Therapeutic ladder for pemphigus vulgaris: Emphasis on achieving complete remission

Background

Pemphigus vulgaris (PV) is a blistering autoimmune bullous disease that is usually fatal without proper treatment. There are no clear treatment guidelines for PV at this time.

Purpose

We suggest a standard treatment regimen for patients with PV based on the success of our treatment.

Methods

A retrospective chart review of 18 patients with PV was conducted to assess response to a similar approach using mycophenolate mofetil (MMF) and prednisone. Diagnosis was confirmed through routine histology, direct immunofluorescence, and indirect immunofluorescence, and patients were followed up for a total average of 35.2 months.

Results

We achieved complete disease control in 89% of patients using our treatment algorithm. Fourteen of 18 patients achieved complete disease control on therapy with prednisone and MMF. Three of the 4 patients who did not achieve control on MMF and prednisone went on to receive rituximab therapy, and two of those patients achieved disease control on rituximab. The average length of time from initiating therapy to 75% clearance of lesions was 4.5 months. Three of 18 patients were able to discontinue therapy after an average of 3 years and have remained in complete remission for more than 1 year.

Limitations

This was a retrospective chart review with a small patient sample size.

Conclusions

The combination therapy of MMF and prednisone is an effective treatment regimen to achieve rapid and complete control of PV. For those patients who fail treatment with MMF and prednisone, rituximab is an efficacious alternative therapy.

Key words: blistering disease, mycophenolate mofetil, rituximab

Abbreviations used: DIF, direct immunofluorescence, IIF, indirect immunofluorescence, IVIG, intravenous immunoglobulin, MMF, mycophenolate mofetil, PV, pemphigus vulgaris