Pemphigus Herpetiformis Medication

Updated: Nov 16, 2018
  • Author: Blanca Anais Estupiñan; Chief Editor: William D James, MD  more...
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Medication

Medication Summary

Because pemphigus herpetiformis is a rare variant of pemphigus, comprehensive therapeutic studies of large numbers of patients have not taken place. The following treatment options are taken from the personal experiences of physician-investigators with a small number of patients.

In general, dapsone is the drug of choice for pemphigus herpetiformis treatment. Other anti-inflammatory and immunosuppressive medications are also effective.

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Anti-inflammatories

Class Summary

Because pemphigus herpetiformis lesions are characterized by prominent infiltration of inflammatory cells, anti-inflammatory medication may inhibit the functions of these cells, therefore improving disease conditions.

Dapsone (Avlosulfon)

Dapsone's mechanism of action is similar to that of sulfonamides, in which competitive antagonists of PABA prevent the formation of folic acid, inhibiting bacterial growth. Dapsone is used alone or in conjunction with other anti-inflammatory medications or immunosuppressives for pemphigus herpetiformis.

Prednisone (Deltasone)

Prednisone is an immunosuppressant used 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 lymphocytes and antibody production. Taken alone, or in conjunction with other anti-inflammatory or immunosuppressive medications, it is useful for controlling pemphigus herpetiformis.

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Immunosuppressives

Class Summary

Pemphigus herpetiformis is an autoimmune disease; immunosuppressives are useful in suppressing autoimmune response.

Azathioprine (Imuran)

Azathioprine antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. It may decrease the proliferation of immune cells, which results in lower autoimmune activity. Azathioprine is usually used as a supplement to anti-inflammatory medication; it is useful in controlling pemphigus herpetiformis.

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Biological Response Modulator

Class Summary

The agents in this class target specific antigens in carcinoma cells and induce cytotoxicity. Rituximab is used off-label for steroid-refractory pemphigus herpetiformis.

Rituximab (Rituxan)

Rituximab is a genetically engineered chimeric murine/human monoclonal antibody against human CD20, a molecule present in normal and malignant B lymphocytes. It is described in case reports as a promising biological treatment for B-lymphocyte–mediated diseases (eg, pemphigus vulgaris).

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