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，疼痛的膿皰或結節會逐漸演變成潰瘍。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。雖然此病可影響任何年齡層，但主要發生於40至50歲的成人。
潰瘍性結腸炎患者的腿部。
relevance score : -100.0%
ReferencesPyoderma gangrenosum 是一種罕見的皮膚病，會形成邊緣呈紅色或紫色、伴有疼痛的潰瘍。它屬於發炎性疾病，屬於嗜中性球性皮膚病的一類。Pyoderma gangrenosum 的病因相當複雜，涉及具有遺傳傾向者的先天免疫與適應性免疫異常。最近的研究指出，毛囊可能是此疾病的潛在起點。
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其原因，但已知它與某些免疫細胞活性的增加有關。治療此疾病仍具挑戰性，我們可使用多種藥物抑制免疫系統或調節其活性。除了藥物治療，我們也會同時處理傷口並控制疼痛。皮質類固醇和環孢素通常是首選治療藥物，近年來，使用 TNF-α 抑制劑等生物製劑的研究逐漸增多。這類生物製劑在患有其他發炎性疾病的患者中越來越受歡迎，且常於疾病早期即開始使用。
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.
