Ronicke Moritz, Sollfrank Lukas, Vitus Martin V, Walter Lukas J, Krieter Manuel, Moelleken Maurice, Dissemond Joachim, Schultz Erwin, Lauffer Felix, von den Driesch Peter, Erfurt-Berge Cornelia
Department of Dermatology, Uniklinikum Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Ulmenweg 18, 91054, Erlangen, Germany.
Deutsches Zentrum Immuntherapie (DZI), Uniklinikum, Erlangen, Germany.
Am J Clin Dermatol. 2025 Jan;26(1):139-146. doi: 10.1007/s40257-024-00904-w. Epub 2024 Nov 14.
Pyoderma gangrenosum (PG) is rare neutrophil skin disease causing painful, progressively enlarging ulcers. Among the treatment options, intravenous immunoglobulin (IVIG) is a therapy of first choice for paraneoplastic PG. Otherwise, it is used in therapy-refractory courses.
To assess the efficacy and safety of IVIG therapy in patients with PG.
A retrospective chart review for patients in five dermatologic wound centres in Germany was performed.
Overall, 81 patients were included. IVIG was used as adjunct therapy with (methyl-) prednisolone and/or a steroid sparing therapy in 77 (95.1%) cases. Response to treatment (combined complete and partial, defined as tendency to heal and cessation of lesion progression, respectively) was 49.3% 1 month after initiation of IVIG. In total 18.8% had a complete response after 6 months. Statistically significantly higher response rates were observed in patients with diabetes mellitus and thyroid disease [odds ratio (OR) 3.49, confidence interval (CI) 1.13-10.80 and OR 6.64, CI 1.01-43.57, respectively]. Patients with solid malignancy tended to have better response (OR 4.36, CI 0.79-23.91). A higher IVIG dose was also associated with a tendency towards better response rates (OR 2.70, CI 0.84-8.63). In total, 1 (1.2%) severe adverse event (myocardial infarction with consequent death) was observed as well as three moderate adverse events, with two thromboembolic events (2.5%) and one acute kidney injury (1.2%). Other adverse events were mild or unlikely to be associated with IVIG therapy, with 14 events in 10 patients overall (12.3%).
This multicentre retrospective study shows the important role of adjunctive IVIG therapy in patients with PG with recalcitrant courses. Identifying subgroups with a higher probability of response could improve future response rates and save patients from ineffective treatment and potential adverse events.
坏疽性脓皮病（PG）是一种罕见的中性粒细胞性皮肤病，可导致疼痛且逐渐扩大的溃疡。在治疗方案中，静脉注射免疫球蛋白（IVIG）是副肿瘤性PG的首选治疗方法。否则，它用于治疗难治性病程。
评估IVIG治疗PG患者的疗效和安全性。
对德国五个皮肤科伤口中心的患者进行回顾性病历审查。
总共纳入了81例患者。在77例（95.1%）病例中，IVIG与（甲基）泼尼松龙和/或类固醇节省疗法联合用作辅助治疗。IVIG开始治疗1个月后，治疗反应（联合完全缓解和部分缓解，分别定义为愈合趋势和病变进展停止）为49.3%。6个月后，共有18.8%的患者完全缓解。糖尿病和甲状腺疾病患者的反应率在统计学上显著更高[优势比（OR）分别为3.49，置信区间（CI）为1.13 - 10.80；OR为6.64，CI为1.01 - 43.57]。实体恶性肿瘤患者往往有更好的反应（OR为4.36，CI为0.79 - 23.91）。较高的IVIG剂量也与更好的反应率趋势相关（OR为2.70，CI为0.84 - 8.63）。总共观察到1例（1.2%）严重不良事件（心肌梗死并导致死亡）以及3例中度不良事件，其中2例血栓栓塞事件（2.5%）和1例急性肾损伤（1.2%）。其他不良事件为轻度或不太可能与IVIG治疗相关，总共10例患者出现14例事件（12.3%）。
这项多中心回顾性研究表明辅助IVIG治疗在病程顽固的PG患者中的重要作用。识别反应可能性较高的亚组可以提高未来的反应率，并使患者避免无效治疗和潜在的不良事件。
