epidermolysis bullosa acquisita
Conditions
Interventions
Four intravenous infusion of 375mg/m2 of rutuximab will be given at weekly intervals.
Sponsors
Department of Dermatology, Kawasaki Medical School
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Patients with recalcitrant epidermolysis bullosa acquisita who meet the following criteria; 1) resistant to combined therapy with prednisolone more than 40mg/d and immuno-suppressant(cyclophosphamide, azathioprine, cyclosporine) 2) resistant to combined therapy with prednisolone more than 40mg/d and plasma exchange or intravenous immunoglobulins. 3) patients with severe side effects due to long-term treatment with steroid. 4) patients who cannot tolerate treatment with high-dose steroid due to complications including diabetes mellitus. Patients who submitted a signed informed consent are eligible to the trial.
Exclusion criteria
Exclusion criteria: Patients with severe infection, type B hepatitis and severe hematological diseases will be excluded.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| We evaluate the disease activity and therapeutic response according to the pemphigus disease area index (PDAI) every one month after the end of the treatment. | — |
Secondary
| Measure | Time frame |
|---|---|
| We examine peripheral blood T/B cell subset(CD3+CD4+ T cells, CD3+CD8+ T cells, CD19+B cells), immunoglobulins(IgG, IgA, IgM), anti-type VII collagen every one month after the end of the treatment. | — |
Countries
Japan
Contacts
Public ContactWataru Fujimoto
Kawasaki Medical School Department of Dermatology
Outcome results
None listed