Pash Syndrome

Pash 综合征
  • 文章类型: Journal Article
    吸附粒细胞和单核细胞分离术(GMA)是一种体外治疗方法,可选择性地从外周血中去除活化的髓系白细胞。该技术由带有醋酸纤维素珠作为吸收性白细胞提取载体的色谱柱组成,最初用于治疗溃疡性结肠炎。进行了文献检索,以提取最近发表的有关GMA在不同皮肤病患者中的临床疗效的研究,报告人口统计信息,临床症状,治疗和临床过程。皮肤病,GMA已经完成,包括全身性脓疱型银屑病,坏疽性脓皮病,掌足底脓疱型银屑病,Behcet病,甜蜜综合症,成人发作的斯蒂尔病,脓疱疮,反应性关节炎,痤疮和化脓性汗腺炎综合征,皮肤过敏性血管炎和系统性红斑狼疮。在大多数患者中,GMA是在常规治疗选择失败后开始的,在大多数情况下都有帮助。根据汇总的信息,对于患有多种皮肤病的患者,GMA可以被认为是一种有效的非药物治疗选择。用其他药物制剂很难治疗。
    Adsorptive granulocyte and monocyte apheresis (GMA) is an extracorporeal treatment that selectively removes activated myeloid lineage leukocytes from peripheral blood. This technique consists of a column with cellulose acetate beads as absorptive leukocytapheresis carriers, and was initially used to treat ulcerative colitis. A literature search was conducted to extract recently published studies about the clinical efficacy of GMA in patients with different skin disorders, reporting information on demographics, clinical symptoms, treatment and clinical course. Dermatological diseases, in which GMA has been performed, include generalized pustular psoriasis, pyoderma gangrenosum, palmoplantar pustular psoriasis, Behcet\'s disease, Sweet\'s syndrome, adult-onset Still\'s disease, impetigo herpetiformis, reactive arthritis, acne and hidradenitis suppurativa syndrome, cutaneous allergic vasculitis and systemic lupus erythematosus. In most patients, GMA was started after the failure of conventional therapeutic options and it was helpful in the majority of cases. Based on the information summarized, GMA could be considered a valid non-pharmacological treatment option for patients with several dermatological conditions, which are difficult to treat with other pharmacological preparations.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    坏疽性脓皮病,痤疮,化脓性汗腺炎综合征是一种罕见的以坏疽性脓皮病(PG)为特征的炎症性疾病,轻度至重度面部痤疮,和化脓性汗腺炎(HS)。它仅影响皮肤并代表一系列自身炎症的皮肤特征。化脓性无菌性关节炎(PA)的缺乏区分坏疽性脓皮病,痤疮,化脓性关节炎引起的化脓性汗腺炎(PASH)综合征,坏疽性脓皮病,痤疮,和化脓性汗腺炎(PA-PASH),坏疽性脓皮病,痤疮,化脓性汗腺炎,强直性脊柱炎(PASS),和化脓性关节炎,坏疽性脓皮病,和痤疮(PAPA)综合征。PASH综合征的确切病因和发病机制尚不清楚。PG和HS都包含在中性粒细胞性皮炎的范围内,这被认为是一种自身炎症综合征。从病理生理学的角度来看,它们表现出相似的机制,包括富含中性粒细胞的皮肤浸润和白细胞介素-1(IL-1)家族的过度表达。这些发现为这些顽固性疾病提供了指导。在这次审查中,我们描述了1例PASH综合征患者,该患者最初对免疫抑制治疗无反应,但对秋水仙碱和沙利度胺联合治疗有反应.我们回顾了有关PASH综合征管理的相关文献。
    Pyoderma gangrenosum, acne, and hidradenitis suppurativa syndrome is a rare inflammatory disease characterized by pyoderma gangrenosum (PG), mild to severe facial acne, and hidradenitis suppurativa (HS). It only affects the skin and represents cutaneous characteristics of a spectrum of autoinflammation. Lack of pyogenic sterile arthritis (PA) distinguishes the pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome from pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PA-PASH), pyoderma gangrenosum, acne, hidradenitis suppurtiva, and ankylosing spondylitis (PASS), and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA) syndromes. The exact etiology and pathogenesis of PASH syndrome remain unknown. Both PG and HS are contained in the spectrum of neutrophilic dermatitis, which is considered as an autoinflammatory syndrome. From a pathophysiological point of view, they show similar mechanisms, including neutrophil-rich cutaneous infiltration and overexpression of the interleukin-1 (IL-1) family. These findings provide guidance for these intractable diseases. In this review, we described a case of PASH syndrome in a patient who initially failed to respond to immunosuppressive treatment but responded to a combination of colchicine and thalidomide. We reviewed the relevant literature that focuses on PASH syndrome management.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号