王萍教授中药治疗儿童泛发性脓疱性银屑病3例

泛发性脓疱性银屑病(generalized pustular psoriasis,GPP)是银屑病中病情严重的一型,属难治性皮肤病。本型约占银屑病病人的0.77%[1],儿童罕见,近十年国内报道不足100例[2-7],应用中药治疗取得疗效,但报道尚少[8,9]。现将北京     

儿童脓疱型银屑病治疗进展

儿童银屑病是一种常见的炎症性皮肤病,而儿童脓疱型银屑病(Childhood pustular psoriasis,CPP)较为少见,是一种发生于儿童的全身性炎症性伴皮肤功能障碍的银屑病类型。CPP顽固且易复发,对患儿及其父母生活质量及发展都影响颇大。对于CPP,皮肤的局部治疗是必不可少的基础,维甲酸类、甲氨蝶呤、环孢素、生物制剂等为系统治疗中的常用药物,脓疱型银屑病儿童通常对光疗有较好的反应。此文介绍了目前针对于儿童脓疱型银屑病的治疗共识与经验,为现在的治疗进展进行了概括。     

泛发性脓疱型银屑病120例临床分析

目的：分析泛发性脓疱型银屑病(generalized pustular psoriasis,GPP)的临床特征、实验室检查及治疗方案,评估疗效。方法：回顾性分析我院皮肤科住院部收住的120例GPP患者（146次住院）的临床资料。结果：①儿童（≤14岁）好发于夏季（40.00%）,以上感(52.00%)为主要诱因;成人（>14岁）好发于春季（34.71%）,以上感（27.27%）和药物（25.62%）为主要诱因,无寻常型银屑病（PV）病史者中主要诱因为上感（45.45%）,有PV病史者主要诱因为药物因素（30.30%）。②无PV病史者较有PV病史者发病年龄早,住院时间和脓疱消退时间长。低钙患者较非低钙患者更易发热。阿维A和阿维A联合白芍总苷治疗GPP患者总有效率为89.57%。结论：儿童组和成人组,有PV病史和无PV病史组,低钙患者和非低钙患者临床特点不尽相同。发热和低钙与疾病严重程度相关。阿维A治疗GPP疗效确切。     

儿童与成人泛发性脓疱性银屑病的临床与治疗比较分析

目的探讨泛发性脓疱性银屑病(generalized pustular psoriasis,GPP)在儿童与成人中临床表现与治疗的不同。方法回顾性分析37例GPP患者的临床资料,从临床表现、实验室检查、治疗等方面进行分析。结果成人发展为GPP前多有寻常性银屑病病史,且病程长;治疗方面成人多选用免疫抑制剂或维A酸类药物,儿童多选用抗生素配合免疫抑制剂。结论儿童与成人GPP发病病程、治疗方面有所不同。     

脓疱型银屑病中的HL-A抗原

作者对97例脓疱型银屑病(PP)患者作了人类白细胞抗原(HL-A)分型的检查,以期明确PP与寻常型银屑病(PV)的区别,澄清PP不同亚型之间的关系。97例PP中包括局限性掌跖脓疱型银屑病(LPP)30例,连续性肢端皮炎(AC)14例,持久性掌跖脓疱病(PPP)35例及泛发性脓疱型银屑病(GPP)18例。在LPP,AC及GPP中22例有关节炎,     

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。     

局限性环形增殖性脓疱型银屑病五例

脓疱型银屑病系银屑病亚型之一.通常分泛发性和局限性两大类,其表现形式有泛发型(Von Zumbusch型)、环型、发疹型、肢端型(Hallapeau型)及掌跖型〔Barher型).     

阿维A与甲氨蝶呤治疗泛发性脓疱型和红皮病型银屑病的疗效及安全性评价

目的:比较阿维A与甲氨蝶呤(MTX)治疗泛发性脓疱型和红皮病型银屑病的疗效。方法:将入选的泛发性脓疱型和红皮病型银屑病患者分为阿维A组与MTX组。结果:泛发性脓疱型银屑病共21例,其中阿维A组10例,治疗总有效率90.0%;MTX组11例,治疗总有效率72.7%。红皮病型银屑病共24例,阿维A组11例,治疗总有效率45.5%;MTX组13例,治疗总有效率61.5%。阿维A与MTX治疗泛发性脓疱型和红皮病型银屑病的总有效率组间差异无显著性(P>0.05)。阿维A组的不良事件发生率为95.2%,MTX组为25.0%,组间差异有显著性(P<0.001)。治疗1月后随访:阿维A组复发率9.5%;MTX组复发率4.2%,组间差异无显著性(P>0.05)。结论:在泛发性脓疱型银屑病的治疗中,阿维A的疗效优于MTX的疗效;在红皮病型银屑病的治疗中,MTX的疗效优于阿维A。阿维A组的不良事件发生率明显高于MTX组。     

麦考酚酯联合阿维A治疗难治性脓疱型银屑病5例临床观察

目的观察小剂量麦考酚酯联合阿维A治疗难治性泛发性脓疱型银屑病的疗效及安全性。方法对本科住院治疗的5例既往予阿维A单独或联合其他药物治疗无效的泛发性脓疱型银屑病患者,分析在予麦考酚酯0.5g(2次/d)联合阿维A 30mg(1次/d)治疗后的疗效及安全性。结果该5例患者联合治疗平均显效时间为2.8天,3例8天内好转出院,1例8天时痊愈出院,治疗及随访中均未发现麦考酚酯或阿维A相关的不良反应,1例复发。结论小剂量麦考酚酯联合阿维A治疗难治性泛发性脓疱型银屑病起效快,可供单独使用阿维A或联合其他药物治疗抵抗的患者选择,无明显不良反应。     

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。     

Successful use of acitretin in conjunction with narrowband ultraviolet B phototherapy in a child with severe pustular psoriasis, von Zumbusch type

Severe pustular psoriasis von Zumbusch type is a therapeutic challenge not only in adults, but even more in children. We report a 3(1/2)-year-old boy who developed a generalized flare of diffusely scattered pustules on erythematous skin which rapidly progressed to large exuding areas. The clinical presentation and investigations including histopathological examination of a biopsy and negative bacterial cultures were consistent with the diagnosis of pustular psoriasis von Zumbusch type. Upon initial treatment with methylprednisolone, acitretin and antibiotics the extent of the disease declined. However, several attempts to reduce the dose of the oral corticosteroid were followed by immediate severe flares. Additional treatment with narrowband ultraviolet B (NB-UVB, 311-313 nm UVB) resulted in a rapid arrest of disease activity and allowed the corticosteroid to be tapered off. After 10 irradiations the patient was both off steroid and disease free. NB-UVB therapy was subsequently reduced to twice-weekly exposures and acitretin gradually diminished to a maintenance dose of 0.3 mg kg(-1) daily. We conclude that NB-UVB in conjunction with acitretin is a potent therapeutic regimen for the treatment of severe pustular psoriasis von Zumbusch type in childhood.     

儿童von Zumbusch型脓疱性银屑病26例临床分析

目的:为了提高对儿童vonZumbusch型脓疱性银屑病的临床认识,提高治愈率。方法:回顾性分析26例vonZum-busch型脓疱性银屑病患儿的临床及实验室资料。结果:所有患儿均有vonZumbusch型临床表现。男女发病之比为1.9∶1,平均年龄5.8岁。其中10例做了T细胞亚群抗原测定,CD4+下降,CD8+平均值增加,CD4/CD8比值异常。17例患儿行皮损组织病理检查示,角质形成细胞变性,表皮水肿,表皮内有中性粒细胞移入,棘层上部海绵样脓疱形成,真皮血管扩张,血管周围有淋巴细胞及中性粒细胞浸润。结论:vonZumbusch型脓疱性银屑病为一种少见的疾病,临床及组织病理上具有特征性,去除诱发因素,及时控制感染,可在实验室检测指标的定期监控下,首选阿维A(新体卡松)口服治疗,症状明显得到改善。     

Acute respiratory distress syndrome as a complication of generalized pustular psoriasis

Since 1991, eight cases of pulmonary leak capilary syndrome have been described associated with pustular or erythrodermic psoriasis induced or not by the use of acitretin or sirolimus - being one of the cases, fatal. We report the case of a female patient with diagnosed GPP or von Zumbusch and multiple hospitalizations due to such condition. At that time, the condition was evolving with pulmonary onset and resolution with corticosteroids. The patient was not using any previously described medication that could precipitate pulmonary condition (methotrexate and acitretin). This is a rare complication associated with psoriasis which has not been described in Brazilian patients so far.     

A dramatic response to a single dose of infliximab as rescue therapy in acute generalized pustular psoriasis of von Zumbusch associated with a neutrophilic cholangitis

Generalized pustular psoriasis of von Zumbusch is an unstable, inflammatory form of psoriasis, with the hallmark of neutrophil infiltration in cutaneous as well as extracutaneous lesions. It is often recalcitrant, making treatment difficult. Tumour necrosis factor-α antagonists including infliximab have been used with success in treating recalcitrant cases. We report a case of a 48-year-old Chinese female patient with a long-standing history of poorly controlled generalized pustular psoriasis which was resistant to multiple therapies. During a severe flare, a single dose of infliximab resulted in rapid clearing of cutaneous lesions, together with resolution of liver function abnormalities that are likely secondary to neutrophilic cholangitis. Subsequent maintenance therapy with acitretin allowed remission of pustular disease for 7 months. This demonstrates the efficacy of single-dose infliximab for both cutaneous lesions and systemic hepatic involvement in generalized pustular psoriasis.     

Angiogenic capability of peripheral blood mononuclear cells in psoriasis

The angiogenic capability of peripheral blood mononuclear cells (MNCs) from patients with psoriasis was tested by lymphocyte-induced angiogenesis assay. The study was performed in 36 patients with psoriasis vulgaris, six with generalized pustular psoriasis of the von Zumbusch type, and five with palmo-plantar pustular psoriasis. When patients with psoriasis vulgaris were considered as a whole, no significant differences in the angiogenic capability of their MNCs could be detected, as compared with controls. However, when cases of psoriasis were classified into various types, the differences in angiogenic capability of MNCs became evident. This capability was found to be markedly increased in the active form of psoriasis vulgaris and decreased in the stationary stage of the disease. It was increased in palmo-plantar pustular psoriasis but markedly decreased in generalized pustular psoriasis of the von Zumbusch type.     

Psoriasis pustulosa generalisata--classification, clinical aspects and therapy. Review and experiences with 18 patients

On the basis of clinico-morphological criteria we suggest the following classification of various pustular forms of psoriasis into four subtypes: generalized pustular psoriasis (von Zumbusch) and its atypical forms, erythema anulare centrifugum-like psoriasis with and without pustulation (EACP); psoriasis vulgaris with pustulation; palmo-plantar pustular psoriasis (K?nigsbeck-Barber) and its acral variant; acrodermatitis continua suppurativa (Hallopeau), and transitional forms. This classification takes into consideration both clinical aspects and response to treatment, and allows a prognosis of the various types of pustular psoriasis. Owing to its mild course and good treatment results, EACP represents a special form of psoriasis pustulosa generalisata. In our group of patients, systemic glucocorticosteroid therapy has proved deleterious, whereas oral photochemotherapy and etretinate have been found to be highly effective modes of therapy with only mild side effects and have been able to induce even permanent remission in EACP.     

Treatment of pustular psoriasis with clofazimine

Two cases of generalized pustular psoriasis (von Zumbusch) were successfully treated with clofazimine. No side effects were noted except deep red staining of the skin which faded afterwards.     

Bullous and non-bullous ichthyosiform erythroderma associated with generalized pustular psoriasis of von Zumbusch type

Bullous ichthyosiform erythroderma (BIE) and non-bullous ichthyosiform erythroderma (NBIE) are rare congenital ichthyoses. Generalized pustular psoriasis (GPP) of von Zumbusch type is a rare and severe form of psoriasis marked by desquamative and pustular erythroderma associated with fever and altered general conditions. We report two adults with an ichthyosis typical of BIE in the first case and NBIE in the second, without any previous history of psoriasis, who presented with a severe and relapsing GPP of von Zumbusch type. Using current knowledge of the genetic relationship between psoriasis and congenital ichthyoses, we discuss the possibility of a common physiopathological link between congenital ichthyoses and GPP, and examine the possible therapeutic problems resulting from this pathological association, especially in BIE.     

Methotrexate toxicity in the treatment of generalized pustular psoriasis

In a sixty-seven year old woman with a seventeen-year history of psoriasis, generalized pustular psoriasis of von Zumbusch developed. She was treated with parenteral methotrexate in two divided doses over a one-week period. Severe acute granulocytopenia developed with a drop in the white blood cell count from 27,000 to 400. We postulate that the pustular psoriasis stimulated rapid proliferation of the granulocyte series with a high percentage of the precursor cells in the bone marrow in the S-phase of the cell cycle. The subsequent dose of methotrexate resulted in a blockage of DNA synthesis in these cells and subsequent failure of maturation beyond the mitotic compartment in the bone marrow. Caution is recommended in the use of methotrexate in patients with acute generalized pustular psoriasis.     

Pustular Psoriasis in Childhood and Adolescence: A 20‐Year Single‐Center Experience

Pustular psoriasis (PP) is rare in children. The small number of reported cases makes deciding on treatment and follow‐up challenging. The current study was an evaluation of treatment approaches and courses of PP in 18 children diagnosed and followed over a 20‐year period. From 1992 to 2011 we treated 1,447 children with psoriasis, 18 of whom had PP. Follow‐up was 2 to 19 years. At the time of initial manifestation of PP, our patients were 1.5 months to 16 years old. Seven patients had a previous history of psoriasis vulgaris. Three children entered long‐term remission after one pustular attack, 10 developed psoriasis vulgaris, 2 are currently under treatment, and 3 were lost to follow‐up. Treatment with acitretin, cyclosporine, or methotrexate was efficacious and well tolerated. PP is rare, but according to our experience, it has a good prognosis in children.