Pyoderma gangrenosum - 壊疽性膿皮症
壊疽性膿皮症 (Pyoderma gangrenosum) は、痛みを伴う膿疱や結節が潰瘍となり、徐々に成長する稀な炎症性皮膚疾患です。 壊疽性膿皮症 (pyoderma gangrenosum) は感染性はありません。治療には、コルチコステロイド、シクロスポリン、またはさまざまなモノクローナル抗体が含まれる場合があります。あらゆる年齢層に影響を与える可能性がありますが、主に 40 代と 50 代の人々に影響を与えます。
潰瘍性大腸炎の人の脚。
References
Pyoderma gangrenosum は、赤または紫がかった縁を伴う痛みを伴う潰瘍を引き起こす、まれな皮膚疾患です。これは炎症性疾患として分類され、好中球性皮膚炎と呼ばれるグループの一部です。 pyoderma gangrenosum の原因は複雑で、遺伝的にその傾向がある人々の自然免疫と適応免疫の両方の問題が関係しています。最近、研究者らは病気の潜在的な起点として毛包に注目しています。
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 は、非常に痛みを伴う潰瘍を引き起こす稀な皮膚疾患です。その原因は完全には理解されていませんが、特定の免疫細胞の活性の増加が関与していることはわかっています。この病気の治療はまだ簡単ではありません。免疫システムを抑制したり、その活性を変更したりするさまざまな薬があります。当院では、傷の治療や痛みの緩和にも力を入れております。多くの場合、コルチコステロイドとシクロスポリンが治療の第一選択となりますが、最近では、TNF-α阻害剤などの生物学的療法を使用する研究が増えています。これらの生物学的製剤は、特に他の炎症状態の患者においてますます好まれており、病気の進行の早い段階で使用されています。
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.
