生物制剂治疗泛发性脓疱型银屑病的研究进展

泛发性脓疱型银屑病(GPP)的治疗较为困难。近年来的研究表明,TNF-α、IL-17、IL-1、IL-36、IL-12/23在GPP的发病机制中均起着重要作用,可以作为GPP的特异性免疫治疗靶点。针对这些靶点的新型生物制剂如TNF-α抑制剂、IL-17抑制剂、IL-1受体拮抗剂、IL-36受体拮抗剂以及IL-12/23拮抗剂的使用,可以有效治疗该病。本文就近年来相关临床研究文献展开综述,为GPP的临床治疗提供新的思路。     

局限性脓疱型银屑病的靶向治疗新进展

局限性脓疱型银屑病是脓疱型银屑病的一种分型,因涉及身体重要功能部位,对患者的生活质量影响较大。目前其治疗无金标准,传统药物治疗疗效欠佳,靶向药物成为治疗新转机。本文将对局限性脓疱型银屑病的靶向治疗进展进行综述。     

关节型银屑病脓疱型银屑病并发1例

患者男性,水产个体,52岁,自1990年8月起头皮、躯干及四肢等处出现鳞屑性红斑.1992年下半年,间断服用"复方青黛丸",外用"去炎松尿素软膏"、"恩肤霜"等.1997年10月,在当地卫生所予醋酸确炎舒松一A局部封闭,每周一次,先后注射100余支,皮损时而缓解,时而复发.2000年元月自行停药,继而全身出现脓疱,左手指关节肿胀、疼痛,活动受限,并伴持续发热,体温曾达39.5℃.在外院用多种中西药治疗,半月前因上感、咳嗽,病情再次加重,于2000年9月拟诊"脓疱型银屑病,关节型银屑病",收住院.     

关节型、脓疱型银屑病1例

报告1例男性患者。全身关节肿痛16年,全身鳞屑性红色斑丘疹7年,发热10个月,出现脓疱2个月,伴白细胞及血小板升高、贫血、血沉加快、低蛋白血症。用环孢素A,新体卡松、抗生素等治疗无效,加用泼尼松后病情逐渐得到控制。     

阿维A治疗脓疱型银屑病3例

阿维A为第２代芳香维A酸类药物，国外已代替阿维A酯广泛用于银屑病的治疗，尤其对脓疱型及红皮病型银屑病有较好的疗效。笔者用阿维A（新体卡松）治疗脓疱型银屑病３例，取得较满意疗效，现报告如下。例１．女，２０岁，脓疱型银屑病史近２０年，此次入院前２月反复出现全身弥漫性红斑脓疱，伴发热（体温最高３９．０℃）。查体：T３８．６℃，系统检查无异常。皮肤科情况：头面部弥漫性红斑鳞屑，躯干四肢也可见弥漫性红斑，上有散在分布针尖至绿豆大小的脓疱，双前臂及双胫前可见“脓湖”。血脂、心电图、X线胸片及腹部B超均正常。入院…     

脓疱型银屑病研究进展

脓疱型银屑病是一种多因素影响及介导下的免疫异常性皮肤病,其病因复杂;遗传因素、免疫机制、感染、药物及环境等多种因素均参与发病;治疗方法包括维甲酸、免疫抑制剂及糖皮质激素、物理治疗、生物制剂等多种疗法。     

泛发性脓疱型银屑病的研究进展

泛发性脓疱型银屑病(generalized pustular psoriasis,GPP)皮损广泛,多伴有全身症状,甚至会危及生命。GPP的发病机制仍不清楚,其治疗同样是非常棘手的问题,选择性生物制剂的应用给GPP的治疗带来新的希望。现将近年来关于GPP的某些研究进展进行简要综述。     

IL-17A单抗治疗儿童泛发性脓疱型银屑病一例并文献复习

传统药物如甲氨蝶呤,阿维A和环孢菌素A等治疗儿童泛发性脓疱型银屑病(GPP)受到限制,目前没有针对青少年GPP的标准化诊疗指南,本文报道IL-17A单抗成功治疗儿童脓疱型银屑病一例并进行文献复习.     

Tigason治疗10例脓疱型银屑病疗效观察

Ｔｉｇａｓｏｎ治疗１０例脓疱型银屑病疗效观察张正中①王官清①眭维耻①＊脓疱型银屑病较为少见，治疗困难。我科于１９９３年１０月～１９９５年９月采用Ｔｉｇａｓｏｎ（瑞士罗氏大药厂出品）治疗１０例，取得满意疗效，报告如下。１资料与方法１．１临床资料：１０例...     

Treatment of pustular psoriasis with infliximab

Pustular psoriasis is a severe form of psoriasis with a generalized pustular eruption. Since TNF‐α plays an important role in pustular psoriasis, we treated two patients who had not responded to established therapy regimens with the TNF‐α antagonist infliximab. Both patients showed significant improvements of their skin eruption and their general condition within three days after treatment and without any side effects. We suggest that infliximab is a therapeutic option for severe therapy‐resistant pustular psoriasis because a single well‐tolerated dose significantly ameliorates the patient's condition.     

Clinical characteristics of Japanese pustular psoriasis: A multicenter observational study

Generalized pustular psoriasis (GPP) is a rare and severe subtype of psoriasis. Because of its rarity, GPP studies with a large sample size have been scarce. We studied the characteristics of GPP and pustular psoriasis using data from the West Japan Psoriasis Registry that had been registered until the end of December 2020. The dataset included 104 patients with pustular psoriasis and 1290 patients with other subtypes of psoriasis. Multivariate analysis revealed a significantly greater number of female patients, a significantly lower mean body mass index, and a significantly lower ratio of habitual drinkers in pustular psoriasis, compared to other subtypes of psoriasis. Of the 104 patients, 102 had GPP, including 88 von Zumbusch, 10 juvenile-onset, and four annular pustular psoriasis. Although the male : female ratio of GPP with psoriasis vulgaris (GPP+PsV) (47/20) was similar to that of psoriasis in Japan, the GPP without PsV (GPP?PsV) group highlighted a female predominance (13/22). The mean age at GPP onset was 45.3 years, and the mean interval from PsV onset to GPP onset was 12.5 years. Four of nine patients with GPP had an IL36RN gene mutation. Infection, medicine, and pregnancy were the precipitating factors for GPP. A family history of psoriasis was present in eight (7.8%) patients with GPP. Twenty-four patients with GPP had psoriatic arthritis. Biologics were used in 76.5% of patients with GPP, followed by etretinate (37.3%), cyclosporine (24.5%), methotrexate (13.7%), apremilast (8.8%), and granulocyte and monocyte adsorption apheresis (6.9%). Etretinate was used in 17 (51.5%) of 33 patients with GPP with less than 10-year history. Thus, etretinate remains a good treatment option for GPP even in the era of biologics. Hypertension was the most commonly identified comorbidity, followed by diabetes. We believe that the characteristics revealed in this study can further contribute to effective GPP management.     

Juvenile generalized pustular psoriasis treated with etanercept

An 8‐year‐old boy with general pustular psoriasis (GPP) and iatrogenic secondary Cushing syndrome was treated successfully with etanercept after he had failed on acitretin, methotrexate, and methylprednisolone therapy. GPP is a severe and very rare variant of psoriasis in children often accompanied by life‐threatening complications. Retinoids, cyclosporine, methotrexate, or dapsone used in a small number of case series and case reports were effective. Etanercept is a recombinant human tumor necrosis factor‐alpha (TNF‐alpha) receptor protein fused with Fc portion of IgG1 that binds to TNF‐alpha, approved by Food and Drug Administration for the treatment of moderate‐to‐severe plaque psoriasis in children and teens who have not responded to other psoriasis treatments. In our patient, etanercept demonstrated significant clinical response associated with long‐term efficacy without acute exacerbation, excellent tolerability, and good safety profile.     

脓疱性银屑病76例临床分析

目的：分析脓疱性银屑病的临床特征。方法：对76例脓疱性银屑病患者按照国际上通行的分类方法进行诊断和分类，并对其病史资料、临床特征和治疗经过进行分析。结果：该76例脓疱性银屑病在同期就诊的银屑病患者中占8．6%，其中以局限性居多．占71．0％．主要累及掌跖部位；泛发性病例中5种临床亚型均各有2～6例患者，表现为程度不一的红斑、脓疱和鳞屑根据不同的皮疹特点给予窄谱中波紫外线(NB-UVB)、长波紫外线(UVA）、维A酸等综合治疗取得较为满意的效果，有效率为49．2％结论：脓疱性银屑病的临床表现和治疗反应在不同患者间差异较大。新的分类方法对指导临床实践具有积极意义。     

Juvenile generalized pustular psoriasis

Generalized pustular psoriasis (GPP) is an erythrodermic, generalized form of pustular psoriasis. GPP is rare in children. The present study describes a case of juvenile GPP and reviews 12 juvenile GPP inpatients treated at our hospital in the period 1978-2005.     

妊娠泛发性脓疱型银屑病研究进展

妊娠泛发性脓疱型银屑病（GPPP）是妊娠期发生的一种罕见而严重的脓疱型银屑病。临床以全身急性泛发性红斑,伴浅在性无菌性脓疱为特征,发展迅速,可融合成脓湖,常伴高热、寒战等严重全身症状,甚至危及母胎生命。糖皮质激素、环孢素、TNF-α抑制剂等治疗有一定疗效,可降低GPPP死亡率。本文从病因、治疗等方面对GPPP研究进展进行概述。     

泛发性脓疱型银屑病44例临床分析

目的：探讨脓疱型银屑病临床表现及治疗效果。方法：对44例脓疱型银屑病患者进行临床回顾。结果：多种因素可导致脓疱型银屑病发病，感染和滥用糖皮质激素是其主要诱因；发热和血象升高是其主要系统性临床表现；糖皮质激素联合第二代维A酸治疗脓疱型银屑病明显优于其他两组治疗效果。结论：系统应用糖皮质激素联合维A酸药物可作为泛发性脓疱型银屑病治疗的首选方案。