Dissemond Joachim, Moelleken Maurice, Tasdogan Alpaslan
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Essen, Hufelandstr. 55, 45122, Essen, Deutschland.
Dermatologie (Heidelb). 2025 Jul;76(7):449-458. doi: 10.1007/s00105-025-05522-z. Epub 2025 Jun 23.
Pyoderma gangrenosum (PG) is a skin disease that rapidly develops from papules or primarily sterile pustules. It is characterized by the formation of extremely painful wounds with a reddish-livid edge and surrounding areas. It can be triggered by trauma, bacteria or drugs in genetically predisposed people. In 70% of cases, the predilection sites manifest at the extensor sides of the lower extremities. Although the exact pathophysiology of this rare neutrophilic dermatosis has not yet been fully elucidated, an autoinflammatory genesis with increased neutrophil granulocyte activity is currently being discussed. Patients often have inflammatory comorbidities such as chronic inflammatory bowel disease or cancer. To confirm the diagnosis, the validated PARACELSUS score, which summarizes the nonspecific criteria, should be used. In addition to the administration of immunosuppressive drugs such as prednisolone and/or ciclosporin, patients with proven PG are treated systemically with biologics, small molecules, or intravenous immunoglobulins. Utilization of these pharmaceutical agents has seen a marked increase in recent cases of PG. This should be accompanied by topical pharmacological treatment of the wound surroundings as well as modern wound and pain therapy.
坏疽性脓皮病(PG)是一种由丘疹或原发性无菌脓疱迅速发展而来的皮肤病。其特征是形成具有红紫色边缘和周围区域的极其疼痛的伤口。在有遗传易感性的人群中,它可由创伤、细菌或药物引发。在70%的病例中,好发部位出现在下肢伸侧。尽管这种罕见的嗜中性皮病的确切病理生理学尚未完全阐明,但目前正在讨论嗜中性粒细胞活性增加的自身炎症性发病机制。患者常伴有炎症性合并症,如慢性炎症性肠病或癌症。为确诊,应使用总结非特异性标准的经过验证的帕拉塞尔苏斯评分。除了使用泼尼松龙和/或环孢素等免疫抑制药物外,确诊为PG的患者还需使用生物制剂、小分子药物或静脉注射免疫球蛋白进行全身治疗。在近期的PG病例中,这些药物的使用显著增加。这应辅以伤口周围的局部药物治疗以及现代伤口和疼痛治疗。