Pyoderma gangrenosum - 坏疽性脓皮病
坏疽性脓皮病 (Pyoderma gangrenosum) 是一种罕见的炎症性皮肤病，其中疼痛的脓疱或结节变成逐渐生长的溃疡。 坏疽性脓皮病 (pyoderma gangrenosum) 不具有传染性。治疗方法可能包括皮质类固醇、环孢素或各种单克隆抗体。虽然它可以影响任何年龄段的人，但主要影响 40 多岁和 50 多岁的人。
溃疡性结肠炎患者的腿上。
References
Pyoderma gangrenosum 是一种罕见的皮肤病，会导致边缘呈红色或紫色的疼痛性溃疡。它被归类为一种炎症性疾病，属于中性粒细胞性皮肤病的一部分。 pyoderma gangrenosum 的病因很复杂，涉及有遗传倾向的人的先天免疫和适应性免疫问题。最近，研究人员将毛囊作为该疾病的潜在起点。
Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses and clinically characterized by painful, rapidly evolving cutaneous ulcers with undermined, irregular, erythematous-violaceous edges. Pyoderma gangrenosum pathogenesis is complex and involves a profound dysregulation of components of both innate and adaptive immunity in genetically predisposed individuals, with the follicular unit increasingly recognized as the putative initial target.
Pyoderma gangrenosum is a rare inflammatory skin disease classified within the group of neutrophilic dermatoses and clinically characterized by painful, rapidly evolving cutaneous ulcers with undermined, irregular, erythematous-violaceous edges. Pyoderma gangrenosum pathogenesis is complex and involves a profound dysregulation of components of both innate and adaptive immunity in genetically predisposed individuals, with the follicular unit increasingly recognized as the putative initial target.
Pyoderma gangrenosum 是一种罕见的皮肤病，会导致极其疼痛的溃疡。虽然我们不完全了解其原因，但我们知道它涉及某些免疫细胞活性的增加。治疗这种疾病仍然不容易。我们有多种药物可以抑制免疫系统或改变其活性。除此之外，我们还专注于治疗伤口和控制疼痛。皮质类固醇和环孢素通常是治疗的首选，但最近，有更多关于使用 TNF-α 抑制剂等生物疗法的研究。这些生物制剂越来越受欢迎，特别是对于患有其他炎症性疾病的患者，并且它们在疾病过程的早期使用。
Pyoderma gangrenosum is a rare neutrophilic dermatosis that leads to exceedingly painful ulcerations of the skin. Although the exact pathogenesis is not yet fully understood, various auto-inflammatory phenomena with increased neutrophil granulocyte activity have been demonstrated. Despite the limited understanding of the pathogenesis, it is no longer a diagnosis of exclusion, as it can now be made on the basis of validated scoring systems. However, therapy remains a major multidisciplinary challenge. Various immunosuppressive and immunomodulatory therapies are available for the treatment of affected patients. In addition, concomitant topical pharmacologic therapy, wound management and pain control should always be addressed. Corticosteroids and/or cyclosporine remain the systemic therapeutics of choice for most patients. However, in recent years, there has been an increasing number of studies on the positive effects of biologic therapies such as inhibitors of tumour necrosis factor-α; interleukin-1, interleukin-17, interleukin-23 or complement factor C5a. Biologics have now become the drug of choice in certain scenarios, particularly in patients with underlying inflammatory comorbidities, and are increasingly used at an early stage in the disease rather than in therapy refractory patients.
Pyoderma gangrenosum is a rare neutrophilic dermatosis that leads to exceedingly painful ulcerations of the skin. Although the exact pathogenesis is not yet fully understood, various auto-inflammatory phenomena with increased neutrophil granulocyte activity have been demonstrated. Despite the limited understanding of the pathogenesis, it is no longer a diagnosis of exclusion, as it can now be made on the basis of validated scoring systems. However, therapy remains a major multidisciplinary challenge. Various immunosuppressive and immunomodulatory therapies are available for the treatment of affected patients. In addition, concomitant topical pharmacologic therapy, wound management and pain control should always be addressed. Corticosteroids and/or cyclosporine remain the systemic therapeutics of choice for most patients. However, in recent years, there has been an increasing number of studies on the positive effects of biologic therapies such as inhibitors of tumour necrosis factor-α; interleukin-1, interleukin-17, interleukin-23 or complement factor C5a. Biologics have now become the drug of choice in certain scenarios, particularly in patients with underlying inflammatory comorbidities, and are increasingly used at an early stage in the disease rather than in therapy refractory patients.
