Pemphigus Vulgaris Medication

Updated: Jun 14, 2018
  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The aim of treatment is to reduce the inflammatory response and autoantibody production. While target-specific therapy is not available, non–target-specific treatments currently are used. The most commonly used medications are corticosteroids.

The introduction of corticosteroids has reduced mortality greatly, but significant morbidity remains. Immunosuppressants should be considered early in the course of disease, as steroid-sparing agents. Mycophenolate mofetil and azathioprine are the usual agents considered as initial choices. [65, 66] Some suggest measuring levels of thiopurine methyltransferase (TPMT), a key enzyme in azathioprine metabolism, before starting patients on azathioprine. [67, 68, 69] Only one placebo-controlled blinded study of mycophenolate has demonstrated more rapid improvement in the short run but no significant steroid-sparing effects in the long term. [70] A retrospective chart review has suggested a therapeutic ladder for patients with pemphigus vulgaris, but these authors' approach has not been validated. [71] Rituximab and intravenous immunoglobulin have also proven useful alone or in combination, and some authorities are now using rituximab as first-line therapy for severe disease. [72] Rituximab was approved by the FDA for the treatment of pemphigus vulgaris in June 2018. [75]

A small case control trial showed improved laboratory and clinical outcomes in patients with pemphigus vulgaris treated with a combination of cytotoxic agents plus intravenous immunoglobulin (IVIG)compared with those treated with just IVIG. [73]

Cyclophosphamide is used for refractory disease. [74] The role of biologic agents is being investigated. Each of these agents should be prescribed and monitored by physicians familiar with them. Wound care for erosions includes daily gentle cleaning, application of topical agents to promote wound healing, and use of nonadhesive dressings. The goal of wound care is to promote healing, minimize trauma to the surrounding skin, and diminish scarring.

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Anti-CD20 Monoclonal Antibodies

Class Summary

Monoclonal antibodies target the CD20 antigen expressed on the surface of pre-B and mature B-lymphocytes. B cells may be acting at multiple sites in the autoimmune/inflammatory process, including T-cell activation and/or proinflammatory cytokine production.

Rituximab (Rituxan)

Rituximab is an anti-CD20 monoclonal antibody indicated for adults with pemphigus vulgaris in combination with short-term corticosteroid therapy.

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Anti-inflammatory agents

Class Summary

Anti-inflammatory agents inhibit the inflammatory process by inhibiting specific cytokine production.

Prednisone (Deltasone, Meticorten, Orasone, Sterapred)

Prednisone may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. It stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. For treating pemphigus vulgaris, administer it orally and use alone or in combination with topical or intralesional steroids or in conjunction with other immunosuppressives.

In pediatric patients, disease management with this medication in consultation with the patient's pediatrician is advised.

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Immunosuppressive agents

Class Summary

Immunosuppressive agents are useful adjuvants in patients with pemphigus vulgaris with generalized disease unresponsive to steroids and/or other anti-inflammatory agents or in patients unable to tolerate prednisone. Some suggest measuring levels of thiopurine methyltransferase (TPMT), a key enzyme in azathioprine metabolism, before starting patients on azathioprine.

Azathioprine (Imuran)

Azathioprine antagonizes purine metabolism and inhibits the synthesis of DNA, RNA, and proteins. It may decrease the proliferation of immune cells, which results in lower autoimmune activity. In conjunction with prednisone, it is more effective than prednisone alone. OFf-label use of azathioprine may be an effective monotherapy in mild cases, although therapeutic effects are delayed 3-5 weeks. Consider withdrawal if no improvement is seen within 3 months.

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