Hogrefe Katharina, Winkler Julia K, Enk Alexander H
Department of Dermatology, University Hospital Heidelberg, Im Neuenheimer Feld 440, 69120, Heidelberg, Germany.
Dermatol Ther (Heidelb). 2025 Jan;15(1):237-244. doi: 10.1007/s13555-024-01326-6. Epub 2025 Jan 3.
Pemphigus vulgaris is a severe and often therapy-resistant bullous autoimmune disease. Standard therapy with steroids often administered together with another immunosuppressant does not respond in all patients or may not be a good therapeutic option in patients with severe underlying diseases. Intravenous immunoglobulins (IVIgs) represent a treatment alternative, often showing a rapid response which allows one to reduce concomitant immunosuppression. Here, we report on two patients with a complex disease history suffering from severe pemphigus vulgaris who received treatment with a new IVIg preparation (Yimmugo, 2 g per kg body weight every 4 weeks). IVIg preparations differ regarding manufacturing process and show a varying side effect profile. Both of our patients did not experience any side effects from IVIgs and showed a significant improvement of skin and mucosal erosions. More reports on IVIgs are desirable to help in selecting the optimal preparation and dosing regarding tolerability and effectiveness for individual patients.
寻常型天疱疮是一种严重且通常对治疗有抵抗性的大疱性自身免疫性疾病。使用类固醇并常与另一种免疫抑制剂联合的标准疗法并非对所有患者都有效，或者对于有严重基础疾病的患者可能不是一个好的治疗选择。静脉注射免疫球蛋白（IVIg）是一种治疗选择，通常显示出快速反应，从而可以减少同时使用的免疫抑制药物剂量。在此，我们报告两名患有复杂病史的严重寻常型天疱疮患者，他们接受了一种新的IVIg制剂（Yimmugo，每4周每千克体重2克）的治疗。IVIg制剂在生产工艺上有所不同，并且副作用情况也各不相同。我们的两名患者均未出现IVIg的任何副作用，皮肤和黏膜糜烂有显著改善。需要更多关于IVIg的报告，以帮助针对个体患者的耐受性和有效性选择最佳制剂和剂量。
