大水疱

Dystrophic epidermolysis bullosa is a group of genetic disease caused by loss-of-function mutations in the type Ⅶ collagen gene. The clinical phenotype is characterized by the blistering and scaring of the skin from trauma and there is increased complication of squamous cell carcinoma, which is the major cause of death in patients with DEB.

营养不良性大疱性表皮松解症是一种由于人体Ⅶ型胶原基因突变或缺失所引起的遗传性疾病，临床特征为创伤诱发的水疱和瘢痕，并发症以恶性鳞状细胞癌多见，是导致患者死亡的主要因素，目前仍无有效的治疗方法。

Epidermolysis bullosa represents a group of genodermatoses characterized by fragility, easy blistering of the skin and mucous membranes.

遗传性大疱型表皮松解症是一组皮肤、粘膜脆性增加，容易出现水疱大疱的遗传性皮肤病。

Background: Epidermolysis bullosa is a rare disease that is usually inherited, begins early in life, and results in bullae and erosion at sites of trauma, usually on the hands, elbows, knees, and feet.

背景： 大疱性表皮松解症是一种罕疾病，通常有遗传性、生命的早期发病、导致受伤部位有大水疱与糜腐蚀形成，通常发生在手、手肘、膝盖和脚。

In the 188 patients with extensive bullous pemphigoid,topical corticosteroids were superior to oral prednisone (p=0.02), with one-year survival rate 76% vs. 58%, disease control at three weeks 99% vs. 91%, and severe complication rate 29% vs.

188位广泛性大水疱性类天疱疮患者中，外用皮质肾上腺类脂醇的效果优於口服prednisone（p＝0.02），二者的年生存率分别为76％及58％，三周疾病控制率分别为99％及91％，而严重并发症发生率则分别为29％及54％。

Feb. 4, 2002 -- People with bullous pemphigoid no longer need to endure the potential side effects of oral corticosteroids, based on a French study reported in the Jan. 31 issue of The New England Journal of Medicine .

2002年2月4日──1月31日《The New England Journal of Medicine》杂志上发表的一篇法国的研究报道，患有大水疱性类天疱疮的患者，不必再忍受口服皮质肾上腺类脂醇所可能导致的副作用。

Bullous pemphigoid, the most common blistering autoimmune disease of the skin, is manifested by cutaneous blisters without mucosal involvement.

大水疱性类天疱疮是最常见的起疱性皮肤自身免疫性疾病，其特徵为皮肤起疱，真皮不受累。

In the 153 patients with moderatebullous pemphigoid, outcome was similar inthe groups receiving topical or oral steroids, but the total number of days of hospitalization and the number of severe complications were lower in patients treated topically.

153位中度大水疱性类天疱疮患者中，接受外用皮质肾上腺类脂醇与口服皮质肾上腺类脂醇治疗的两组，治疗效果类似。但在使用外用皮质肾上腺类脂醇的患者，总住院日以及严重并发症的数目都较低。

"Topical corticosteroid therapy is effective forboth moderate and severe bullous pemphigoid and is superiorto oral corticosteroid therapy for extensive disease," write Pascal Joly, MD, PhD, from the University of Rouen in France, and colleagues."Topical corticosteroids should be considered the standard treatment for patients with extensive bullous pemphigoid."

外用皮质肾上腺类脂醇治疗对於中度及严重大水疱性类天疱疮都很有效，对於广泛性大水疱性类天疱疮，其效果优於口服皮质肾上腺类脂醇，」法国鲁昂大学的Pascal Joly医师，PhD及其同事写道：「应该考虑将外用皮质肾上腺类脂醇作为广泛性大水疱性类天疱疮治疗的黄金标准。

In a randomized, multicenter trial, 341 patients with moderate or extensive bullous pemphigoid received large doses of high-potency topical corticosteroids -- either topical clobetasolpropionate cream (40 g per day) or oral prednisone (0.5 mg perkilogram of body weight per day for those with moderate diseaseand 1 mg/kg/day for those with extensive disease)-- applied over their entire body.

在此次追踪多中心的试验中，341位中度或广泛性大水疱性类天疱疮患者接受大剂量较强外用皮质肾上腺类脂醇全身治疗──使用外用clobetasol propionate乳剂（40g/日）或者口服prednisone（中度患者0.5mg/千克体重/日，重度患者1mg/千克体重/日）。

The skin lesions progressed with numerous grouped bullae, and erosions on a noninflamed base on the palms and soles which resulted in syndactyl deformities and flexion contracture of hands and feet.

皮肤的损害进展成许多的大水疱与手掌、足掌未发炎的糜腐蚀，导致手脚的并指畸形与屈曲挛缩。

The split between the two groups can hardly be papered over.

这两个团体间的分歧难以掩饰。

This approach not only encourages a greater number of responses, but minimizes the likelihood of stale groupthink.

这种做法不仅鼓励了更多的反应，而且减少跟风的可能性。