Efficacy of rituximab in the treatment of epidermolysis bullosa acquisita.

BACKGROUND

Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous disease that is mediated by antibodies that bind collagen VII. The treatment of EBA can be challenging and the use of multiple immunomodulatory drugs is often required. Rituximab has been reported to be an effective treatment for recalcitrant EBA, although the evidence base for this treatment is limited to case reports and case series.

OBJECTIVES

This study aimed to evaluate the efficacy of rituximab for the treatment of patients with EBA.

METHODS

Patients with EBA who were treated with rituximab were identified by searching electronic medical records. The diagnostic criteria for EBA were mechanobullous skin lesions and/or mucosal ulceration, indirect immunofluorescence localizing to the base of salt-split skin, and positive collagen VII antibodies. Clinical disease activity, collagen VII antibody levels and serum immunoglobulin levels were recorded at each follow-up visit over a 600-day period. Treatment responses were classified as follows: complete remission (CR) was defined as the absence of new or established lesions on minimal therapy for 2 months, partial remission (PR) was defined as transient lesions that heal within 1 week on minimal therapy, and active disease was defined as the development of new lesions.

RESULTS

In total, 14 patients with EBA were treated with rituximab. CR or PR was observed in 11 patients, and the duration of response varied between 4 and 24 months. A reduction in collagen VII antibody levels was observed in all patients. No significant adverse events were reported.

CONCLUSIONS

Rituximab is a safe and effective treatment for patients with recalcitrant EBA, although there was significant heterogeneity in the disease response.