Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation

两名患有难治性寻常型天疱疮和严重基础疾病的患者对一种新型静脉注射免疫球蛋白制剂反应良好。

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Abstract

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.

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