The clinical, immunological and pathological features for relapse of pemphigus herpetiformis: a univariate analysis of 26 cases

Wang, Y.M.; Mao, Xuming; Zhao, Wenling; Wang, Y.H.; Zuo, Ya‐Gang; Jin, Hongzhong; Li, L.

doi:10.1111/bjd.18518

Cited by 2 publications

(1 citation statement)

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“…Serum levels of pemphigus antibodies (including Dsg-1 and Dsg-3) were measured on admission and periodically during the treatment by IIF according to the standard protocol of dermatologic pathology laboratory as reported previously. 24 The basic procedure for indirect immunofluorescence in our hospital is as follows 25 : Blood is drawn from the patients and centrifuged to separate serum. The selected substrate (monkey esophagus in our hospital) is incubated with progressive dilutions of patient serum.…”

Section: Antibodies Measurementmentioning

confidence: 99%

Double‐filtration plasmapheresis combined with immunosuppressive treatment for severe pemphigus: 10 years' experience of a single center in China

Liang

Zhang

et al. 2020

J of Clinical Apheresis

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Background: Pemphigus is a group of rare and severe autoimmune blistering disease mediated by pathogenic autoantibodies against desmogleins. Plasmapheresis can directly remove autoantibodies from circulation, which has been applied to the treatment of pemphigus as an adjuvant therapy. But the results of the researches are controversial. This study aims to evaluate the efficacy and safety of double filtration plasmapheresis (DFPP) combined with immunosuppressive treatment for patients with severe pemphigus in our single center. Methods: We retrospectively analyzed 17 patients with severe pemphigus who were unresponsive to high-dose corticosteroid and received DFPP treatment between January 2010 and January 2020. The information on demographic characteristics, clinical and laboratory data, treatment regimens, and clinical outcomes were collected. Results: All the patients were diagnosed as severe pemphigus and had a period of at least 1 week of high-dose prednisone (1-1.5 mg/kg/day), but they were unresponsive to corticosteroid and immunosuppressants treatment. They received DFPP treatment as an adjuvant therapy. After DFPP treatment, the titers of desmogleins antibodies significantly decreased (P < .001), Nikolsky's sign became negative and no new blisters appeared. The dosage of corticosteroid could begin to taper down rapidly in 9 ± 4 days. On discharge, the dosage of prednisone decreased significantly (51 ± 3 mg/day, P < .001). No major adverse events happened that could lead to the termination of DFPP treatment. Conclusion: Double filtration plasmapheresis combined with immunosuppressive treatment is an effective and safe therapeutic regimen for severe pemphigus. DFPP can also contribute to the dosage reduction of steroid to avoid more drug-related side effect.

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Section: Antibodies Measurementmentioning

confidence: 99%