Background: Rituximab, a monoclonal antibody against CD20, is widely used in autoimmune blistering diseases. However, the therapeutic value of rituximab in linear IgA dermatosis (LAD) is uncertain because there are only a few case reports on successful use of rituximab in recalcitrant LAD.
Objectives: To evaluate the efficacy of rituximab for the treatment of recalcitrant LAD.
Methods: Three patients were diagnosed as LAD based on the clinical, histological, and immunofluorescence studies. All patients had been refractory to high-dose systemic corticosteroid (16~24 mg/day of methylprednisolone) with other conventional agents over the past 2 months. Patient #1 and #2 were treated with two 1000 mg infusions of rituximab separated by 2 weeks, and patient #3 only received 1st infusion due to pneumonia during the rituximab treatment.
Results: Follow-up periods were 6 months in patient #1, 4 months in patient #2, and 11 months in patient #3. Remission with minimal therapy (8mg/day of methylprednisolone) was achieved 2 months after rituximab therapy in patient #1 and #2, and 5 months after rituximab therapy in patient #3. Blister formation did not appear during 6-month and 3-month follow-up period in patient #1 and #2, respectively. Patient #3 experienced relapse 10 months after rituximab therapy.
Conclusion: Rituximab should be considered as a feasible treatment in severe cases of LAD refractory to conventional treatments.