寻常型天疱疮患者疱液及血清白介素-10的检测及其临床意义

目的：探讨白细胞介素10(IL-10)在寻常型天疱疮患者的表达及其临床意义。方法：采用ELISA方法检测36例寻常型天疱疮患者活动期血清、疱液和缓解期血清中IL—10的水平。结果：寻常型天疱疮患者活动期血清中IL—10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—10的水平明显下降，活动期疱液IL-10水平明显高于活动期血清中的水平。结论：IL—10可能在寻常型天疱疮的发病起重要作用，并且与病情的活动性有关。     

寻常型银屑病患者血清白介素-2水平测定

2000年10月～2001年10月笔者采用ELISA方法检测了30例寻常型银屑病患者血清中白介素-2(IL-2)的浓度,并进行了银屑病面积和严重程度指数(PASI)评分,了解两者之间的相关性,进一步探讨银屑病的发病机制。病例组:30例寻常型银屑病患者均来自笔者所在科室门诊,排除相关疾病及各种影响因素。其中男14例,女16例,平均年龄(28.23±12.60)岁(9～60岁)。平均病程(32.81±50.63)个月(20d～16年)。对照组:20例,男8例,女12例;平均年龄(30.10±10.00)岁(17～45岁)。经t检验,性别、年龄差异无显著性。ELISA检测严格按照IL-2试剂盒(深圳晶美公司)说明…     

寻常型天疱疮患者娩出一天疱疮患儿

报告1例寻常型天疱疮患者娩出天疱疮患儿。患者女,33岁,妊娠4个月时口腔、外阴黏膜反复出现糜烂,嗣后躯干,四肢陆续出现水疱,水疱破溃后形成不易愈合的糜烂面。皮损经组织病理,免疫病理检查,诊断为寻常型天疱疮,经糖皮质激素等药物治疗后皮损痊愈。治疗中患者早产娩出一女婴,也患有天疱疮,该新生儿未经治疗7d后皮损自愈。     

寻常型天疱疮患者的护理体会

寻常型天疱疮是以表皮内棘细胞松解为特点的大疱性皮肤病,好发于中青年,无性别年龄差异,发病机制与自身免疫有关。其病程长,易感染,性质严重,治疗中除早期足量的皮质类固醇激素、免疫抑制剂、支持疗法外,皮损的护理也十分重要,护理不当仍可危及生命。我科收治了1例重症寻常型天疱疮患者,经精心治疗和护理,住院17天,病情好转出院。     

寻常型天疱疮诊断和治疗的专家建议

寻常型天疱疮（pemphigus vulgaris,PV）是天疱疮中最常见类型,其主要特点是血清中产生针对表皮细胞间桥粒的自身抗体,临床表现为松弛性水疱、大疱,伴有顽固性、痛性黏膜糜烂和溃疡,组织病理出现特征性棘层松解现象。PV治疗的主要目的是控制病情,促使皮损和黏膜尽快愈合,减少治疗的不良反应和提高患者的生活质量,力争长期缓解,直至痊愈。治疗的挑战在于长期稳定控制病情、减少复发、避免长期应用糖皮质激素（简称激素）和免疫抑制剂的不良反应,减少激素用量。由于该病较少见,大样本随机、双盲、安慰剂对照的前瞻性研究较少。欧洲和日本已经形成了自己的治疗共识,但目前我国尚无类似共识形成。本文在综合不同国家和地区共识性文献的基础上,结合中国国情,经国内不同地区的专家反复讨论修订,形成了PV的诊断和治疗指导性意见和建议……     

皮肌炎并发寻常型天疱疮

寻常型天疱疮作为一种自身免疫性疾病,有时可并发其他的自身免疫性疾病,然而并发皮肌炎者罕见报道.报告1 例中年女性发生皮肌炎5 个月后,四肢出现水疱,口腔出现溃疡,经组织病理检查及免疫荧光检查诊断为寻常型天疱疮.     

强的松龙联合环磷酰胺冲击治疗寻常型天疱疮4例

4例均为 30～ 6 0岁女性 ,表现为全身散在松弛性水疱 ,尼氏征 ( ) ,皮损面积占体表面积 40 % 1例 ,超过 5 0 % 3例 ,口腔粘膜均受累 ,病程 4月～ 3年 ,反复发作 ,1例空腹血糖及血压偏高 ,余无明显异常。入院后予强的松龙 (ＣＳ) 1.5～ 2 .0ｍｇ/(ｋｇ .ｄ)加硫唑嘌呤 10 0ｍｇ/ｄ治疗 ,7～ 10天后皮损无好转 ,有新疱出现 ,停硫唑嘌呤 ,改用环磷酰胺 (ＣＴＸ) 8～ 10ｍｇ/ (ｋｇ .ｄ)加入 5 %葡萄糖液静滴 ,每 2周连续用药 2天为 1疗程 ,共 3～ 4疗程 ,嘱病人多饮水 ,并监测血尿常规、肝肾功能等。ＣＴＸ冲击2～ 3天后水疱渐干涸 ,经 1～ 3…     

寻常型天疱疮伴肺癌1例

1临床资料患者,男,74岁。因"全身皮肤反复水疱6年,咳嗽、咳痰5个月"就诊。患者6年前无明显诱因口唇出现水疱,易破溃,逐渐全身皮肤出现绿豆大小水疱、糜烂。曾在多家医院就诊,皮肤活检病理显示表皮内水庖,基底层上部棘突松解,有绒毛形成(图1),诊断     

新生儿寻常型天疱疮1例

新生儿天疱疮是由于患天疱疮的产妇血中致病性IgG抗体通过胎盘,导致胎儿在出生时出现类似产妇的疾病表现.我们报告1例患儿为新生儿寻常型天疱疮,而其母亲为增殖型天疱疮患者.     

Nail involvement in pemphigus vulgaris

BACKGROUND: Involvement of the nail unit in pemphigus vulgaris (PV) is thought to be rare. OBJECTIVES: To determine the frequency of nail changes among patients with PV. METHODS: Nail changes were recorded in patients with PV and were analysed retrospectively. Biopsies were taken from the nail bed and nail matrix for histological and immunofluorescence observations. Microscopic (potassium hydroxide) examination and fungal culture were performed. RESULTS: Nail changes were present in 30 of 64 (47%) patients with PV. Sixteen patients had onychomycosis and 14 had nail changes due to PV. These included onychomadesis, discoloration, deformity, onychorrhexis, onycholysis, subungual haemorrhage, subungual hyperkeratosis and paronychia. Onychomycosis affected fingernails and toenails, but changes due to PV occurred only in fingernails, most commonly on the thumb and index finger. No connection was found between the patient's occupation and nail involvement, and there was no correlation with the severity or duration of PV. In a control group of 64 patients of similar sex and age, only two had non-specific fingernail changes. CONCLUSIONS: Nail changes in PV may be more frequent than previously thought.     

Childhood pemphigus vulgaris: five cases in 16 years

Pemphigus vulgaris (PV) usually occurs in adults. There are only a few reports of large PV series concerning childhood cases. We report here five cases of PV in patients younger than 16 years. They were analyzed among 169 PV cases out of a total of 192 pemphigus patients diagnosed between 1988-2004. The ratio of childhood cases was 2.9% in our large PV series. This relatively high ratio of childhood patients suggests that PV should not be neglected in the differential diagnosis of bullous lesions in childhood. Four of the five cases were followed up between 2-4 years and all of these four cases showed at least one relapse. PV also seems to show a relapsing course in the pediatric age group like in adults.     

Direct immunofluorescence of the outer root sheath in anagen and telogen hair in pemphigus vulgaris and pemphigus foliaceus

Direct immunofluorescence of peri‐lesional skin is the gold standard in the diagnosis of pemphigus. A specific immunofluorescence pattern may also be demonstrated in the outer root sheath of anagen and telogen hair. We demonstrated an intercellular reticular deposition of immunoglobulin G in the outer root sheath of plucked anagen and telogen hair in all pemphigus vulgaris patients with active disease and for the first time in all patients with active pemphigus foliaceus. Moreover, we demonstrated for the first time that plucked hair samples may be kept at ?20°C for at least 2 weeks before immunofluorescent staining and analysis.     

Signalling pathways in pemphigus vulgaris

Acantholysis in pemphigus vulgaris is induced by binding of autoantibodies to desmoglein 3 (Dsg3). The roles of signalling pathways on development of acantholysis have recently been extensively studied. In the study by Sayar et al., recently published in Exp Dermatol, epidermal growth factor receptor (EGFR) signalling was activated in both in vivo and in vitro pemphigus vulgaris experimental models. However, while EGFR inhibitors suppressed activity of p38 mitogen‐activated protein kinase (p38MAPK) linearly, they suppressed activity of c‐Myc and acantholysis in a non‐linear, V‐shaped relationship. These findings indicated complicated interactions among EGFR, p38MAPK and c‐Myc in pemphigus vulgaris pathology.     

The transition of pemphigus vulgaris into pemphigus foliaceus: a reflection of changing desmoglein 1 and 3 autoantibody levels in pemphigus vulgaris

The transition of pemphigus vulgaris (PV) into pemphigus foliaceus (PF) is rare and the immunological changes underlying this event are not well understood. We report a 44-year-old woman who presented with oral and cutaneous erosions typical of PV. Over a 9-year period, the clinical features evolved into those of PF. To examine whether quantitative changes in desmoglein (Dsg) antibodies were associated with this transition, Dsg1 and Dsg3 antibody levels were measured by enzyme-linked immunosorbent assay in 82 sequential serum samples collected over this period. At presentation, when the phenotype was PV with oral and cutaneous erosions, antibodies to both Dsg1 and Dsg3 were detected. The disappearance of oral involvement was associated with a decline in Dsg3 antibodies, which are now undetectable, while the development of more severe skin involvement was associated with rising Dsg1 antibody levels. These data strongly suggest that the change in clinical features is a reflection of qualitative and quantitative changes in antibody profile. It is not known whether the transition to PF is permanent or whether disease relapses in the future may be associated with the re-emergence of Dsg3 antibodies, oral ulceration and a PV phenotype.     

Rituximab in refractory pemphigus vulgaris

ABSTRACT:  Pemphigus vulgaris (PV) is a severe chronic autoimmune blistering disease of skin and mucous membranes. The use of systemic corticosteroids in pemphigus has dramatically reduced its mortality rate, but the long-term use of steroids leads to severe side effects, many of which are serious. For this reason it is often necessary to add immunosuppressive agents to the regimen. However, there are occasional refractory cases in which therapy with conventionally accepted modalities is either not efficacious or not possible on account of side effects. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. We present an instance of refractory PV successfully treated with rituximab. The successful treatment of pemphigus described here demonstrates that rituximab is a viable therapeutic option for patients with refractory PV.     

Herpes simplex virus 1 and cytomegalovirus are associated with pemphigus vulgaris but not with pemphigus foliaceus disease

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are blistering autoimmune diseases that depend on interaction between genetic and environmental factors. Viral infections, like herpes simplex viruses 1 and 2 (HSV1/2), cytomegalovirus (CMV), Epstein‐Barr virus and dengue virus, could trigger or exacerbate pemphigus. IgM and IgG antibodies against these viruses in serum from PV and PF, their relatives and controls were determined. HSV1/2 expression was evaluated by direct immunofluorescence (DIF) and qPCR in affected or not oral mucosa from PV patients compared with uninjured PF mucosa. IgG anti‐HSV1 was higher in the PV group compared with all groups. IgG anti‐CMV resulted higher in PV group compared with PF patients and PV relatives. HSV1 was confirmed by DIF and qPCR on oral samples from patients with PV. Lack of HSV1 expression in the oral mucosa of patients with PF corroborate that immunosuppressive therapy cannot be the main cause for HSV1 replication in PV disease.     

Outcome of pemphigus vulgaris

Background Pemphigus vulgaris can be divided into mucosal, mucocutaneous and cutaneous subtypes. A higher mortality rate has been shown with mucocutaneous involvement. Objectives The aim of this retrospective study was to analyse the association of clinical subtypes of pemphigus vulgaris with remission rates. Patients/methods One hundred twenty‐eight patients with pemphigus vulgaris, treated with prednisolone 2 mg/kg/day plus azathioprine 2 to 2.5 mg/kg/day, were enrolled. The partial and complete remission rates, at the end of the first and second years of treatment, and the number of relapses were compared in the three groups. Results After disease establishment, 71.1% had mucocutaneous, 18.8% had mucosal, and 10.2% had only cutaneous involvement. The mean duration of follow‐up was 53.5 ± 39.6 months. The mean duration it took the mucocutaneous group to reach a prednisolone dosage of 30 mg/day was significantly longer (P = 0.050). Mucocutaneous patients had a significantly lower rate of remission (31.9%) compared with those with only mucosal or cutaneous involvement (48.6%) at the end of the first year of the treatment (P = 0.029). After 2 years, mucocutaneous patients again had a lower remission rate (32.9% vs. 44.5%). Relapses were also more frequent in this subtype. Those presenting with mucosal or mucocutaneous erosions had a higher rate of active disease after receiving treatment for a year compared with those with only cutaneous presentation (66.7% vs. 45%; P = 0.057). Conclusions In mucocutaneous subtype, clinical control was achieved later, and they had a lower rate of remission at the end of the first and second years of treatment. They were also prone to relapses.     

A case of pemphigus vulgaris possibly triggered by quinolones

Pemphigus vulgaris is an autoimmune blistering disorder in which both genetic and environmental factors, mainly drugs, are thought to play a part in its aetiopathogenesis. Although some drugs that contain thiol groups, such as D-penicillamine and captopril, and non-thiol drugs, such as cephalosporin, have been reported to trigger or induce pemphigus, there are no previous reports regarding the influence of quinolones in triggering this disease. Here we present a case of pemphigus possibly triggered by quinolones.