儿童大疱性类天疱疮

大疱性类天疱疮是一获得性自身免疫性疾病.常累及老年人,儿童少见.儿童大疱性类天疱疮的发病率无明显的人种、性别区别,年龄跨度较大,儿童期各年龄组均有报道.儿童大疱性类天疱疮的诊断主要依据临床表现、组织病理及直接免疫荧光检查.在病因及临床表现上儿童有别于成人,但在组织病理及直接免疫荧光上与成人大疱性类天疱疮相似.     

非大疱性类天疱疮

【摘要】 非大疱性类天疱疮与大疱性类天疱疮相关,临床表现多样,多数伴有瘙痒,缺乏大疱性类天疱疮的紧张性水疱或大疱的典型临床表现,误诊率高。组织病理缺乏特异性,需要依靠直接免疫荧光、间接免疫荧光或盐裂间接免疫荧光明确诊断。部分无疱性类天疱疮会发展为大疱性类天疱疮,预后较大疱性类天疱疮好,但由于易被延迟诊断,使得控制症状的药物用量大,药物不良反应多。     

结节性类天疱疮

结节性类天疱疮是大疱性类天疱疮的罕见型,多见于老年患者,主要临床表现是结节、水疱,病理、直接免疫荧光、间接免疫荧光显示大疱性类天疱疮特征。治疗上需系统应用皮质类固醇激素,有时需合并使用免疫抑制剂。免疫印迹试验发现多数PN患者血清与230kD的表皮抗原相结合。     

儿童大疱性类天疱疮2例

报告2例儿童大疱性类天疱疮,都是以面部先发皮疹,但临床表现迥异,1例表现类似脓疱疮,另一例酷似儿童线性IgA大疱皮病。两例患者均经组织病理和免疫荧光确诊,对皮质类固醇激素治疗敏感,停药后分别随访1年和3个月无复发。儿童大疱性类天疱疮临床表现不典型,但预后较成人好。     

80例大疱性皮肤病组织病理及直接免疫荧光结果分析

目的：探讨组织病理及直接免疫荧光检查对大疱性皮肤病的诊断意义。方法：对80例大疱性皮肤病患者的皮损进行组织病理检查,并采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,对结果进行回顾性分析。结果：本组患者自身免疫性大疱性皮肤病占60.00%,以天疱疮和类天疱疮为主,非自身免疫性大疱性皮肤病以大疱性表皮松解症及大疱性多形红斑多见,不同类型的大疱性皮肤病组织病理及免疫荧光具有特征性。结论：组织病理及直接免疫荧光检查对大疱性皮肤病的诊断、鉴别诊断、治疗、预后判断具有重要的意义。     

儿童大疱性类天疱疮1例

报告1例儿童大疱性类天疱疮,患儿女,8岁,2月前躯干、四肢皮肤出现红斑、水疱、大疱,尼氏征阴性。皮损组织病理检查示:表皮下水疱,疱腔内有嗜酸性粒细胞、中性粒细胞浸润;直接免疫荧光示:IgG、C3线状沉积于基底膜带。诊断为儿童大疱性类天疱疮,静注甲强龙治疗后效果良好,随访至今未复发。     

婴儿大疱性类天疱疮1例

婴儿男,6月大时出现全身红斑、水疱和大疱,皮损组织病理可见真皮内嗜酸性细胞浸润,直接及间接免疫荧光显示:IgG基底膜带线状沉积。诊断:婴儿大疱性类天疱疮。糖皮质激素治疗有效。1岁以内婴儿大疱性类天疱疮国内报道少见。     

扁平苔藓类天疱疮1例

报告1例扁平苔藓类天疱疮.患者男,36岁.因四肢紫红色扁平丘疹2个月,起水疱半个月就诊.皮肤科检查:四肢广泛分布紫红色扁平丘疹,上覆少许鳞屑;在红斑基础上及正常皮肤上散在分布米粒至黄豆粒大水疱,疱壁紧张,疱液清,尼氏征(-).皮损组织病理检查:兼具扁平苔藓及类天疱疮组织学改变.直接免疫荧光示表皮基膜C3强阳性;IgM、IgA、IgG弱阳性呈线状沉积.结合临床和组织病理检查符合扁平苔藓类天疱疮诊断.该病应与大疱性扁平苔藓及大疱性类天疱疮等鉴别.     

婴儿大疱性类天疱疮

报告1例婴儿大疱性类天疱疮。患儿女,6个月。皮肤科检查:躯干及四肢多发红斑、水疱和大疱。皮损组织病理检查:真皮浅层血管及胶原间大量嗜酸性粒细胞浸润,直接及间接免疫荧光均可见基膜带免疫球蛋白(Ig)G带状沉积,疱病自身抗体提示抗大疱性类天疱疮(BP)180抗体明显升高。诊断:婴儿大疱性类天疱疮,予糖皮质激素治疗有效。     

儿童大疱性类天疱疮

报告1例儿童大疱性类天疱疮.患儿男,18个月.2个月前躯干和双下肢皮肤出现紧张性水疱、大疱,尼氏征阴性,外用糖皮质激素治疗效果不佳.皮损组织病理检查示表皮下水疱;卣接免疫荧光示:lgG、C3沉积于基膜,间接免疫荧光示抗表皮基膜抗体阳性.诊断为儿童大疱性类天疱疮,予以口服泼尼松治疗后痊愈.     

Childhood bullous pemphigoid: a clinicopathologic study and review of the literature

Bullous pemphigoid (BP) is an acquired bullous disorder that predominantly affects the elderly. It is rare in children but when it occurs, there is considerable clinical and histologic overlap with other acquired or congenital blistering disorders. A definitive diagnosis of childhood BP requires direct immunofluorescence and, in some cases, characterization of the target antigen. Three cases of childhood BP are presented, with their histologic and immunofluorescence findings. The first was a 5-month-old male infant who presented with erythema and bullae of the palms and soles and was found to have linear deposition of IgG and C3 along the dermoepidermal junction on direct immunofluorescence (DIF). Histopathologic examination revealed a subepidermal blister containing eosinophils. Type IV collagen was demonstrated along the floor of the blister cavity by a direct immunoperoxidase technique. The second case was an 8-month-old female infant who presented with a blistering eruption of her palms and soles that then became widespread. Direct immunofluorescence showed linear IgG and C3 at the dermoepidermal junction, with laminin deposition at the base of the blister. The third case was a 7-year-old female with bullae and erosions on the vulva and vaginal mucosa. A subepidermal blister was seen on microscopic examination whereas immunofluorescence demonstrated linear IgG and C3 deposition at the basement membrane zone (BMZ). A literature review uncovered 50 cases of childhood BP confirmed by direct or indirect immunofluorescence, or both, and often with evidence of autoantibodies against either the 180 kD or the 230 kD human bullous pemphigoid antigens (BP180 or BP230). This review was used to delineate characteristics of childhood BP, including the newly proposed subtypes: infantile BP and childhood localized vulval BP. Infantile BP presents within the first year of life and is characterized by BP-like lesions on erythematous or normal acral skin. Localized vulval BP is a self-limited, nonscarring BP-like process that involves only the vulva. Both subtypes are normally self-limited and respond well to either topical or systemic steroids, if treatment is initiated before the disease becomes widespread.     

Childhood bullous pemphigoid – report of a case with dermal fluorescence on salt-split skin

Summary Bullous pemphigoid (BP) is an acquired bullous disorder which predominantly affects the elderly. It is rare in children, and may be clinically indistinguishable from other immunobullous disorders. As routine histology may be non-specific, a definitive diagnosis of childhood BP usually depends on the results of direct and indirect immunofluorescence investigations.
We report a 5-year-old girl who developed bullous pemphigoid, associated with atypical immunofluorescence findings. Indirect immunofluorescence on split-skin showed a pure dermal pattern of IgG binding. This is usually suggestive of epidennolysis bullosa acquisit, but Western immunoblotting was positive with epidermal extracts, confirming a diagnosis of BP. Dermal binding on split-skin occurs in about 5% of adult cases of BP, and has not been reported previously in childhood BP.     

Childhood bullous pemphigoid: report of three cases

Bullous pemphigoid (BP) is a disorder that rarely occurs in children. We hereby describe three cases of childhood BP aged 2-4 months, which are among the youngest reported in the literature. BP was confirmed by histopathology, direct and indirect immunofluorescence with salt-split skin test and immunoblotting. These patients were successfully treated with systemic corticosteroids with a complete clinical remission.     

Bullous Pemphigoid and Epidermolysis Bullosa Acquisita: Presentation,Prognosis, and Immunopathology in 11 Children

Abstract: The immunobullous diseases bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) are very rare in childhood. Although case studies have been detailed, there are no reports of a large series of patients documenting the effectiveness of treatment and long-term prognosis. We report the clinical presentation, immunopathologic features, disease course, and long-term prognosis of BP and EBA in a series of 11 children. The initial diagnoses based on clinical features were BP (5), EBA (3), and chronic bullous disease of childhood (CBDC) (3). These were subsequently revised from BP to EBA (2), CBDC to BP (2), and CBDC to BP or EBA (1) following the results of direct and indirect immunofluorescence and immunoblotting. Analysis of IgG subclasses in eight cases showed that the predominant subclasses were lgG1 (8) and lgG4 (6). The clinical features appeared to be highly variable, and in patients presenting with inflammatory blistering, laboratory studies were required in order to differentiate between BP and EBA. All patients improved on treatment with corticosteroids and/or sulfones, although treatment regimens showed wide variation. Their diseases tended to remit within 2 years, and their long-term prognosis was good.     

Anti-BP180 autoantibodies as a marker of poor prognosis in bullous pemphigoid: a cohort analysis of 94 elderly patients

The prognosis of bullous pemphigoid (BP), a disorder which usually affects elderly patients, is not well established and conflicting data have been reported about the mortality rate of the disease. Our objective in this study was to assess the clinical and immunological factors determining survival in a prospective series of 94 patients with BP. A cohort of 94 consecutive patients with BP (mean age ±SD: 81 ±4 years) was studied over an 8-year period (1987–94) in one department and patients followed up for at least 1 year. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (i.e. linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting. Our analysis (median duration of follow-up: 5 years) showed that 37% of BP patients were dead within a year of starting treatment. The clinical or immunological factors which may influence the prognosis of BP were studied according to the criterion of death or survival by the end of the first year of treatment. None of the following factors was found to be significantly linked to the prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, or the presence of circulating basement membrane zone autoantibodies by indirect IF. An impaired general condition and a history of coronary artery disease indicated a bad prognosis. The presence of circulating autoantibodies against BP180 autoantigen but not autoantibodies against BP230, as detected by immunobiotting on epidermal extracts, was found to be significantly more frequent (60% vs. 25%) in BP patients who died within the first year of treatment (P < 0·01). We conclude that the presence of circulating autoantibodies against BP180 represents the first intrinsic prognostic factor that has been demonstrated in BP. This result supports the growing body of evidence for the pathophysiological importance of the anti-BP180 autoantibodies.     

Unusual subepidermal bullous diseases with immunologic features of bullous pemphigoid.

Sixty-seven patients with histologic and immunologic features of bullous pemphigold (BP) were evaluated. Eleven patients had a localized blistering disease that was predominantly confined to one area of the body, most commonly the lower extremities. Two patients displayed a dapsone-responsive blistering disease that was characterized by a flexural distribution of ten to 20 1-cm or less, intensely pruitic, subepidermal bullae and linear IgA basement membrane zone deposition. Two patients had a chronic recalcitrant generalized scarring, hyperkeratotic, subepidermal blistering eruption that demonstrated serologic and direct immunofluorescence (IF) findings of BP. One patient displayed grouped small vesicles surmounted on an erythematous base; the clinical diagnosis was dermatitis herpetiformis, but direct IF examination of the biopsy specimen showed features of BP. One patient with epidermolysis bullosa acquisita had serologic and direct IF features suggestive of BP.     

Lichen planus pemphigoides: clinical and immunofluorescent findings in four cases

During the last 5 years we examined four black male patients who had features of both lichen planus (LP) and bullous pemphigoid (BP). The clinical disorder included classical lesions of LP along with bullae that developed subsequently and arose on both lesional and nonlesional skin. Histologic findings correlated well with the type of lesion on which biopsy was done, as did the immunofluorescent findings. All patients had features of both LP and BP by direct immunofluorescence and of BP by indirect immunofluorescence of serum and blister fluid.     

Polymorphic pemphigoid.

We describe 20 patients with a chronic polymorphic eruption; they shared clinical, histopathological, and therapeutic features of both dermatitis herpetiformis and bullous pemphigoid (BP). In 14 of these 20 cases, direct and indirect immunofluorescence studies corresponded to BP. The remaining six patients showed IgA deposits in a linear pattern at the basement membrane zone, and two of these six showed IgA pemphigoid antibodies in their sera as well. No significant clinical and histological differences were detected in the patients, in connection with the immunological findings. Furthermore, one patient's condition, which was studied by repeated immunofluorescence examinations, changed from a linear IgA pattern and a negative indirect test to a linear IgG pattern and a positive reaction for IgG pemphigoid antibodies. We concluded that these cases constitute a polymorphic variant of BP.