Treatment of bullous pemphigoid with rituximab: critical analysis of the current literature.

The objective of this review was to critically analyze the currently available literature on the use of rituximab to treat patients with bullous pemphigoid (BP). The focus was to highlight clinical outcomes, treatment protocols, and adverse effects. A PubMed search from 2000 to the present day was conducted, using "bullous pemphigoid" and "rituximab" as keywords. Inclusion criteria were a description of the clinical disease, histology and immunopathology typical of BP, the use of at least 1 infusion of rituximab, and the availability of follow-up after rituximab treatment. Sixteen patients (12 adults and 4 children) were identified. Fourteen patients were treated according to the Lymphoma Protocol and 2 patients according to the Rheumatoid Arthritis Protocol. In the final clinical outcome after treatment with rituximab, 11 out of 16 (69%) had Complete Response, 1 (6%) had Partial Response, 1 (6%) had No Response, and 3 (19%) died. Two died of sepsis, including 1 child, and 1 died from cardiac effects. Three (20%) had serious infections. More than 1 cycle of rituximab was required in 38% of the patients who achieved Complete Response. The mean follow-up period was 15.6 months (range 1-36), which is a serious limitation of the available data. Rituximab is a useful option for BP patients who are recalcitrant to conventional therapy. A specific protocol for the use of rituximab to treat BP patients is not yet available. Since infection and mortality rates are of concern, careful and close monitoring may be necessary.