Pyoderma gangrenosum - 壞疽性膿皮症
壞疽性膿皮症 (Pyoderma gangrenosum) 是一種罕見的發炎性皮膚病，其中疼痛的膿皰或結節變成逐漸生長的潰瘍。 壞疽性膿皮症 (pyoderma gangrenosum) 不具傳染性。治療方法可能包括皮質類固醇、環孢素或各種單株抗體。雖然它可以影響任何年齡層的人，但主要影響 40 多歲和 50 多歲的人。
潰瘍性結腸炎患者的腿上。
References
Pyoderma gangrenosum 是一種罕見的皮膚病，會導致邊緣呈現紅色或紫色的疼痛性潰瘍。它被歸類為一種發炎性疾病，屬於嗜中性球性皮膚病的一部分。 pyoderma gangrenosum 的病因很複雜，涉及有遺傳傾向的人的先天免疫和適應性免疫問題。最近，研究人員將毛囊作為疾病的潛在起點。
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 是一種罕見的皮膚病，會導致極度疼痛的潰瘍。雖然我們不完全了解原因，但我們知道它涉及某些免疫細胞活性的增加。治療這種疾病仍然不容易。我們有多種藥物可以抑制免疫系統或改變其活性。除此之外，我們也專注於治療傷口和控制疼痛。皮質類固醇和環孢素通常是治療的首選，但最近，有更多關於使用 TNF-α 抑制劑等生物療法的研究。這些生物製劑越來越受歡迎，特別是對於患有其他發炎性疾病的患者，並且它們在疾病過程的早期使用。
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.
