Pemphigus Vulgaris Medication
- Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD more...
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.[47, 48] 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.[49] A retrospective chart review has suggested a therapeutic ladder for patients with pemphigus vulgaris, but these authors' approach has not been validated.[50] 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.[51]
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.[52]
Cyclophosphamide is used for refractory disease. 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.
Anti-inflammatory agents
Class Summary
Inhibit the inflammatory process by inhibiting specific cytokine production.
Prednisone (Deltasone, Meticorten, Orasone, Sterapred)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. For treating pemphigus vulgaris, administered PO and used 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.
Immunosuppressive agents
Class Summary
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.[53, 54, 55] Also see the clinical guideline summary from the British Association of Dermatologists, Guidelines for prescribing azathioprine in dermatology.[56]
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. In conjunction with prednisone, more effective than prednisone alone. May be an effective monotherapy in mild cases, although therapeutic effect is delayed 3-5 wk.
Consider withdrawal if no improvement within 3 mo.
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