019年6月在本科室诊治的13例LABD,其中男11例,女2例.儿童型6例,成人7例.临床表现:红斑基础上或外观正常的皮肤上出现水疱和大疱,疱壁紧张,疱液清亮,尼氏征(-).皮损组织病理均见表皮下疱,直接免疫荧光见IgA线状沉积于基底膜带.1例患者合并类风湿关节炎,1例患者合并器质性焦虑障碍,1例...     

Bullous Pemphigoid Associated with Shy-Drager Syndrome

We report a patient with Shy-Drager syndrome who developed multiple tense blisters mainly on the extremities. Circulating anti-basement membrane zone autoantibodies were detected by the indirect immunofluorescence method. Immunoblot analysis using normal human epidermal extracts demonstrated that this patient's serum reacted only with 230 kD bullous pemphigoid antigen (BPAG1). Concerning the pathoetiology of the association of bullous pemphigoid and Shy-Drager syndrome, we discuss a sequence similarity between BPAG1 and dystonin, a candidate gene for dystonia musculorum.     

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.     

A unique case of subepidermal bullous disease

A 72-year-old Japanese woman developed small, asymptomatic, subepidermal vesicles on the limbs, back, chest and abdomen. Immunoperoxidase staining of the lesional skin showed linear deposition of IgG, IgA and C₃ along the basement membrane zone (BMZ), and indirect immunofluorescence studies revealed IgG and IgA class circulating anti-BMZ auto-antibodies in the patient's serum. Ultrastructurally, the vesicles were caused by dermo-epidermal separation at the lamina lucida, and the immune deposits were located just beneath the basal lamina in a band-like pattern. Immunoelectron microscopic observation of normal human skin incubated with the patient's serum using an organ culture system revealed that the anti-BMZ antibodies reacted with the anchoring fibrils. Administration of dapson was not effective, but betamethasone was. This case of subepidermal bullous disease is unique and cannot be classified into any existing category of bullous dermatoses.     

Bullous systemic lupus erythematosus in a 6‐year‐old boy

Bullous systemic lupus erythematosus (BSLE) is a rare subepidermal blistering disorder characterized by an acute vesiculobullous eruption in a subset of individuals with systemic lupus erythematosus. BSLE most commonly affects young women and only rarely affects children. Herein we report a rare case of BSLE in a 6‐year‐old boy.     

Detection of Autoantibody against 97-kD Antigen by Immunoblot,but not by Indirect Immunofluorescence,in a Patient with Linear IgA Bullous Dermatosis

A case of linear IgA bullous dermatosis in an 85-year-old man is reported. Direct immunofluorescence (IF) of the lesional skin showed linear deposition of IgA and weak deposition of IgG at the basement membrane zone. Although no circulating autoantibody was detected by indirect IF, immunoblotting analysis using NaCl-separated normal human epidermal extracts revealed a circulating IgA antibody which bound to the 97-kD antigen.     

水疱型毛母质瘤1例

患者,男,12岁。右侧颈部及右上臂囊性肿物2年。皮损组织病理示:表皮正常,真皮内见镜影细胞及嗜碱性粒细胞浸润,可见多处钙化区,其周围被纤维组织包绕。诊断:水疱型毛母质瘤。     

大疱性类天疱疮89例临床分析

目的 探讨大疱性类天疱疮患者的临床表现、实验室检查和治疗的特点。方法 回顾性分析89例大疱性类天疱疮患者的临床资料。结果 89例患者,男女之比1.07:1,平均发病年龄58岁。皮损除典型的水疱、红斑外,还有多形红斑、疱疹样皮炎样损害。33.7%的患者有口腔粘膜损害,6.7%的患者以口腔水疱、糜烂为首发症状。18%的患者尼氏征阳性。间接免疫荧光法检测阳性率74.4%,直接免疫荧光法检测阳性率94.9%.皮质类固醇以及皮质类固醇联合免疫抑制剂是治疗大疱性类天疱疮的主要手段,除接受冲击治疗的患者外,控制皮损所需的皮质类固醇剂量平均值为65.5mg(相当于泼尼松).结论 组织病理和免疫荧光检测是确诊的主要依据,控制皮损的皮质类固醇最大用量存在个体差异。     

Plectin, an unusual target antigen in bullous pemphigoid

BACKGROUND: Bullous pemphigoid (BP) is a blistering disease associated with autoantibodies directed against two components of hemidesmosomes, BP180 and BP230. OBJECTIVES: To assess whether BP patients have autoantibodies targeting plectin, another hemidesmosomal component showing extensive homology to BP230. METHODS: Examination of sera from 16 patients with BP, using immunoprecipitation studies followed by immunoblotting. RESULTS: Serum of one of the 16 (6%) patients with BP contain autoantibodies binding to plectin, while no reactivity was found with sera from three control subjects. Sera from all 16 BP patients immunoprecipitated BP230 from extracts of biosynthetically radiolabelled human keratinocytes. CONCLUSIONS: Our results indicate that sera from BP patients might contain autoantibodies binding to plectin. Although this protein and BP230 are closely sequence-related, the occurrence of autoantibodies binding to plectin is a rare phenomenon in BP.     

IgA autoantibody may be the foremost pathogenic in three cases of linear IgA/IgG bullous dermatosis

Linear IgA/IgG bullous dermatosis (LAGBD) is a relatively rare autoimmune bullous disease characterized by both IgA and IgG antibodies to basement membrane zone. The heterogeneity and pathogenesis of antibodies and the relationship between IgA and IgG in LAGBD have not been fully elucidated. We observed clinical, histological and immunological features of three LAGBD cases at different time points in the disease course. In our cohort, two cases showed IgA antibodies to epidermal antigens vanished when their lesions cleared after 3 months of treatment. One refractory case showed increasing antigens targeted by IgA antibodies with the progression of the disease. Collectively, the results suggest that IgA antibodies may play a major role in LAGBD. In addition, epitope spreading may be related to disease relapse and treatment refractory.     

大疱性疥疮

报告1例大疱性疥疮。患者男,65岁。3个月前无明显诱因躯干、四肢出现散在红斑、水疱、糜烂、结痂,伴瘙痒。患者1个月前指缝间、阴囊处出现类似红斑、丘疹,瘙痒明显。皮肤科检查:躯干、四肢可见弥漫性红斑,部分红斑上可见紧张性水疱,疱壁紧张,疱液清亮,部分水疱破裂后糜烂,可见红色糜烂面,部分表面结痂;双手指缝间、阴囊散在红斑,苔藓样变,可见抓痕。皮损组织病理检查:(左股内侧皮损)角化不全,浆液渗出,可见表皮下疱,疱内可见浆液,大量嗜酸性粒细胞及红细胞,真皮浅层可见嗜酸性粒细胞及中性粒细胞浸润;直接免疫荧光:皮肤全层未见特异性荧光,间接免疫荧光:真皮与表皮交界处(-);镜检发现疥螨。诊断:大疱性疥疮。     

Pseudoporphyria secondary to narrowband UVB phototherapy for psoriasis

A 59-year-old woman undergoing narrowband UVB phototherapy for treatment of psoriasis presented suddenly with tense blisters on both hips. The blisters were asymptomatic and disappeared within 24 hours. Histopathology together with a negative porphyrin screen made the diagnosis of pseudoporphyria. Blood tests including a full blood count, urea and electrolytes, liver function tests and antinuclear autoantibodies were normal. The patient was otherwise healthy; there was no history of extensive sun exposure, sunbeds or medication with non-steroidal anti-inflammatory drugs. This presentation of pseudoporphyria secondary to narrowband UVB radiation is unusual, as UVB radiation is not known to be associated with pseudoporphyria.     

大疱性疥疮三例并文献复习

大疱性疥疮（bullous scabies,BS）是疥疮的一种罕见的临床表现,极易误诊。本文报道3例既往误诊的BS患者,并通过万方及PubMed学术数据库检索“大疱性疥疮”文献,检索到44篇文献,包括60例患者,总结分析该病的人口统计学信息、发病机制、临床表现、病理特点及治疗,发现BS好发于老年群体及男性患者,约1/3患者存在误诊史,3/4患者在接受糖皮质激素治疗后病情未见缓解或进一步进展。皮肤科医生应提高对该病的认识,并且治疗上应谨慎使用糖皮质激素。     

Case of localized bullous pemphigoid with unique clinical manifestations in the lower legs

We report the case of a 71-year-old Japanese woman who presented with persistent band-like erosions in the lower legs. Histological examination suggested subepidermal blister formation. Direct and indirect immunofluorescence studies revealed tissue-deposited and circulating immunoglobulin G autoantibodies against the basement membrane. Western blotting revealed autoantibodies to BP230, but not to BP180. Based on these findings, the patient was diagnosed as having a localized type of bullous pemphigoid. We suggest that the unique clinical manifestations in this patient could be attributable to bacterial or fungal infection, and/or mechanical trauma, such as the pressure of her socks.