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疾病，表现为疼痛的脓疱或结节逐渐演变成溃疡。该疾病不具传染性。治疗方案可能包括皮质类固醇、环孢素或多种单克隆抗体。虽然任何年龄段均可受累，但主要发生在40至50岁人群。
溃疡性结肠炎患者的腿上。
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问题。最近，研究人员认为毛囊是该疾病的潜在起点。
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，可导致极度疼痛的溃疡。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，常用药物旨在抑制或调节免疫系统。此外，还需同时进行伤口护理和疼痛管理。皮质类固醇和环孢素通常是首选治疗方案，近年来，关于使用 TNF-α 抑制剂等生物制剂的研究逐渐增多。这类生物制剂在患有其他炎症性疾病的患者中越来越受欢迎，且常在疾病早期使用。
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.
