Pemphigus Foliaceus Medication

Updated: Mar 30, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

Numerous medications are used to treat patients with pemphigus foliaceus. They are often used in combination. However, a systematic review and meta-analysis according to the methodology of the Cochrane Collaboration showed that available evidence is inadequate at present to ascertain the optimal therapy for pemphigus foliaceus. [59]

Pemphigus is usually treated with systemic prednisone in combination with adjuvant immunosuppressants. [60] Refractory pemphigus foliaceus has been treated with the anti-CD20 monoclonal antibody rituximab. [61, 62, 63] Unfortunately, it increases the risk of infection with Pneumocystis jiroveci pneumonia, which may result in a fatal outcome. [64] Intravenous immunoglobulin (IVIg) may also be used because it lowers serum levels of pemphigus antibodies. [65, 66] Concurrent administration of a cytotoxic drug appears to be beneficial. Mycophenolate mofetil (MMF) and enteric-coated mycophenolate sodium may be considered. [67]

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Corticosteroid agents

Class Summary

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.

Prednisone (Deltasone, Orasone)

Prednisone is a synthetic adrenocortical steroid with predominantly glucocorticoid properties. It is an immunosuppressant for the treatment of autoimmune disorders; it may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Prednisone stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.

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Antibiotic agents

Class Summary

Empiric antimicrobial therapy must be comprehensive and should cover all likely pathogens in the context of the clinical setting.

Minocycline (Dynacin, Minocin)

Minocycline is a semisynthetic derivative of tetracycline. It treats infections caused by susceptible gram-negative and gram-positive organisms, in addition to infections caused by susceptible Chlamydia, Rickettsia, and Mycoplasma species. Minocycline was found to be effective in some nontuberculotic mycobacterial infections.

Dapsone (Avlosulfon)

Dapsone is bactericidal and bacteriostatic against mycobacteria; its mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Dapsone is used to control the dermatologic symptoms of dermatitis herpetiformis. It can be used for patients with pemphigus and may be the drug of choice for pemphigus foliaceus and IgA pemphigus foliaceus. It may be provided as monotherapy or in combination with systemic steroids and immunosuppressants.

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Antimalarial agents

Class Summary

Hydroxychloroquine has immunosuppressive effects.

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine is a 4-aminoquinoline derivative active against a variety of autoimmune disorders. It inhibits chemotaxis of eosinophils, locomotion of neutrophils, and impairs complement-dependent antigen-antibody reactions. Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

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Immunomodulatory agents

Class Summary

These agents have antiproliferative and immunosuppressive effects.

Azathioprine (Imuran)

Azathioprine may be used alone or as steroid-sparing agent. It antagonizes purine metabolism and inhibits the synthesis of DNA, RNA, and proteins. Azathioprine may decrease the proliferation of immune cells, which results in lower autoimmune activity.

Cyclophosphamide (Cytoxan, Neosar)

Cyclophosphamide may be used as monotherapy or as a steroid-sparing agent. It is chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells.

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