Pyoderma gangrenosum - 괴저 고름 피부증
괴저 고름 피부증 (Pyoderma gangrenosum) 은 통증이 있는 농포나 결절이 점차 커지는 궤양으로 변하는 드문 염증성 피부질환입니다. 괴저 고름 피부증 (pyoderma gangrenosum) 은 전염성이 없습니다. 치료에는 코르티코스테로이드, 시클로스포린 또는 다양한 단클론 항체가 포함될 수 있습니다. 모든 연령층에 영향을 미칠 수 있지만 주로 40~50대에 영향을 미칩니다.
궤양성 대장염 환자의 다리.
References
Pyoderma gangrenosum 은 가장자리가 붉거나 보라색을 띠는 고통스러운 궤양을 유발하는 드문 피부 질환입니다. 이는 염증성 질환으로 분류되며 호중구성 피부병이라는 그룹의 일부입니다. Pyoderma gangrenosum 의 원인은 복잡하며 유전적으로 취약한 사람들의 선천성 면역과 적응성 면역 모두에 문제가 있습니다. 최근 연구자들은 이 질병의 잠재적인 시작점으로 모낭에 초점을 맞췄습니다.
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 은 극도로 고통스러운 궤양을 일으키는 드문 피부 질환입니다. 그 원인을 완전히 이해하지는 못하지만 특정 면역 세포의 활동 증가와 관련이 있다는 것을 알고 있습니다. 질병을 치료하는 것은 아직 쉽지 않습니다. 우리는 면역 체계를 억제하거나 그 활동을 조절하는 다양한 약물을 보유하고 있습니다. 이와 함께 상처 치료와 통증 관리에도 중점을 두고 있습니다. 코르티코스테로이드와 사이클로스포린이 치료를 위한 첫 번째 선택인 경우가 많지만, 최근에는 TNF-α 억제제와 같은 생물학적 치료법을 사용하는 것에 대한 연구가 더 많이 이루어지고 있습니다. 이러한 생물학적 제제는 특히 다른 염증성 질환이 있는 환자에게 점점 더 선호되고 있으며 질병 진행 초기에 사용되고 있습니다.
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.
