中性粒细胞沿基底层呈线状排列的大疱性类天疱疮一例

患者男,71岁。全身反复发生红斑3个月,出现水疱1周,伴剧痒。患者3个月前躯干、四肢陆续出现浮肿性红斑伴瘙痒,一度按湿疹治疗皮疹仍反复发作,时轻时重。就诊前1周在正常皮肤或红斑基础上发生水疱,破溃后易结痂。患脑卒中8年。家族中无类似皮肤病史。体检：右侧肢体偏瘫。余各系统检查未见异常。皮肤科检查：躯干、四肢散在大小不一的浮肿性红斑,部分红斑上可见水疱、大疱、糜烂及结痂。水疱Nikolsky征阴性,疱液为透明浆液性（图1）……     

Linear dermo-epidermal IgA deposition in bullous pemphigoid.

A case is reported of a patient with a bullous eruption with in vivo linear deposition of IgA and complement (C3) along the basement membrane zone of perilesional skin (DIF method). Some data presented here are in favour of the concept that cases of linear in vivo deposition of IgA alone, or in combination with other IF findings, might be classified as bullous pemphigoid (BP). Clearing of the lesions due to oral prednisone therapy was accompanied by disappearance of complement deposition, while IgA deposition remained unchanged. Some aspects of tissue injury in this case are briefly discussed.     

Erythrodermic bullous pemphigoid is a clinical variant of bullous pemphigoid

Bullous pemphigoid (BP) is an autoimmune blistering disease of the skin. Several variants olBP have been described but until recently the relationship of these variants to generalized BP was unclear. Several studies have shown that pemphigoid nodularis, pemphigoid vegetans, localized BP and vesicular pemphigoid are true variants of BP as the circulating antibodies in these patients recognize the same 230kDa BP antigen as found in patients with generalized BP. Erythrodermic BP is a very unusual variant characterized by an erythroderma along with blister formation. We describe the third known patient to develop erythrodermic BP and characterize the antigenic specificity of the circulating antibodies in both our newly reported patient with erythrodermic BP and in one of the two other previously reported cases of erythrodermic BP. Both patients with erythrodermic BP had circulating IgG antibodies which bound to the epidermal side of salt-split human skin in a pattern identical to two patients with immunopathologically proven generalized BP. Sera from four erythrodermic patients without blisters and from a healthy normal volunteer, as controls, failed to demonstrate detectable circulating IgG autoantibodies. Immunoprecipitation studies revealed that both patients with erythrodermic BP had circulating IgG autoantibodies which recognized, to varying degrees, the same 230 and 180kDa BP antigens as recognized by sera from two patients with immunopathologically proven generalized BP. Sera from four erythrodermic patients without blisters and from a healthy normal volunteer, as controls, failed to recognize any specific polypeptides. These observations demonstrate that erythrodermic BP is a distinct clinical variant of BP.     

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.     

Amoxicillin-induced bullous pemphigoid in a child

The Authors describe the case of a 6-year old female child who presented a bullous dermatitis following the administration of amoxicillin, with clinical, histopathological and immunological features typical of childhood bullous pemphigoid. The peculiar aspects of drug-induced bullous pemphigoid are therefore reviewed, with particular reference to the hypotheses behind the relative induction mechanism.     

Clinical, histological and immunological studies in 50 patients with bullous pemphigoid

Fifty patients with bullous pemphigoid were investigated over periods of up to 7 years. Sequential studies of circulating basement membrane zone (BMZ) antibody titres revealed two groups of patients: 44% had a high initial titre (greater than 1:160) and 56% had a low initial titre (less than 1:160). Subjects with titres greater than 1:160 continued to have high titres, whereas in those with lower titres the BMZ antibodies usually became undetectable after 4 months. The BMZ antibody titres were of no prognostic value. High titres remained high even in patients who were in remission and off treatment, and in these subjects, there was no evidence of a change in the IgG subclass. The initial BMZ IgG antibody titres showed a highly significant correlation with total serum IgE concentrations. A significant correlation also existed between total serum IgE and IgG4 concentrations, although not between the serum IgE and the peripheral blood eosinophil count. No anti-BMZ antibody of IgE class was found, nor specific IgE against inhalants and foods identified, and the significance of the high total serum IgE remains unexplained. The majority of those followed for more than 2 years were able to stop their steroid therapy without further blistering: this confirms that pemphigoid tends to be a self-limiting disease. Nine patients had a recent history of malignant disease, and this is no more than would be expected for this age group of patients: however, 3 patients presented with pemphigoid and figurate erythema and all died of neoplastic disease, suggesting that such patients should be investigated thoroughly for neoplasia.     

红皮病性大疱性类天疱疮1例

 患者女,78岁。因“躯干、四肢红斑、鳞屑伴瘙痒5年,加重3年余”入院,入院后1周大腿及腹部出现紧张性水疱。住院第6天、第9天血清抗BP180抗体均阳性,IgE>2 500 U/mL。皮损组织病理可见表皮下水疱,真皮浅层大量淋巴细胞为主的浸润。直接免疫荧光示:基底膜带IgG和C3线状沉积,IgA阴性。诊断：红皮病性大疱性类天疱疮。给予甲泼尼龙40 mg静脉滴注1天1次,治疗1周,病情好转出院。1年后随访,患者泼尼松片减量至15 mg口服1天1次,躯干四肢可见轻度红斑,无水疱、糜烂和瘙痒     

IgA bullous pemphigoid: a distinct blistering disorder

We report a patient with an eccrine carcinoma who developed localized blistering which clinically resembled pemphigoid, histologically showed subepidermal blistering with features of both dermatitis herpetiformis and bullous pemphigoid, responded to dapsone and exhibited linear IgA deposition on direct immunofluorescence. The nosological position of patients with linear IgA deposition and subepidermal blistering is not clear. A review of the literature reveals that in adults linear IgA deposition may occur in three separate situations: dermatitis herpetiformis, bullous pemphigoid and a third condition of which our case is an example which is best termed IgA bullous pemphigoid. This condition is distinguished from cases of dermatitis herpetiformis with linear IgA by the clinical features and the site of IgA deposition on immunoelectronmicroscopy. It is distinguished from cases of bullous pemphigoid with linear IgA by the absence of circulating IgG antibasement membrane zone antibody, the therapeutic response to dapsone and the frequent occurrence of circulating IgA antibasement membrane zone antibody. IgA bullous pemphigoid has not previously been reported with a carcinoma but the association lends further support to the concept that this eruption represents a variant of pemphigoid.     

Localized bullous pemphigoid: a commonly delayed diagnosis

Localized bullous pemphigoid is a rare clinical variant of bullous pemphigoid. We report three cases of localized bullous pemphigoid in which the diagnosis was delayed because the presentation simulated other local vesicular or bullous diseases. Localized bullous pemphigoid differs from the generalized form by its more benign course. Early diagnosis will help avoid unnecessary and costly treatment for unrelated conditions.     

药物诱发的大疱性类天疱疮一例

患者,女,20岁。全身皮肤红斑水疱伴瘙痒1个月。1个月前患者因口服“氨酚黄那敏颗粒”后全身出现红斑水疱。皮肤专科查体：上唇、颈部、胸背、腋下、外阴及双大腿内侧见红斑,其上多发黄豆至蚕豆大小的水疱、大疱,疱壁紧张,部分水疱破溃,上附淡黄色痂,尼氏征(-),部分区域可见虹膜样改变,口腔黏膜、舌部受累,可见散在糜烂、水疱。实验室检查、组织病理、直接免疫荧光结合盐裂试验,诊断为药物诱发的大疱性类天疱疮。经甲泼尼龙等治疗1个月后痊愈停药。     

Unilateral bullous pemphigoid in a hemiplegic pagtient

A 78-year-old man with a long-standing left-sided hemiplegia presented with bullous pemphigoid which affected his paralysed side only. Although the rash was unilateral, direct immunofluorescence demonstrated IgG antibody at the dermo-epidermal junction on both sides of the body. Indirect immunofluorescence was also positive. A suction-blister test showed increased skin fragility on the affected side.     

Mortality of bullous pemphigoid in Switzerland: a prospective study

Background Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and has been associated with a significant morbidity and an increased mortality rate. Objectives Our aims were: (i) to evaluate the mortality rate of BP in Swiss patients during the first 3 years after the diagnosis of BP, as the primary endpoint; and (ii) to compare this mortality rate with that of the general Swiss population and determine prognostics factors, as secondary endpoints. Methods All new cases of BP diagnosed in Switzerland between 1 January 2001 and 31 December 2002 were collected prospectively by means of a standardized data collection form. After collection of the data, the treating physicians were asked to specify the course and evolution of the disease. In total, 115 patients were included in our study. Results The 1‐, 2‐ and 3‐year probability of death were 20·9%, 28·0% and 38·8%, respectively. The mortality rate of patients with BP was three times higher than that of age‐ and sex‐matched members of the general Swiss population. Besides age, the presence of neurological diseases at diagnosis was associated with increased mortality in multivariate analysis. Conclusion This is the first prospective study analysing the mortality rate of BP in an entire country. The calculated mortalities are in the lower range of those reported in previous European studies. However, in line with the latter, our findings confirm a high case‐fatality rate for BP, with an increased 1‐year mortality rate compared with the expected mortality rate for age‐ and sex‐adjusted general population.     

Subacute prurigo variant of bullous pemphigoid: autoantibodies show the same specificity compared with classic bullous pemphigoid

We describe a 76-year-old white woman with a 6-month history of intensive pruritus and excoriated papules resembling subacute prurigo. Histopathology showed signs of chronic dermatitis, whereas findings by direct and indirect immunofluorescence microscopy were compatible with bullous pemphigoid (BP). The patient's serum contained IgG autoantibodies that recognized epitopes on both BP180 and BP230 by Western blot analysis of epidermal extracts. In addition, we found strong reactivity with recombinant NC16A, an immunodominant region of BP180 targeted in the majority of BP sera, whereas no antibodies against the keratinocyte-derived soluble BP180 ectodomain (LAD-1) or the recombinant intracellular domain of BP180 were detected. The patient's disease responded well to oral methylprednisolone and mycophenolate mofetil. Disease activity correlated with enzyme-linked immunosorbent assay reactivity of antibodies to BP180 but not with titers of antibodies to the dermoepidermal junction as determined by indirect immunofluorescence on salt-split skin. Our findings suggest that the subacute prurigo form of BP is a true variant of BP.     

眼周局限性大疱性类天疱疮一例

患者,女,12岁.眼周反复红斑、水疱4个月,伴瘙痒.皮损组织病理示:表皮下水疱,真皮浅层淋巴细胞、嗜酸粒细胞、中性粒细胞等炎性细胞浸润.直接免疫荧光(DIF):表皮基底膜IgG、C3阳性线状沉积.ELISA检测:血清抗BP230抗体2.6 U/mL,抗BP180抗体阴性.诊断:局限性大疱性类天疱疮.予以甲泼尼龙、氨苯砜...     

Pathogenesis of the blister in cicatricial pemphigoid and in bullous pemphigoid

Summary A comparative ultrastructural study of bullous lesions of four cases of Cicatricial Pemphigoid and three cases of Bullous Pemphigoid showed: a) in Bullous Pemphigoid the blister seems to form in the intermembrane space while the basal lamina is still visible on the floor of the blister, b) in Cicatricial Pemphigoid it is the basal lamina which appears to be directly affected, its disappearance probably being the consequence of a marked edema of the superficial dermis.It is pointed out that controversial ultrastructural information concerning dermo-epidermal bullous diseases can often be ascribed to the speed of blister formation, to the time at which the biopsy is performed and to the different techniques used in the preparation of specimens.

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Zusammenfassung Bei 4 Patienten mit sogenanntem benignem Schleimhaut-Pemphigoid sowie 3 Patienten mit bullösem Pemphigoid wurden Blasen excidiert und ultramikroskopisch untersucht. Die Blasenbildung scheint beim bullösen Pemphigoid im intermembranösen Raum stattzufinden, während die Basallamina am Blasengrund sichtbar bleibt. Beim benignen Schleimhautpemphigoid zeigen sich die wesentlichen Veränderungen in der Basallamina, die infolge eines starken Ödems der oberen Dermis verloren geht.Die Widersprüchlichkeit vieler ultrastruktureller Beobachtungen über den Mechanismus der Blasenbildung ist auf folgende Faktoren zurückzuführen: Die Geschwindigkeit, mit der eine Blase entsteht; der Zeitraum, an dem die Probeexcision durchgeführt wird, sowie die verschiedenen Techniken, die in der Aufbereitung des Excisionsmaterial befolgt werden.