Erythrodermic bullous pemphigoid

We report a case of erythrodermic type of bullous pemphigoid which is a rare variant of bullous pemphigoid. Our patient had a peculiar clinical presentation with bullae, erosions and extensive erythrodermic areas, and distinct direct immunofluorescent findings which included linear IgG and C3 deposits in the basement membrane and also IgG in the intercellular spaces. Very high levels of serum IgE were also detected in our patient.     

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.     

Childhood bullous pemphigoid following hepatitis B immunization

Bullous pemphigoid (BP) is an acquired autoimmune bullous disorder which predominantly affects the elderly. It is very rare in children. There are approximately 50 reported cases of childhood BP. Although the cause of childhood BP is unknown, drug intake and vaccination have been incriminated in some cases. A total of 13 patients with BP (10 adults and 3 infants) have been described to be related to various vaccines and tetanus toxoid booster. However, no case related to hepatitis B vaccination has previously been reported. Our case of childhood BP developed one week after hepatitis B immunization in a Turkish caucasian child. This case suggests that the hepatitis B surface antigen can function as the triggering factor for BP by inducing a nonspecific immune reactivation which unmasks subclinical BP or by stimulating a specific antibody production that may cross-react with BP antigens.     

药物相关性大疱性类天疱疮研究进展

药物相关性大疱性类天疱疮(drug-associated bullous pemphigoid, DABP)特指应用特定药物诱发的一种特殊类型的类天疱疮,其临床表现、组织学或免疫病理特征与特发性大疱性类天疱疮相同或相近。近年由药物导致大疱性类天疱疮的报道增多,本文就DABP的研究进展进行综述。     

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.     

Treatment of bullous pemphigoid

Bullous pemphigoid (BP) is the most frequent auto-immune blistering skin disease. Up to recently, it was treated with oral corticosteroids. High dose steroids are poorly tolerated in the elderly and probably contributed to the high mortality rates observed in several cohorts. For years, efforts have been devoted to looking for steroid sparing agents including immunosuppressive drugs, plasma exchanges, intravenous immunoglobulins, tetracycline. Many seemed useful in open series but proved ineffective or marginally effective when tested in randomized controlled trials. An important breakthrough was the demonstration by a large randomized trial that a "super-potent" topical corticosteroid (clobetasol propionate) was not only associated with a significant decrease in severe complications and mortality of BP patients but was also more effective than oral prednisone. New strategies for BP should include topical clobetasol propionate as the first line treatment and consider adjuvant therapy only in the very rare cases that are either resistant to or intolerant of this treatment.     

A child with localized vulval pemphigoid and IgG autoantibodies targeting the C-terminus of collagen XVII/BP180

Localized vulval pemphigoid of childhood (LVPC) has previously been reported in six girls. Clinical features and immunopathological data have suggested it to be a morphological variant of bullous pemphigoid. Epitope targets of the autoantibodies of these patients have not been defined in detail. We describe a 9-year-old girl with possible cicatricial LVPC and circulating IgG antibodies directed against native collagen XVII/BP180, its 120-kDa soluble ectodomain and against the C-terminus of collagen XVII/BP180. No reactivity was detected towards the NC16A domain of collagen XVII/BP180. Linear IgG and C3 deposits were found along the cutaneous basement membrane zone. On 1 mol/L salt-split skin, IgG autoantibodies were shown to bind to the epidermis, and the HLA type II allele DQB1*0301, a marker with significantly increased occurrence in patients with ocular and oral cicatricial pemphigoid, was identified in this patient. Our data suggest that LVPC is a variant of bullous pemphigoid in which direct immunofluorescence microscopy combined with immunoblot analysis can deliver valuable diagnostic information for differential diagnosis. However, differentiation between the scarring and non-scarring course of the disease cannot be made with the present diagnostic markers and therefore careful follow-up of patients with LVPC is required.     

幼儿大疱性类天疱疮1例

报道1例儿童大疱性类天疱疮。患儿男．1岁8个月。全身泛发大疱生皮损，疱壁紧张，不易破裂，疱液清澈，尼氏征阴性。皮肤组织病理检查见表皮下水疱，真皮层见以嗜酸粒细胞为主的浸润。皮肤免疫病理检查见表皮基膜带有IgM及C3沉积，患儿经皮质类固醇治疗，疗效满意。     

Bullous pemphigoid in an infant

An acral blistering eruption in a 10 week old baby was found on histology and direct immunofluorescence to be bullous pemphigoid. Circulating auto-antibodies were not detected. He responded quickly to oral prednisolone and there have been no sequelae.     

Bullous pemphigoid of childhood: immunofluorescent investigation

A four-month-old Japanese girl with bullous pemphigoid of childhood (BPC) was reported. The characteristics of BPC antigen were studied by immunofluorescent technique using the serum of this patient with variously treated normal human skin as substrate. This study showed that her BPC antigen was quite similar to bullous pemphigoid antigen seen in adult patients and that this BPC antigen was differed from epidermolysis bullosa acquisita (EBA) antigen.     

大疱性类天疱疮患者发根毛囊中抗体的检测

目的：通过检测大疱性类天疱疮患者拔出的头发上残留毛囊中大疱性类天疱疮抗体的沉积情况,建立一种快捷、损伤小的检测大疱性类天疱疮的方法。方法：直接免疫荧光方法测定35例大疱性类天疱疮患者拔出的头发上残留毛囊中类天疱疮抗体的沉积,10例健康人及20例天疱疮病人头发作为对照。结果：35例大疱性类天疱疮患者的毛发,其中阳性28例,阴性7例,阳性率80%;类天疱疮IgG和C3在毛囊表皮下的外毛根鞘外层与结缔组织鞘之间呈明显的线状沉积。10例正常人及20例天疱疮患者均无线状沉积。结论：本实验方法简单、快速、敏感,且具有较高特异性,有可能成为诊断和鉴别诊断大疱性类天疱疮的一种有效的方法。     

Dyshidrosiform bullous pemphigoid: trigger factors

This report presents 2 patients with a rare form of bullous pemphigoid on (heir palms and soles. To date, at least 16 cases of dyshidrosiform pemphigoid have been reported. The first case was accompanied by a bacterial fool infection, the second by contact dermatitis. The possibility that dyshidrosiform bullous pemphigoid was induced by these trigger factors is discussed.     

Trichophytic granuloma associated with bullous pemphigoid

A 69-year-old Japanese man developed multiple nodular granulomatous cutaneous lesions caused by Trichophyton rubrum during treatment of bullous pemphigoid. One and a half months after the initiation of oral corticosteroid therapy, the lesions appeared. He had suffered from tinea pedis for several decades. Oral ketoconazole was given for 6 weeks with remarkable improvement. Trichophytic granuloma is a rare but important complication of bullous pemphigoid.     

Childhood bullous pemphigoid associated with IgA antibodies against BP180 or BP230 antigens

Linear IgA bullous dermatosis (LABD) comprises a heterogeneous group of subepidermal blistering disorders characterized by in situ bound IgA antibodies in epidermal basement membrane. We report three children presenting clinical and immunopathological features characteristic of LABD. By immunoblotting, the three patients' sera contained IgA antibodies that reacted against the bullous pemphigoid (BP) antigen 180 and or BP230, molecular markers for BP. In addition, IgG antibodies directed against the ectodomain of BP180 were detected by an enzyme-linked immunosorbent assay using a eukaryotic recombinant form of BP180. Consistent with recent studies suggesting that the LABD antigen 1, the predominant autoantigen of LABD, is either a proteolytic product of BP180 or an isoform of the BP180 gene, our findings indicate that a subset of children with features of LABD have a distinct form of BP associated with an IgA response.     

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.     

A population-based study of acute medical conditions associated with bullous pemphigoid

Background  Bullous pemphigoid is associated with poorly understood dramatically increased early mortality rates.
Objectives  To assess the incidence of acute events predisposing to early mortality.
Methods  Computerized medical records from the Health Improvement Network, a large population-based U.K. general practice database, were used to conduct a cohort analysis. Outcome measures were incidence rates of myocardial infarction, pulmonary embolism, pneumonia and sepsis compared with a matched control population.
Results  People with bullous pemphigoid were three times as likely to develop pneumonia, adjusted rate ratio 2·94 [95% confidence interval (CI) 2·01–4·31] or pulmonary embolism, adjusted rate ratio 3·12 (95% CI 1·37–7·12) compared with matched controls. No statistically significant increase was seen for myocardial infarction, adjusted rate ratio 1·24 (95% CI 0·66–2·33), or sepsis, adjusted rate ratio 2·02 (95% CI 0·78–5·21).
Conclusions  The risk of pulmonary embolism and pneumonia is increased following a diagnosis of bullous pemphigoid. It may be possible to reduce associated mortality through considering prophylaxis with either antithromboembolic measures or antibiotic therapy and vaccination.     

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。