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会逐渐演变成溃疡。坏疽性脓皮病 (pyoderma gangrenosum) 不具传染性。治疗方法可能包括皮质类固醇、环孢素或各种单克隆抗体。虽然可影响任何年龄段的人，但主要见于40至50岁的人群。
溃疡性结肠炎患者的腿部。
relevance score : -100.0%
ReferencesPyoderma 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 的病因十分复杂，涉及具有遗传倾向者的先天免疫和适应性免疫异常。最近，研究人员将毛囊视为该疾病的潜在起始部位。
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。虽然其确切原因尚不完全清楚，但已知与某些免疫细胞活性增强有关。治疗该疾病仍具挑战性。我们可使用多种药物抑制免疫系统或调节其活性，同时还需关注伤口护理和疼痛控制。皮质类固醇和环孢素通常是首选治疗药物，近年来，关于使用 TNF-α 抑制剂等生物制剂的研究日益增多。这类生物制剂在伴有其他炎症性疾病的患者中尤其受欢迎，并且在疾病早期使用效果更佳。
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.
