Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation
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 diseas...
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| Format: | Article |
| Language: | English |
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Adis, Springer Healthcare
2025-01-01
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| Series: | Dermatology and Therapy |
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| Online Access: | https://doi.org/10.1007/s13555-024-01326-6 |
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| author | Katharina Hogrefe Julia K. Winkler Alexander H. Enk |
| author_facet | Katharina Hogrefe Julia K. Winkler Alexander H. Enk |
| author_sort | Katharina Hogrefe |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-35a76eadb6bd432a9865d8a02ab80b7f |
| institution | Kabale University |
| issn | 2193-8210 2190-9172 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Adis, Springer Healthcare |
| record_format | Article |
| series | Dermatology and Therapy |
| spelling | doaj-art-35a76eadb6bd432a9865d8a02ab80b7f2025-02-02T12:09:44ZengAdis, Springer HealthcareDermatology and Therapy2193-82102190-91722025-01-0115123724410.1007/s13555-024-01326-6Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg PreparationKatharina Hogrefe0Julia K. Winkler1Alexander H. Enk2Department of Dermatology, University Hospital HeidelbergDepartment of Dermatology, University Hospital HeidelbergDepartment of Dermatology, University Hospital HeidelbergAbstract 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.https://doi.org/10.1007/s13555-024-01326-6Pemphigus vulgarisIVIgImmunosuppressionParaneoplastic |
| spellingShingle | Katharina Hogrefe Julia K. Winkler Alexander H. Enk Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation Dermatology and Therapy Pemphigus vulgaris IVIg Immunosuppression Paraneoplastic |
| title | Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation |
| title_full | Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation |
| title_fullStr | Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation |
| title_full_unstemmed | Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation |
| title_short | Two Patients with Therapy-Resistant Pemphigus Vulgaris and Severe Underlying Disease Showing Good Response to a New IVIg Preparation |
| title_sort | two patients with therapy resistant pemphigus vulgaris and severe underlying disease showing good response to a new ivig preparation |
| topic | Pemphigus vulgaris IVIg Immunosuppression Paraneoplastic |
| url | https://doi.org/10.1007/s13555-024-01326-6 |
| work_keys_str_mv | AT katharinahogrefe twopatientswiththerapyresistantpemphigusvulgarisandsevereunderlyingdiseaseshowinggoodresponsetoanewivigpreparation AT juliakwinkler twopatientswiththerapyresistantpemphigusvulgarisandsevereunderlyingdiseaseshowinggoodresponsetoanewivigpreparation AT alexanderhenk twopatientswiththerapyresistantpemphigusvulgarisandsevereunderlyingdiseaseshowinggoodresponsetoanewivigpreparation |