婴儿湿疹合并大疱性类天疱疮1例

1岁以内的婴儿大疱性类天疱疮国外仅有10余例报告,国内未见报告,婴儿湿疹合并大疱性类大疱疮至今国内外均未见报告.文中报告首例婴儿湿疹合并大疱性类天疱疮患者,用丙种球蛋白治疗有效,该例婴儿湿疹合并大疱性类天疱疮的发病可能与疫苗接种有关.     

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。     

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。     

类天疱疮样扁平苔藓

扁平苔藓患者伴有水疱、大疱时常称之为大疱性扁平苔藓、类天疱疮样扁平苔藓或扁平苔藓与大疱性类天疱疮并发。长期以来分类模糊,命名混乱,一直有争议。近来研究发现类天疱疮样扁平苔藓不同于大疱性扁平苔藓和大疱性类天疱疮,可能是一个独立的疾病。     

天疱疮和大疱性类天疱疮共存

作者报告3例天疱疮和大疱性类天疱疮共存的患者。每一病人在血清中有天疱疮和大疱性类天疱疮的抗体,直接免疫荧光检查发现在棘层细胞间隙和基底膜带区有IgG的存在。例1:女性84岁,病期4个月,广泛出现大疱性损害,部分大疱为松弛性,易溃破并向周围发展;另外部分为紧张性和出血性大疱。伴有瘙痒和灼热感。一般临床检查和常规化验无特殊发现。对3个水疱进行了组织学检查,符合天疱疮和大疱性类天疱疮表现。荧光抗体检查在天疱疮和大疱性类天疱疮损害中均有IgG存在。用大量强的松(70毫克/天)治     

IgA型基底膜带自身抗体研究近况

研究发现,瘢痕性类天疱疮、大疱性类天疱疮、获得性大疱表皮松解症、大疱性系统性红斑狼疮及线状IgA大疱病等表皮下大疱病均存在IgA型基底膜带自身抗体。线状IgA大疱病与瘢痕性类天疱疮、大疱性类天疱疮、获得性大疱表皮松解症不仅IgA型基底膜带自身抗体在基底膜带处沉积和结合方式相似,而且IgA型基底膜带自身抗体的靶抗原亦可相互重叠。大疱性类天疱疮及大疱性系统性红斑狼疮IgA型基底膜带自身抗体与疾病严重程度相关。     

大疱性类天疱疮白细胞介素8的测定

目的:探讨IL-8在大疱性类天疱疮发病机制的作用及临床意义.方法:采用ELISA方法检测大疱性类天疱疮患者活动期血清、疱液和缓解期血清中IL-8的水平.结果:大疱性类天疱疮患者活动期血清中IL-8水平明显高于健康对照者血清中的水平,病情缓解后血清中IL-8的水平明显下降,活动期疱液IL-8水平明显高于活动期血清中的水平.结论:IL-8可能参与了大疱性类天疱疮的发病,可作为观察病情活动的指标.     

大疱性疥疮

成人的疥疮可能是容易诊断的,但婴儿和儿童的疥疮常存在诊断的问题。本文报导了一例病孩因为以大疱性损害为主而被认为系患天疱疮或大疱性类天疱疮,实际上却是患的大疱性疥疮。这是一名22个月的男孩,因2个月来泛发瘙痒     

辛酸-硫酸胺法对大疱性类天疱疮疱液IgG的纯化

目的:评价辛酸-硫酸胺法纯化的大疱性类天疱疮疱液中IgG的免疫活性。方法:采用辛酸-硫酸胺法分别从正常人血清和大疱性类天疱疮患者疱液中纯化IgG,采用免疫比浊法和SDS-PAGE测定两者提纯后IgG的浓度和纯度,并用ELISA法鉴定疱液提纯前后的免疫活性。结果:辛酸-硫酸胺法从大疱性类天疱疮患者疱液中提纯IgG的回收率为51.4%,略低于正常人血清,提纯前后的免疫活性不变。结论:辛酸-硫酸胺法从大疱性类天疱疮患者疱液中纯化IgG简便可行,纯度高,不影响免疫活性。     

类天疱疮样扁平苔藓

扁平苔藓患者伴有水疱、大疱时常称之为大疱性扁平苔藓、类天疱疱样扁平苔藓或扁平苔藓与大疱性类天疱疮并发。长期以来分类模糊，命名混乱，一直有争议。近来研究发现天疱疮样扁平苔藓不同大疱性扁平苔藓和大疱性类天疱疮，可能是一个独立的疾病。     

Bullous pemphigoid in infancy-a case report including new immunoblotting observations

A 1-year-old boy developed bullous pemphigoid. Immunoblotting, which has not been previously reported in childhood pemphigoid, showed the serum reacted to a 190 kDa band only. Bullous pemphigoid is rare in infancy and childhood with only 40 cases previously reported. A brief review of the literature is given and treatment of childhood bullous pemphigoid is discussed.     

Bullous pemphigoid and multiple sclerosis: more than a coincidence? Report of three cases

In three patients with long-standing multiple sclerosis, bullous pemphigoid developed. The diagnosis of bullous pemphigoid was based on histologic findings, direct and indirect immunofluorescence, and Western blots showing IgG reacting with the 220 to 240 kD bullous pemphigoid antigen in the serum of three patients. Contrary to previous observations, bullous pemphigoid associated with multiple sclerosis was not different from bullous pemphigoid alone. Three similar cases have been reported previously, so the occurrence of bullous pemphigoid in patients with multiple sclerosis may be more than a coincidence.     

Lichen planus pemphigoides: case report and results of immunofluorescence and immunoelectron microscopic study

A Japanese woman with lichen planus pemphigoides is reported. Immunologic characteristics of lichen planus pemphigoides antigen in the patient were investigated by indirect immunofluorescence and compared with those of bullous pemphigoid antigen or epidermolysis bullosa acquisita antigen. Ultrastructural localization of lichen planus pemphigoides antigen was studied with the use of immunoelectron microscopic techniques. Lichen planus pemphigoides antigen showed localization similar to that of bullous pemphigoid antigen but different from that of epidermolysis bullosa acquisita antigen. The antigenic stability of lichen planus pemphigoides antigen was different from that of bullous pemphigoid antigen or epidermolysis bullosa acquisita antigen. Thus this study demonstrates that lichen planus pemphigoides antigen is different from bullous pemphigoid antigen.     

Localized bullous pemphigoid induced by thermal burn

BACKGROUND: Induced bullous pemphigoid is known to be triggered by drugs and some physical agents. Six cases of bullous pemphigoid induced by thermal burns have been reported since 1991. CASE REPORT: We observed a bullous eruption around thermal burns of the left leg in a 89 year-old woman. Bullous pemphigoid was diagnosed by histological and immunohistochemical findings with a sub-epidermal blister and linear IgG and C3 deposition at the dermal-epidermal junction. Indirect immunofluorescence was negative. The eruption rapidly resolved with topical steroids. DISCUSSION: We describe a bullous pemphigoid induced by a thermal burn. This case is original because the eruption was localized only around the site of the burn and healed with topical steroids. In the literature, there are 6 other cases reported but only one localized. We discuss the hypothesis of asymptomatic bullous pemphigoid exacerbated by presentation of bullous pemphigoid antigen by thermal burns or self immunization against antigens altered by burns.     

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.     

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.     

Delayed onset of bullous pemphigoid after PUVA and sunlight treatment of psoriasis

The coexistence of bullous pemphigoid and psoriasis has previously been noted. In most patients, the bullous lesions start during PUVA or UVB therapy of psoriasis. We report on a psoriatic patient who developed bullous pemphigoid approximately three to four weeks after discontinuing PUVA treatment, and then three to four weeks after exposure to natural sunlight. Delayed induction of bullous pemphigoid has not been previously reported, and it raises the question of ultraviolet therapy inducing subclinical or latent bullous pemphigoid in psoriatic patients.     

Dual diagnosis of Pemphigus and pemphigoid. Retrospective review of thirty cases in the literature.

BACKGROUND: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. OBJECTIVE: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. METHODS: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. RESULTS: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. CONCLUSION: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.     

Polymorphic pemphigoid. Is it still worth using this terminology?

The term polymorphic pemphigoid has been used in the literature as a variant of bullous pemphigoid. But this term is imprecise and now obsolete, since patients with linear IgA dermatosis have been reported under this terminology. The patients who develop an atypical subepidermal bullous disease with clinical and histological features of both bullous pemphigoid and dermatitis herpetiformis may actually be classified into three groups: (1) vesicular variant of bullous pemphigoid; (2) linear IgA dermatosis, and (3) mixed subepidermal bullous disease.     

Eosinophilic spongiosis in association with bullous pemphigoid and chronic lymphocytic leukaemia

We report the onset of an unusual blistering eruption following a diagnosis of B-cell chronic lymphocytic leukaemia (CLL). The histology consistently showed eosinophilic spongiosis, but the diagnosis of bullous pemphigoid was only confirmed after 13 years by repeated immunofluorescence studies. The occurrence of subepidermal blistering diseases in association with B-cell lymphoproliferative disorders is rare; a recent study showed that the majority of these cases are epidermolysis bullosa acquisita, confirmed by immunological studies. Only two cases of immunofluorescence-proven bullous pemphigoid in association with CLL have been previously reported.